{"id":163324,"date":"2026-07-27T11:53:24","date_gmt":"2026-07-27T11:53:24","guid":{"rendered":"https:\/\/pabau.com\/?p=163324"},"modified":"2026-08-17T11:48:36","modified_gmt":"2026-08-17T11:48:36","slug":"icd-10-code-m041","status":"publish","type":"post","link":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/","title":{"rendered":"ICD-10 Code M04.1: Periodic fever syndromes (FY2026)"},"content":{"rendered":"\n<script type=\"application\/ld+json\">{\"@context\":\"https:\/\/schema.org\",\"@type\":\"MedicalWebPage\",\"headline\":\"ICD-10 Code M04.1: Periodic fever syndromes\",\"description\":\"ICD-10 Code M04.1 covers periodic fever syndromes including FMF, TRAPS (TNF receptor-associated periodic syndrome), and HIDS\/MKD (hyperimmunoglobin D syndrome \/ mevalonate kinase deficiency). Billable for FY2026.\",\"url\":\"https:\/\/pabau.com\/diagnostic-codes\/icd-10-code-m041-periodic-fever-syndromes\/\",\"audience\":{\"@type\":\"MedicalAudience\",\"audienceType\":\"Clinician\"},\"reviewedBy\":{\"@type\":\"Person\",\"name\":\"Dr Vanja Kitanova\",\"jobTitle\":\"Medical Reviewer\",\"affiliation\":{\"@type\":\"Organization\",\"name\":\"Pabau\"},\"knowsAbout\":[\"Medical Coding\",\"Clinical Documentation\",\"Healthcare Billing\",\"Clinical Safety\"],\"sameAs\":\"https:\/\/pabau.com\/blog\/author\/vanja\/\"},\"datePublished\":\"2026-07-25\",\"dateModified\":\"2026-07-25\"}<\/script>\n\n\n        <div id=\"key_takeaways\">\n            <div class=\"header\">\n                                    <img decoding=\"async\" src=\"https:\/\/pabau.com\/wp-content\/uploads\/2025\/12\/Key-Takeaways-Icon.svg\" alt=\"Key Takeaways\" height=\"42\" width=\"42\">\n                                <h3>Key Takeaways<\/h3>\n            <\/div>\n            <div class=\"list\">\n                                                            <div class=\"item\">\n                            <div>\n                                <svg width=\"20\" height=\"27\" viewBox=\"0 0 20 27\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\">\n                                    <path\n                                        d=\"M18.6223 14.3857C19.1246 13.8975 19.1246 13.1045 18.6223 12.6162L12.1938 6.36621C11.6915 5.87793 10.8759 5.87793 10.3737 6.36621C9.87143 6.85449 9.87143 7.64746 10.3737 8.13574L14.6125 12.2529H2.28571C1.57455 12.2529 1 12.8115 1 13.5029C1 14.1943 1.57455 14.7529 2.28571 14.7529H14.6085L10.3777 18.8701C9.87545 19.3584 9.87545 20.1514 10.3777 20.6396C10.8799 21.1279 11.6955 21.1279 12.1978 20.6396L18.6263 14.3896L18.6223 14.3857Z\"\n                                        fill=\"#3D3D46\" \/>\n                                <\/svg>\n                            <\/div>\n                            <p>ICD-10 Code M04.1 is a billable\/specific ICD-10-CM diagnosis code for periodic fever syndromes, effective FY2026 (October 1, 2025).<\/p>\n                        <\/div>\n                                                                                <div class=\"item\">\n                            <div>\n                                <svg width=\"20\" height=\"27\" viewBox=\"0 0 20 27\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\">\n                                    <path\n                                        d=\"M18.6223 14.3857C19.1246 13.8975 19.1246 13.1045 18.6223 12.6162L12.1938 6.36621C11.6915 5.87793 10.8759 5.87793 10.3737 6.36621C9.87143 6.85449 9.87143 7.64746 10.3737 8.13574L14.6125 12.2529H2.28571C1.57455 12.2529 1 12.8115 1 13.5029C1 14.1943 1.57455 14.7529 2.28571 14.7529H14.6085L10.3777 18.8701C9.87545 19.3584 9.87545 20.1514 10.3777 20.6396C10.8799 21.1279 11.6955 21.1279 12.1978 20.6396L18.6263 14.3896L18.6223 14.3857Z\"\n                                        fill=\"#3D3D46\" \/>\n                                <\/svg>\n                            <\/div>\n                            <p>M04.1 covers three hereditary autoinflammatory conditions: familial Mediterranean fever (FMF), TRAPS, and HIDS\/MKD.<\/p>\n                        <\/div>\n                                                                                <div class=\"item\">\n                            <div>\n                                <svg width=\"20\" height=\"27\" viewBox=\"0 0 20 27\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\">\n                                    <path\n                                        d=\"M18.6223 14.3857C19.1246 13.8975 19.1246 13.1045 18.6223 12.6162L12.1938 6.36621C11.6915 5.87793 10.8759 5.87793 10.3737 6.36621C9.87143 6.85449 9.87143 7.64746 10.3737 8.13574L14.6125 12.2529H2.28571C1.57455 12.2529 1 12.8115 1 13.5029C1 14.1943 1.57455 14.7529 2.28571 14.7529H14.6085L10.3777 18.8701C9.87545 19.3584 9.87545 20.1514 10.3777 20.6396C10.8799 21.1279 11.6955 21.1279 12.1978 20.6396L18.6263 14.3896L18.6223 14.3857Z\"\n                                        fill=\"#3D3D46\" \/>\n                                <\/svg>\n                            <\/div>\n                            <p>The code sits under parent code M04 (Autoinflammatory syndromes) in chapter M00-M99; it carries an Excludes2 note only (no Excludes1 note) that coders must check before assigning.<\/p>\n                        <\/div>\n                                                                                <div class=\"item\">\n                            <div>\n                                <svg width=\"20\" height=\"27\" viewBox=\"0 0 20 27\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\">\n                                    <path\n                                        d=\"M18.6223 14.3857C19.1246 13.8975 19.1246 13.1045 18.6223 12.6162L12.1938 6.36621C11.6915 5.87793 10.8759 5.87793 10.3737 6.36621C9.87143 6.85449 9.87143 7.64746 10.3737 8.13574L14.6125 12.2529H2.28571C1.57455 12.2529 1 12.8115 1 13.5029C1 14.1943 1.57455 14.7529 2.28571 14.7529H14.6085L10.3777 18.8701C9.87545 19.3584 9.87545 20.1514 10.3777 20.6396C10.8799 21.1279 11.6955 21.1279 12.1978 20.6396L18.6263 14.3896L18.6223 14.3857Z\"\n                                        fill=\"#3D3D46\" \/>\n                                <\/svg>\n                            <\/div>\n                            <p>Pabau&#8217;s claims management software supports accurate ICD-10 coding workflows, helping practitioners document and submit diagnosis codes like M04.1 without errors.<\/p>\n                        <\/div>\n                                                <\/div>\n        <\/div>\n    \n\n\n<p class=\"wp-block-paragraph\">ICD-10 Code M04.1 is a billable, specific ICD-10-CM code for periodic fever syndromes, covering familial Mediterranean fever (FMF), TRAPS, and HIDS\/MKD. It&#8217;s the code to use in place of a generic fever code once one of these hereditary autoinflammatory diagnoses is confirmed, since payers validate medical necessity against the specific diagnosis on the claim.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">This reference covers the full scope of ICD-10 Code M04.1: its billable status, the three included conditions, parent and sibling codes, Excludes notes, the ICD-9-CM crosswalk, and documentation requirements for accurate submission.<\/p>\n\n\n\n<figure class=\"wp-block-embed is-type-video is-provider-youtube wp-block-embed-youtube wp-embed-aspect-16-9 wp-has-aspect-ratio\"><div class=\"wp-block-embed__wrapper\">\n<iframe title=\"ICD-10 Revisions: Stay Updated or Fall Behind\" width=\"800\" height=\"450\" src=\"https:\/\/www.youtube.com\/embed\/bqAyb-BCA0w?feature=oembed\" frameborder=\"0\" allow=\"accelerometer; autoplay; clipboard-write; encrypted-media; gyroscope; picture-in-picture; web-share\" referrerpolicy=\"strict-origin-when-cross-origin\" allowfullscreen><\/iframe>\n<\/div><\/figure>\n\n\n\n<h2 id=\"h-icd-10-code-m04-1-definition-billable-status-and-code-details\" class=\"wp-block-heading\">ICD-10 Code M04.1: Definition, billable status, and code details<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">ICD-10 Code M04.1 is a billable, specific ICD-10-CM diagnosis code representing <strong>periodic fever syndromes<\/strong>. It is valid for use on claims submitted for dates of service on or after October 1, 2025, under the FY2026 edition of ICD-10-CM. Coders who work in general practice or rheumatology will encounter this code when documenting hereditary autoinflammatory diseases that cause recurrent fever episodes.<\/p>\n\n\n\n<h3 id=\"h-code-details-at-a-glance\" class=\"wp-block-heading\">Code details at a glance<\/h3>\n\n\n\n<table style=\"width:100%;border-collapse:separate;border-spacing:0;border-radius:12px;overflow:hidden;font-size:15px;line-height:1.5;margin:1.5em 0 2em;box-shadow:0 2px 12px rgba(0,0,0,0.08)\">\n<thead>\n<tr>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Field<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Detail<\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">Code<\/td>\n<td style=\"padding:12px 16px;color:#374151\">M04.1<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0;background:#F9FAFB\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">Description<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Periodic fever syndromes<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">Billable\/Specific<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Yes<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0;background:#F9FAFB\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">Effective date<\/td>\n<td style=\"padding:12px 16px;color:#374151\">October 1, 2025 (FY2026)<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">Chapter<\/td>\n<td style=\"padding:12px 16px;color:#374151\">M00-M99 (Musculoskeletal and connective tissue diseases)<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0;background:#F9FAFB\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">Block<\/td>\n<td style=\"padding:12px 16px;color:#374151\">M04-M04 (Autoinflammatory syndromes)<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">Parent code<\/td>\n<td style=\"padding:12px 16px;color:#374151\">M04 (Autoinflammatory syndromes)<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0;background:#F9FAFB\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">HIPAA-mandated code set<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Yes (ICD-10-CM)<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n\n\n\n<p class=\"wp-block-paragraph\">The <a href=\"https:\/\/icd10cmtool.cdc.gov\/\" target=\"_blank\" rel=\"nofollow noopener\">CDC\/NCHS ICD-10-CM web tool<\/a> confirms M04.1 as a valid FY2026 code. Because it is a specific, billable code rather than a header or non-billable parent, it can appear as a primary or secondary diagnosis on a claim without further subdivision.<\/p>\n\n\n\n<h2 id=\"h-conditions-included-under-m04-1-applicable-to\" class=\"wp-block-heading\">Conditions included under M04.1 (Applicable To)<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">The Applicable To note in the ICD-10-CM tabular list defines the conditions that map to ICD-10 Code M04.1: familial Mediterranean fever (FMF), TNF receptor-associated periodic syndrome (TRAPS), and hyperimmunoglobin D syndrome \/ mevalonate kinase deficiency (HIDS\/MKD, two names for the same condition).<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">These are distinct hereditary autoinflammatory diseases, each driven by mutations in specific genes that dysregulate the innate immune system. All three cause recurrent fever episodes and systemic inflammation.<\/p>\n\n\n\n<h3 id=\"h-familial-mediterranean-fever-fmf\" class=\"wp-block-heading\">Familial Mediterranean fever (FMF)<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">FMF is the most common hereditary periodic fever syndrome. It results from mutations in the <em>MEFV<\/em> gene and causes recurrent episodes of fever lasting 12-72 hours, typically accompanied by serositis (peritonitis, pleuritis, or pericarditis). Episodes resolve spontaneously. Clinicians at functional medicine practices managing patients with recurrent unexplained fevers should consider FMF and use M04.1 when the diagnosis is confirmed.<\/p>\n\n\n\n<h3 id=\"h-traps-and-hids-mkd\" class=\"wp-block-heading\">TRAPS and HIDS\/MKD<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">The remaining two conditions under M04.1 share the periodic fever pattern but differ in genetic basis, episode duration, and associated features.<\/p>\n\n\n\n<table style=\"width:100%;border-collapse:separate;border-spacing:0;border-radius:12px;overflow:hidden;font-size:15px;line-height:1.5;margin:1.5em 0 2em;box-shadow:0 2px 12px rgba(0,0,0,0.08)\">\n<thead>\n<tr>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Condition<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Full name<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Gene<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Typical episode duration<\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">TRAPS<\/td>\n<td style=\"padding:12px 16px;color:#374151\">TNF receptor-associated periodic syndrome<\/td>\n<td style=\"padding:12px 16px;color:#374151\">TNFRSF1A<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Days to weeks<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">HIDS<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Hyperimmunoglobulinemia D syndrome (mevalonate kinase deficiency)<\/td>\n<td style=\"padding:12px 16px;color:#374151\">MVK<\/td>\n<td style=\"padding:12px 16px;color:#374151\">3-7 days<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n\n\n\n<p class=\"wp-block-paragraph\">Both are rare diseases. Clinical diagnosis typically involves genetic testing, inflammatory marker panels, and specialist referral. The documentation in the medical record must reflect a confirmed or suspected autoinflammatory syndrome to justify M04.1 on a claim.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The same principle applies across the M00-M99 chapter: an unspecified code such as M12.9 carries the same denial risk as vague fever documentation does here.<\/p>\n\n\n        <div id=\"pro_tip\">\n            <div class=\"img\">\n                <svg width=\"42\" height=\"42\" viewBox=\"0 0 42 42\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\">\n                    <path fill-rule=\"evenodd\" clip-rule=\"evenodd\"\n                        d=\"M12.6383 19.5238C12.6632 21.2071 12.8734 22.9926 12.0685 24.5523C11.0341 26.8917 8.45076 28.169 7.56292 30.5918C6.63082 33.1061 7.19505 36.185 9.31371 37.9786C10.929 39.5678 13.4183 39.9873 15.6061 39.5463C17.4592 39.073 19.1049 37.8388 19.9706 36.1608C20.781 34.7141 20.7866 32.9904 20.5293 31.3985C20.1006 29.3092 18.3775 27.8705 16.9807 26.362C14.8814 24.1005 14.6684 20.6854 15.595 17.8915C16.4331 15.4768 19.0026 14.2344 20.0425 11.9461C20.7202 10.6769 20.7506 9.20327 20.6068 7.81304C20.1864 5.40098 18.3139 3.23631 15.8716 2.56674C13.9742 2.03969 11.8224 2.30052 10.1739 3.37614C8.70522 4.34688 7.5878 5.86618 7.28356 7.58178C6.76081 9.82981 7.53525 12.2822 9.20307 13.9118C10.7658 15.4741 12.4806 17.2273 12.6383 19.5238Z\"\n                        fill=\"#2BADD4\" \/>\n                    <path fill-rule=\"evenodd\" clip-rule=\"evenodd\"\n                        d=\"M22.7048 19.8387C22.6822 22.3279 24.4507 24.6234 26.7493 25.4682C28.0531 25.961 29.4725 25.9183 30.7989 25.5487C32.0575 24.9955 33.301 24.2237 34.0069 23.0092C34.8384 21.5811 35.2982 19.8286 34.7832 18.2094C34.3611 16.2507 32.8739 14.6541 31.0275 13.9426C28.6736 12.9595 25.8073 13.7942 24.1719 15.7001C23.2022 16.8366 22.6143 18.3326 22.7048 19.8387Z\"\n                        fill=\"#2BADD4\" \/>\n                <\/svg>\n            <\/div>\n            <div class=\"text\">\n                <h3>Pro Tip<\/h3>\n                <p>Document the specific named syndrome (FMF, TRAPS, or HIDS\/MKD) in the clinical note, not just &#8216;periodic fever.&#8217; Payers and auditors reviewing claims under M04.1 expect the record to identify which autoinflammatory condition supports the code. Generic fever language in the note creates denial risk.<\/p>\n            <\/div>\n        <\/div>\n    \n\n\n<h2 id=\"h-parent-code-m04-autoinflammatory-syndromes\" class=\"wp-block-heading\">Parent code M04: Autoinflammatory syndromes<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">ICD-10 Code M04.1 sits beneath parent code M04 (Autoinflammatory syndromes), which is itself within the musculoskeletal and connective tissue diseases chapter (M00-M99). M04 is a non-billable header code. It cannot be submitted on a claim. Coders must use a specific subcategory such as M04.1 or one of the sibling codes listed below.<\/p>\n\n\n\n<h3 id=\"h-sibling-codes-under-m04\" class=\"wp-block-heading\">Sibling codes under M04<\/h3>\n\n\n\n<table style=\"width:100%;border-collapse:separate;border-spacing:0;border-radius:12px;overflow:hidden;font-size:15px;line-height:1.5;margin:1.5em 0 2em;box-shadow:0 2px 12px rgba(0,0,0,0.08)\">\n<thead>\n<tr>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Code<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Description<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Billable<\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">M04<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Autoinflammatory syndromes (parent, non-billable header)<\/td>\n<td style=\"padding:12px 16px;color:#374151\">No<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0;background:#F9FAFB\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">M04.1<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Periodic fever syndromes (FMF, TRAPS, HIDS\/MKD)<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Yes<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">M04.2<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Cryopyrin-associated periodic syndromes<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Yes<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0;background:#F9FAFB\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">M04.8<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Other autoinflammatory syndromes<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Yes<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">M04.9<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Autoinflammatory syndrome, unspecified<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Yes<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n\n\n\n<p class=\"wp-block-paragraph\">Two related autoinflammatory syndromes are coded elsewhere, not under M04.1: cryopyrin-associated periodic syndromes (CAPS) \u2014 including CINCA, familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and NOMID \u2014 have their own dedicated code, M04.2, and periodic fever aphthous stomatitis pharyngitis adenitis (PFAPA) syndrome is coded under M04.8 (Other autoinflammatory syndromes).<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Coders should confirm the specific diagnosis in the record before assigning M04.1, M04.2, or M04.8, since these are distinct, non-overlapping codes. The <a href=\"https:\/\/www.cms.gov\/medicare\/coding-billing\/icd-10-codes\" target=\"_blank\" rel=\"nofollow noopener\">CMS ICD-10 codes page<\/a> provides the official tabular list to resolve these distinctions.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">In <a href=\"https:\/\/pabau.com\/industry\/chiropractic-software\/\">software built for chiropractic practices<\/a>, M-code diagnoses drive care plans measured visit by visit.<\/p>\n\n\n\n<h2 id=\"h-excludes-notes-what-icd-10-code-m04-1-does-not-cover\" class=\"wp-block-heading\">Excludes notes: What ICD-10 Code M04.1 does not cover<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">Coding accuracy depends on understanding what a code explicitly excludes. The ICD-10-CM tabular list defines two types of exclusion notes (Excludes1 and Excludes2), but M04.1 itself carries only an Excludes2 note \u2014 there is no Excludes1 note attached to this code.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Clinicians managing patients with complex inflammatory histories, such as those seen at integrative medicine practices, should review this before applying M04.1.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">M04.1 carries an <strong>Excludes2 note<\/strong>, inherited from parent code M04, which lists Crohn&#8217;s disease (regional enteritis), K50.-, as a condition not included in M04.1 but which may legitimately coexist with it.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">A patient can have both M04.1 and an Excludes2 condition such as Crohn&#8217;s disease. Both codes may be reported if documented. The M00-M99 chapter also carries its own chapter-level Excludes2 notes that apply across the block.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The specific excluded codes should be confirmed against the official ICD-10-CM tabular list before submission. The <a href=\"https:\/\/www.aapc.com\/codes\/icd-10-codes-range\/\" target=\"_blank\" rel=\"nofollow noopener\">AAPC ICD-10-CM code reference<\/a> and the CDC web tool both display the current excludes notes for M04.1 with the applicable code numbers. Review the official tabular list for FY2026 to confirm the precise Excludes2 entry before billing.<\/p>\n\n\n        <div id=\"book_a_demo\">\n            <div class=\"left-side\">\n                <div class=\"logo\">\n                    <img decoding=\"async\"\n                        src=\"https:\/\/pabau.com\/wp-content\/uploads\/2026\/01\/Logo.svg\"\n                        alt=\"Logo\"\n                        loading=\"lazy\"\n                    \/>\n                <\/div>\n\n                <h3 class=\"heading\">\n                    Reduce claim denials with accurate ICD-10 coding workflows                <\/h3>\n\n                <p class=\"description\">\n                    Pabau&#8217;s claims management tools help your practice document diagnosis codes correctly, track submissions, and resolve denials faster. See how it works in a live demo.                <\/p>\n\n                <div class=\"button-group\">\n                    <a href=\"\/book-demo\/\" class=\"btn btn-scale-effect btn-cta-color btn-md\">\n                        <span class=\"btn-text\">Book a demo<\/span>\n                        <div class=\"btn-icon btn-icon-right\" aria-hidden=\"true\">\n                            <svg\n                                width=\"14\"\n                                height=\"12\"\n                                viewBox=\"0 0 14 12\"\n                                fill=\"none\"\n                                xmlns=\"http:\/\/www.w3.org\/2000\/svg\"\n                            >\n                                <path\n                                    d=\"M0.75 4.77295C0.335786 4.77295 0 5.10874 0 5.52295C0 5.93716 0.335786 6.27295 0.75 6.27295V5.52295V4.77295ZM13.2803 6.05328C13.5732 5.76039 13.5732 5.28551 13.2803 4.99262L8.50736 0.219648C8.21447 -0.073245 7.73959 -0.073245 7.4467 0.219648C7.15381 0.512542 7.15381 0.987415 7.4467 1.28031L11.6893 5.52295L7.4467 9.76559C7.15381 10.0585 7.15381 10.5334 7.4467 10.8263C7.73959 11.1191 8.21447 11.1191 8.50736 10.8263L13.2803 6.05328ZM0.75 5.52295V6.27295L12.75 6.27295V5.52295V4.77295L0.75 4.77295V5.52295Z\"\n                                    fill=\"currentColor\"\n                                ><\/path>\n                            <\/svg>\n                        <\/div>\n                    <\/a>\n                <\/div>\n            <\/div>\n\n            <div class=\"right-side\">\n                <img decoding=\"async\"\n                    src=\"https:\/\/pabau.com\/wp-content\/uploads\/2026\/01\/Home-Page-Concept-4.webp\"\n                    alt=\"Pabau practice management platform\"\n                    loading=\"lazy\"\n                \/>\n            <\/div>\n        <\/div>\n        \n\n\n<h2 id=\"h-icd-9-cm-to-icd-10-cm-crosswalk-for-m04-1\" class=\"wp-block-heading\">ICD-9-CM to ICD-10-CM crosswalk for M04.1<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">Practices still reconciling older claims or auditing historical records need to know which legacy ICD-9-CM code corresponds to M04.1. Per the EHR integration workflows used by most modern practices, code crosswalks are handled automatically by billing software. For manual reconciliation, the General Equivalence Mappings (GEMs) published by CMS provide the authoritative mapping.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The ICD-9-CM code that crosswalks to M04.1 is <strong>277.31<\/strong> (Familial Mediterranean fever), which was the closest legacy equivalent. Because ICD-9-CM had limited granularity for autoinflammatory syndromes, the crosswalk is approximate: 277.31 maps to M04.1 as a forward mapping, but M04.1 also receives patients who were previously coded under broader fever or arthropathy categories.<\/p>\n\n\n\n<table style=\"width:100%;border-collapse:separate;border-spacing:0;border-radius:12px;overflow:hidden;font-size:15px;line-height:1.5;margin:1.5em 0 2em;box-shadow:0 2px 12px rgba(0,0,0,0.08)\">\n<thead>\n<tr>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">ICD-9-CM code<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Description<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">ICD-10-CM code<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Notes<\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">277.31<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Familial Mediterranean fever<\/td>\n<td style=\"padding:12px 16px;color:#374151\">M04.1<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Primary forward mapping per CMS GEMs<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n\n\n\n<p class=\"wp-block-paragraph\">Verify the crosswalk against the <a href=\"https:\/\/resdac.org\/articles\/international-classification-disease-icd-codes-medicare-files\" target=\"_blank\" rel=\"nofollow noopener\">ResDAC ICD-Medicare guide<\/a> before using legacy codes for claims reconciliation. Crosswalk data is not always one-to-one, and payers may have specific guidance for adjudicating historical claims.<\/p>\n\n\n\n<h2 id=\"h-coding-guidelines-and-documentation-requirements-for-periodic-fever-syndromes\" class=\"wp-block-heading\">Coding guidelines and documentation requirements for periodic fever syndromes<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">The American Hospital Association (AHA) Coding Clinic and the CMS ICD-10-CM Official Guidelines for Coding and Reporting set the documentation standards that support M04.1 assignment. These guidelines apply to all providers billing under HIPAA&#8217;s mandated ICD-10-CM code set.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The record must support the code. For M04.1, the minimum documentation includes:<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>A physician or qualified provider&#8217;s documented diagnosis naming the specific periodic fever syndrome (FMF, TRAPS, or HIDS\/MKD)<\/li>\n\n\n\n<li>Clinical evidence supporting the diagnosis (genetic test results, inflammatory marker values, or specialist confirmation)<\/li>\n\n\n\n<li>The episode or condition being treated at the current encounter<\/li>\n\n\n\n<li>Any relevant comorbidities documented separately with their own codes<\/li>\n<\/ul>\n\n\n\n<p class=\"wp-block-paragraph\">The same standard applies to other musculoskeletal diagnoses. A note that says &#8220;recurrent fever, query FMF&#8221; is not equivalent to &#8220;familial Mediterranean fever, confirmed by MEFV gene mutation testing.&#8221;<\/p>\n\n\n\n<h3 id=\"h-sequencing-guidelines\" class=\"wp-block-heading\">Sequencing guidelines<\/h3>\n\n\n\n<p class=\"wp-block-paragraph\">M04.1 may be reported as the principal diagnosis when the periodic fever syndrome is the condition primarily responsible for the visit. It may also be a secondary diagnosis when the patient is seen for a related complication or comorbidity.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">The ICD-10-CM Official Guidelines require that the condition established after study to be chiefly responsible for the encounter becomes the principal diagnosis. For outpatient visits, the condition after the highest level of evaluation during the encounter guides code selection.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Practices using claims management software can flag encounters where secondary diagnosis codes like M04.1 require supporting documentation before submission, reducing the audit exposure that comes from unsupported autoinflammatory coding.<\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" src=\"https:\/\/cdn.pabau.com\/cdn\/attachments\/pulse\/content-engine\/diagnostic_codes\/icd-10-code-m041\/track-claims-from-start-to-finish.webp\" alt=\"Track claims from start to Finish\"\/><figcaption class=\"wp-element-caption\"><em>Track claims from start to Finish<\/em><\/figcaption><\/figure>\n\n\n\n<div style=\"height:20px\" aria-hidden=\"true\" class=\"wp-block-spacer\"><\/div>\n\n\n\n<h2 id=\"h-how-to-use-icd-10-code-m04-1-in-ehr-systems-and-practice-management-workflows\" class=\"wp-block-heading\">How to use ICD-10 Code M04.1 in EHR systems and practice management workflows<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">Code reference sites list the definition and excludes notes for M04.1, but they stop before the workflow question: how does a busy practice apply the code correctly inside its EHR and billing system?<\/p>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Search by condition name, not code number.<\/strong> Most EHR ICD-10 search tools surface M04.1 more reliably when you type &#8220;familial Mediterranean fever&#8221; or &#8220;periodic fever syndrome&#8221; than when you type the code directly. This also prevents accidental selection of the parent non-billable code M04.<\/li>\n\n\n\n<li><strong>Link the code to encounter documentation before checkout.<\/strong> In integrated practice management platforms, the diagnosis code should be attached to the encounter note at the time of visit. This creates a documented connection between the clinical note and the claim, which is what auditors and payers inspect when they review M04.1 submissions.<\/li>\n\n\n\n<li><strong>Flag encounters for documentation review.<\/strong> Because periodic fever syndromes require genetic or specialist confirmation, a front-end coding review step helps catch instances where the provider has entered M04.1 before documentation is complete. Set a workflow rule to hold claims with M04.1 until the relevant test result or specialist letter is attached.<\/li>\n\n\n\n<li><strong>Check payer-specific guidelines before submission.<\/strong> Not all payers follow CMS guidelines identically. Some commercial insurers require prior authorization or additional documentation for rare autoinflammatory disease codes. Confirm with the specific payer before submitting M04.1 on a new patient claim.<\/li>\n\n\n\n<li><strong>Use a secondary code for manifestations.<\/strong> If the patient&#8217;s periodic fever syndrome has caused a documented complication (such as amyloidosis in long-standing FMF), that complication should be coded separately alongside M04.1. The HIPAA compliance requirements for diagnosis code accuracy require that all documented conditions at an encounter be coded.<\/li>\n<\/ol>\n\n\n\n<p class=\"wp-block-paragraph\">Practices using practice management software like Pabau can attach ICD-10 codes directly to treatment records via the client record system, ensuring that every claim submission has a corresponding clinical note rather than a standalone diagnosis entry.<\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" src=\"https:\/\/cdn.pabau.com\/cdn\/attachments\/pulse\/content-engine\/diagnostic_codes\/icd-10-code-m041\/detailed-client-records-in-pabau.webp\" alt=\"Detailed client records in Pabau\"\/><figcaption class=\"wp-element-caption\"><em>Detailed client records in Pabau<\/em><\/figcaption><\/figure>\n\n\n\n<div style=\"height:20px\" aria-hidden=\"true\" class=\"wp-block-spacer\"><\/div>\n\n\n        <div id=\"pro_tip\">\n            <div class=\"img\">\n                <svg width=\"42\" height=\"42\" viewBox=\"0 0 42 42\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\">\n                    <path fill-rule=\"evenodd\" clip-rule=\"evenodd\"\n                        d=\"M12.6383 19.5238C12.6632 21.2071 12.8734 22.9926 12.0685 24.5523C11.0341 26.8917 8.45076 28.169 7.56292 30.5918C6.63082 33.1061 7.19505 36.185 9.31371 37.9786C10.929 39.5678 13.4183 39.9873 15.6061 39.5463C17.4592 39.073 19.1049 37.8388 19.9706 36.1608C20.781 34.7141 20.7866 32.9904 20.5293 31.3985C20.1006 29.3092 18.3775 27.8705 16.9807 26.362C14.8814 24.1005 14.6684 20.6854 15.595 17.8915C16.4331 15.4768 19.0026 14.2344 20.0425 11.9461C20.7202 10.6769 20.7506 9.20327 20.6068 7.81304C20.1864 5.40098 18.3139 3.23631 15.8716 2.56674C13.9742 2.03969 11.8224 2.30052 10.1739 3.37614C8.70522 4.34688 7.5878 5.86618 7.28356 7.58178C6.76081 9.82981 7.53525 12.2822 9.20307 13.9118C10.7658 15.4741 12.4806 17.2273 12.6383 19.5238Z\"\n                        fill=\"#2BADD4\" \/>\n                    <path fill-rule=\"evenodd\" clip-rule=\"evenodd\"\n                        d=\"M22.7048 19.8387C22.6822 22.3279 24.4507 24.6234 26.7493 25.4682C28.0531 25.961 29.4725 25.9183 30.7989 25.5487C32.0575 24.9955 33.301 24.2237 34.0069 23.0092C34.8384 21.5811 35.2982 19.8286 34.7832 18.2094C34.3611 16.2507 32.8739 14.6541 31.0275 13.9426C28.6736 12.9595 25.8073 13.7942 24.1719 15.7001C23.2022 16.8366 22.6143 18.3326 22.7048 19.8387Z\"\n                        fill=\"#2BADD4\" \/>\n                <\/svg>\n            <\/div>\n            <div class=\"text\">\n                <h3>Pro Tip<\/h3>\n                <p>Run a quarterly audit of all claims submitted with M04.1. Pull encounters where the code was used and verify that the associated note names the specific syndrome and includes supporting documentation. Payers flagging autoinflammatory codes for audit will look for exactly this chain of evidence.<\/p>\n            <\/div>\n        <\/div>\n    \n\n\n<h2 id=\"h-code-history-and-annual-updates-for-icd-10-code-m04-1\" class=\"wp-block-heading\">Code history and annual updates for ICD-10 Code M04.1<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">ICD-10 Code M04.1 has been a stable code since the ICD-10-CM category M04 (Autoinflammatory syndromes) was introduced. Other codes in the M00-M99 chapter. M04.1 is active and billable for FY2026 (October 1, 2025 through September 30, 2026).<\/p>\n\n\n\n<table style=\"width:100%;border-collapse:separate;border-spacing:0;border-radius:12px;overflow:hidden;font-size:15px;line-height:1.5;margin:1.5em 0 2em;box-shadow:0 2px 12px rgba(0,0,0,0.08)\">\n<thead>\n<tr>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Fiscal year<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Status<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Effective dates<\/th>\n<th style=\"background:#121D36;color:#fff;padding:14px 16px;text-align:left;font-weight:600;font-size:14px;letter-spacing:0.3px;border-bottom:2px solid #2BADD4\">Notes<\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr style=\"border-bottom:1px solid #E8ECF0\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">FY2026<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Active \/ Billable<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Oct 1, 2025 &#8211; Sep 30, 2026<\/td>\n<td style=\"padding:12px 16px;color:#374151\">No description change from FY2025<\/td>\n<\/tr>\n<tr style=\"border-bottom:1px solid #E8ECF0;background:#F9FAFB\">\n<td style=\"padding:12px 16px;font-weight:600;color:#121D36\">FY2025<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Active \/ Billable<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Oct 1, 2024 &#8211; Sep 30, 2025<\/td>\n<td style=\"padding:12px 16px;color:#374151\">Stable code, no revision<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n\n\n\n<p class=\"wp-block-paragraph\">The World Health Organization maintains the <a href=\"https:\/\/www.who.int\/classifications\/classification-of-diseases\" target=\"_blank\" rel=\"nofollow noopener\">ICD classification<\/a> framework on which ICD-10-CM is based. The National Center for Health Statistics (NCHS) and CMS jointly maintain the US clinical modification. Practices should check the CMS ICD-10 update files each October to confirm that codes used routinely remain active in the new fiscal year edition.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">For practices managing patient data security alongside clinical coding, keeping ICD-10 code libraries updated in EHR systems is part of maintaining accurate, HIPAA-compliant records. Outdated codes on claims trigger automated edits and can delay payment for legitimate encounters.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">Related ICD-10 codes<\/h2>\n\n\n\n<ul class=\"wp-block-list\">\n<li><a href=\"https:\/\/pabau.com\/diagnostic-codes\/icd-10-code-m1990\/\">ICD-10 code M19.90 \u2014 Degenerative Joint Disease Documentation<\/a><\/li>\n\n\n<li><a href=\"https:\/\/pabau.com\/diagnostic-codes\/icd-10-code-m042\/\">ICD-10 code M04.2 \u2014 Cryopyrin-associated periodic syndromes<\/a><\/li>\n\n\n<li><a href=\"https:\/\/pabau.com\/diagnostic-codes\/icd-10-code-m049\/\">ICD-10 code M04.9 \u2014 Autoinflammatory syndrome, unspecified<\/a><\/li>\n\n\n<li><a href=\"https:\/\/pabau.com\/diagnostic-codes\/icd-10-code-m059\/\">ICD-10 Code M05.9 \u2014 Rheumatoid Arthritis with RF<\/a><\/li>\n<\/ul>\n\n\n\n<h2 id=\"h-conclusion\" class=\"wp-block-heading\">Conclusion<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">Periodic fever syndromes are rare, but coding them incorrectly is a common and preventable problem. ICD-10 Code M04.1 is the right code for confirmed hereditary autoinflammatory diseases including FMF, TRAPS, and HIDS\/MKD. The documentation threshold is higher than for common diagnosis codes: the clinical note must name the specific syndrome and reference supporting evidence.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Practices that pair accurate ICD-10 coding with a structured workflow can reduce denials and audit exposure. Pabau&#8217;s claims management software helps practices attach diagnosis codes to encounter notes, review submissions before they leave the practice, and track denial patterns over time. To see how this works in your practice, <a href=\"https:\/\/pabau.com\/book-demo\/\">book a demo<\/a>.<\/p>\n\n\n        <div id=\"expert_picks\">\n            <div class=\"header\">\n                                    <img decoding=\"async\" src=\"https:\/\/pabau.com\/wp-content\/uploads\/2025\/11\/Expert-Picks.svg\" alt=\"Continue your research\" height=\"42\" width=\"42\">\n                                <h3>Continue your research<\/h3>\n            <\/div>\n            <div class=\"content\">\n                                                            <div class=\"item\">\n                            <div>\n                                <svg width=\"20\" height=\"27\" viewBox=\"0 0 20 27\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\">\n                                    <path\n                                        d=\"M18.6223 14.3857C19.1246 13.8975 19.1246 13.1045 18.6223 12.6162L12.1938 6.36621C11.6915 5.87793 10.8759 5.87793 10.3737 6.36621C9.87143 6.85449 9.87143 7.64746 10.3737 8.13574L14.6125 12.2529H2.28571C1.57455 12.2529 1 12.8115 1 13.5029C1 14.1943 1.57455 14.7529 2.28571 14.7529H14.6085L10.3777 18.8701C9.87545 19.3584 9.87545 20.1514 10.3777 20.6396C10.8799 21.1279 11.6955 21.1279 12.1978 20.6396L18.6263 14.3896L18.6223 14.3857Z\"\n                                        fill=\"#54B2D3\" \/>\n                                <\/svg>\n                            <\/div>\n                            <p><strong>Coding a different but equally rare diagnosis?<\/strong> <a href=\"https:\/\/pabau.com\/diagnostic-codes\/icd-10-code-h3554\/\">H35.54<\/a> walks through the same specificity requirements for a retinal condition.<\/p>\n                        <\/div>\n                                                                                <div class=\"item\">\n                            <div>\n                                <svg width=\"20\" height=\"27\" viewBox=\"0 0 20 27\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\">\n                                    <path\n                                        d=\"M18.6223 14.3857C19.1246 13.8975 19.1246 13.1045 18.6223 12.6162L12.1938 6.36621C11.6915 5.87793 10.8759 5.87793 10.3737 6.36621C9.87143 6.85449 9.87143 7.64746 10.3737 8.13574L14.6125 12.2529H2.28571C1.57455 12.2529 1 12.8115 1 13.5029C1 14.1943 1.57455 14.7529 2.28571 14.7529H14.6085L10.3777 18.8701C9.87545 19.3584 9.87545 20.1514 10.3777 20.6396C10.8799 21.1279 11.6955 21.1279 12.1978 20.6396L18.6263 14.3896L18.6223 14.3857Z\"\n                                        fill=\"#54B2D3\" \/>\n                                <\/svg>\n                            <\/div>\n                            <p><strong>Need a consent form ready for your practice?<\/strong> <a href=\"https:\/\/pabau.com\/templates\/dental-treatment-consent-form\/\">Dental treatment consent form<\/a> is a free template you can adapt for other documentation needs.<\/p>\n                        <\/div>\n                                                                                <div class=\"item\">\n                            <div>\n                                <svg width=\"20\" height=\"27\" viewBox=\"0 0 20 27\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\">\n                                    <path\n                                        d=\"M18.6223 14.3857C19.1246 13.8975 19.1246 13.1045 18.6223 12.6162L12.1938 6.36621C11.6915 5.87793 10.8759 5.87793 10.3737 6.36621C9.87143 6.85449 9.87143 7.64746 10.3737 8.13574L14.6125 12.2529H2.28571C1.57455 12.2529 1 12.8115 1 13.5029C1 14.1943 1.57455 14.7529 2.28571 14.7529H14.6085L10.3777 18.8701C9.87545 19.3584 9.87545 20.1514 10.3777 20.6396C10.8799 21.1279 11.6955 21.1279 12.1978 20.6396L18.6263 14.3896L18.6223 14.3857Z\"\n                                        fill=\"#54B2D3\" \/>\n                                <\/svg>\n                            <\/div>\n                            <p><strong>Billing for a related joint procedure?<\/strong> <a href=\"https:\/\/pabau.com\/procedure-codes\/cpt-code-20605\/\">20605<\/a> covers arthrocentesis billing for joint injections and aspirations.<\/p>\n                        <\/div>\n                                                <\/div>\n        <\/div>\n    \n\n\n<h2 id=\"h-frequently-asked-questions\" class=\"wp-block-heading\">Frequently asked questions<\/h2>\n\n\n\n<div class=\"schema-faq wp-block-yoast-faq-block\"><div class=\"schema-faq-section\" id=\"faq-question-1784874483389\"><h3 class=\"schema-faq-question\">What is ICD-10 Code M04.1?<\/h3> <p class=\"schema-faq-answer\">ICD-10 Code M04.1 is a billable, specific ICD-10-CM diagnosis code for periodic fever syndromes. It covers hereditary autoinflammatory diseases including familial Mediterranean fever (FMF), TNF receptor-associated periodic syndrome (TRAPS), and hyperimmunoglobin D syndrome \/ mevalonate kinase deficiency (HIDS\/MKD), all of which cause recurrent episodes of fever and systemic inflammation driven by innate immune dysregulation.<\/p> <\/div> <div class=\"schema-faq-section\" id=\"faq-question-1784874483390\"><h3 class=\"schema-faq-question\">Is ICD-10 Code M04.1 billable?<\/h3> <p class=\"schema-faq-answer\">Yes. M04.1 is a billable\/specific ICD-10-CM code and can be used as a primary or secondary diagnosis on claims. It is valid for the FY2026 edition, effective October 1, 2025. The parent code M04 is not billable and cannot be submitted on a claim.<\/p> <\/div> <div class=\"schema-faq-section\" id=\"faq-question-1784874483391\"><h3 class=\"schema-faq-question\">What conditions are included under ICD-10 Code M04.1?<\/h3> <p class=\"schema-faq-answer\">The Applicable To note for M04.1 includes three conditions: familial Mediterranean fever (FMF), TNF receptor-associated periodic syndrome (TRAPS), and hyperimmunoglobin D syndrome \/ mevalonate kinase deficiency (HIDS\/MKD, two names for one condition). CAPS is coded separately under M04.2, and PFAPA syndrome is coded under M04.8.<\/p> <\/div> <div class=\"schema-faq-section\" id=\"faq-question-1784874483392\"><h3 class=\"schema-faq-question\">What is the ICD-9-CM equivalent of M04.1?<\/h3> <p class=\"schema-faq-answer\">The primary ICD-9-CM crosswalk for M04.1 is code 277.31 (Familial Mediterranean fever). Because ICD-9-CM had limited specificity for autoinflammatory syndromes, the mapping is approximate. Verify against the CMS General Equivalence Mappings (GEMs) before using this crosswalk for historical claims reconciliation.<\/p> <\/div> <div class=\"schema-faq-section\" id=\"faq-question-1784874483393\"><h3 class=\"schema-faq-question\">What is the effective date for ICD-10-CM M04.1 in 2026?<\/h3> <p class=\"schema-faq-answer\">M04.1 became effective October 1, 2025 under the FY2026 edition of ICD-10-CM, and it remains active through September 30, 2026. The code description has not changed from the FY2025 edition.<\/p> <\/div> <div class=\"schema-faq-section\" id=\"faq-question-1784874483394\"><h3 class=\"schema-faq-question\">How do you distinguish M04.1 from other fever diagnosis codes?<\/h3> <p class=\"schema-faq-answer\">M04.1 applies only to confirmed hereditary autoinflammatory syndromes with documented genetic or clinical evidence. Nonspecific fever codes (such as R50.9 for fever unspecified) are appropriate when the cause is undetermined. Coders should not assign M04.1 based on symptom presentation alone. The clinical record must confirm the specific syndrome.<\/p> <\/div> <\/div>\n","protected":false},"excerpt":{"rendered":"<p>ICD-10 Code M04.1 is a billable, specific ICD-10-CM code for periodic fever syndromes, covering familial Mediterranean fever (FMF), TRAPS, and HIDS\/MKD. It&#8217;s the code to use in place of a generic fever code once one of these hereditary autoinflammatory diagnoses is confirmed, since payers validate medical necessity against the specific diagnosis on the claim. This [&hellip;]<\/p>\n","protected":false},"author":81,"featured_media":163322,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_yoast_wpseo_linkdex":"91","_yoast_wpseo_content_score":"60","_yoast_wpseo_is_cornerstone":"","_yoast_wpseo_keywordsynonyms":"[\"\",\"\",\"\",\"\"]","_yoast_wpseo_focuskw_text_input":"","_seo_original_html":"","footnotes":""},"categories":[1549,1550],"tags":[],"class_list":["post-163324","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-diagnostic-codes","category-icd-10-cm"],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v28.3 (Yoast SEO v28.3) - https:\/\/yoast.com\/product\/yoast-seo-premium-wordpress\/ -->\n<title>ICD-10 Code M04.1: Periodic fever syndromes (FY2026)<\/title>\n<meta name=\"description\" content=\"M04.1 is the billable ICD-10 code for periodic fever syndromes: FMF, TRAPS, and HIDS\/MKD, effective FY2026.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/\" \/>\n<meta property=\"og:locale\" content=\"nl_NL\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"ICD-10 Code M04.1: Periodic fever syndromes (FY2026)\" \/>\n<meta property=\"og:description\" content=\"M04.1 is the billable ICD-10 code for periodic fever syndromes: FMF, TRAPS, and HIDS\/MKD, effective FY2026.\" \/>\n<meta property=\"og:url\" content=\"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/\" \/>\n<meta property=\"og:site_name\" content=\"Pabau\" \/>\n<meta property=\"article:publisher\" content=\"https:\/\/www.facebook.com\/Pabau\/\" \/>\n<meta property=\"article:published_time\" content=\"2026-07-27T11:53:24+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2026-08-17T11:48:36+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/pabau.com\/wp-content\/uploads\/2026\/07\/icd-10-code-m041.webp\" \/>\n\t<meta property=\"og:image:width\" content=\"1200\" \/>\n\t<meta property=\"og:image:height\" content=\"630\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/webp\" \/>\n<meta name=\"author\" content=\"Aleksandar Kochovski\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:creator\" content=\"@pabaucrm\" \/>\n<meta name=\"twitter:site\" content=\"@pabaucrm\" \/>\n<meta name=\"twitter:label1\" content=\"Geschreven door\" \/>\n\t<meta name=\"twitter:data1\" content=\"Aleksandar Kochovski\" \/>\n\t<meta name=\"twitter:label2\" content=\"Geschatte leestijd\" \/>\n\t<meta name=\"twitter:data2\" content=\"11 minuten\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#article\",\"isPartOf\":{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/\"},\"author\":{\"name\":\"Aleksandar Kochovski\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/#\\\/schema\\\/person\\\/73b672a49e20bfba8161e3c17ee3dda2\"},\"headline\":\"ICD-10 Code M04.1: Periodic fever syndromes (FY2026)\",\"datePublished\":\"2026-07-27T11:53:24+00:00\",\"dateModified\":\"2026-08-17T11:48:36+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/\"},\"wordCount\":2252,\"publisher\":{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/#organization\"},\"image\":{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#primaryimage\"},\"thumbnailUrl\":\"https:\\\/\\\/pabau.com\\\/wp-content\\\/uploads\\\/2026\\\/07\\\/icd-10-code-m041.webp\",\"articleSection\":[\"Diagnostic Codes\",\"ICD-10-CM\"],\"inLanguage\":\"nl-NL\"},{\"@type\":[\"WebPage\",\"FAQPage\"],\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/\",\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/\",\"name\":\"ICD-10 Code M04.1: Periodic fever syndromes (FY2026)\",\"isPartOf\":{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/#website\"},\"primaryImageOfPage\":{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#primaryimage\"},\"image\":{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#primaryimage\"},\"thumbnailUrl\":\"https:\\\/\\\/pabau.com\\\/wp-content\\\/uploads\\\/2026\\\/07\\\/icd-10-code-m041.webp\",\"datePublished\":\"2026-07-27T11:53:24+00:00\",\"dateModified\":\"2026-08-17T11:48:36+00:00\",\"description\":\"M04.1 is the billable ICD-10 code for periodic fever syndromes: FMF, TRAPS, and HIDS\\\/MKD, effective FY2026.\",\"mainEntity\":[{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483389\"},{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483390\"},{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483391\"},{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483392\"},{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483393\"},{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483394\"}],\"inLanguage\":\"nl-NL\",\"potentialAction\":[{\"@type\":\"ReadAction\",\"target\":[\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/\"]}]},{\"@type\":\"ImageObject\",\"inLanguage\":\"nl-NL\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#primaryimage\",\"url\":\"https:\\\/\\\/pabau.com\\\/wp-content\\\/uploads\\\/2026\\\/07\\\/icd-10-code-m041.webp\",\"contentUrl\":\"https:\\\/\\\/pabau.com\\\/wp-content\\\/uploads\\\/2026\\\/07\\\/icd-10-code-m041.webp\",\"width\":1200,\"height\":630},{\"@type\":\"WebSite\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/#website\",\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/\",\"name\":\"Pabau\",\"description\":\"Clinic Software for your Business | Go Paperless Today\",\"publisher\":{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/#organization\"},\"potentialAction\":[{\"@type\":\"SearchAction\",\"target\":{\"@type\":\"EntryPoint\",\"urlTemplate\":\"https:\\\/\\\/pabau.com\\\/nl\\\/?s={search_term_string}\"},\"query-input\":{\"@type\":\"PropertyValueSpecification\",\"valueRequired\":true,\"valueName\":\"search_term_string\"}}],\"inLanguage\":\"nl-NL\"},{\"@type\":\"Organization\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/#organization\",\"name\":\"Pabau\",\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/\",\"logo\":{\"@type\":\"ImageObject\",\"inLanguage\":\"nl-NL\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/#\\\/schema\\\/logo\\\/image\\\/\",\"url\":\"https:\\\/\\\/pabau.com\\\/wp-content\\\/uploads\\\/2022\\\/05\\\/Logo-4-e1654525062364.png\",\"contentUrl\":\"https:\\\/\\\/pabau.com\\\/wp-content\\\/uploads\\\/2022\\\/05\\\/Logo-4-e1654525062364.png\",\"width\":736,\"height\":195,\"caption\":\"Pabau\"},\"image\":{\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/#\\\/schema\\\/logo\\\/image\\\/\"},\"sameAs\":[\"https:\\\/\\\/www.facebook.com\\\/Pabau\\\/\",\"https:\\\/\\\/x.com\\\/pabaucrm\",\"https:\\\/\\\/www.linkedin.com\\\/company\\\/pabau-ltd\\\/\",\"https:\\\/\\\/www.instagram.com\\\/pabausoftware\\\/\"],\"description\":\"Pabau is an all-in-one healthcare practice management software platform designed for clinics, medspas, therapists, and medical professionals. It combines scheduling, electronic health records, clinical forms, payments, marketing automation, reporting, and patient engagement tools into one secure cloud system to help practices streamline operations and grow their business.\",\"foundingDate\":\"2011-10-20\",\"numberOfEmployees\":{\"@type\":\"QuantitativeValue\",\"minValue\":\"201\",\"maxValue\":\"500\"}},{\"@type\":\"Person\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/#\\\/schema\\\/person\\\/73b672a49e20bfba8161e3c17ee3dda2\",\"name\":\"Aleksandar Kochovski\",\"image\":{\"@type\":\"ImageObject\",\"inLanguage\":\"nl-NL\",\"@id\":\"https:\\\/\\\/pabau.com\\\/wp-content\\\/uploads\\\/2026\\\/06\\\/cropped-aleksandar-kochovski-profile-picture-96x96.jpeg\",\"url\":\"https:\\\/\\\/pabau.com\\\/wp-content\\\/uploads\\\/2026\\\/06\\\/cropped-aleksandar-kochovski-profile-picture-96x96.jpeg\",\"contentUrl\":\"https:\\\/\\\/pabau.com\\\/wp-content\\\/uploads\\\/2026\\\/06\\\/cropped-aleksandar-kochovski-profile-picture-96x96.jpeg\",\"caption\":\"Aleksandar Kochovski\"},\"description\":\"Aleksandar is a content strategist and editor working in tech publishing, with a background in higher education. He specializes in topics around compliance in software solutions. When he's not writing at Pabau, you can find him tending to his bonsai collection or hitting the gym.\",\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/blog\\\/author\\\/aleksandar-kochovski\\\/\"},{\"@type\":\"Question\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483389\",\"position\":1,\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483389\",\"name\":\"What is ICD-10 Code M04.1?\",\"answerCount\":1,\"acceptedAnswer\":{\"@type\":\"Answer\",\"text\":\"ICD-10 Code M04.1 is a billable, specific ICD-10-CM diagnosis code for periodic fever syndromes. It covers hereditary autoinflammatory diseases including familial Mediterranean fever (FMF), TNF receptor-associated periodic syndrome (TRAPS), and hyperimmunoglobin D syndrome \\\/ mevalonate kinase deficiency (HIDS\\\/MKD), all of which cause recurrent episodes of fever and systemic inflammation driven by innate immune dysregulation.\",\"inLanguage\":\"nl-NL\"},\"inLanguage\":\"nl-NL\"},{\"@type\":\"Question\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483390\",\"position\":2,\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483390\",\"name\":\"Is ICD-10 Code M04.1 billable?\",\"answerCount\":1,\"acceptedAnswer\":{\"@type\":\"Answer\",\"text\":\"Yes. M04.1 is a billable\\\/specific ICD-10-CM code and can be used as a primary or secondary diagnosis on claims. It is valid for the FY2026 edition, effective October 1, 2025. The parent code M04 is not billable and cannot be submitted on a claim.\",\"inLanguage\":\"nl-NL\"},\"inLanguage\":\"nl-NL\"},{\"@type\":\"Question\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483391\",\"position\":3,\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483391\",\"name\":\"What conditions are included under ICD-10 Code M04.1?\",\"answerCount\":1,\"acceptedAnswer\":{\"@type\":\"Answer\",\"text\":\"The Applicable To note for M04.1 includes three conditions: familial Mediterranean fever (FMF), TNF receptor-associated periodic syndrome (TRAPS), and hyperimmunoglobin D syndrome \\\/ mevalonate kinase deficiency (HIDS\\\/MKD, two names for one condition). CAPS is coded separately under M04.2, and PFAPA syndrome is coded under M04.8.\",\"inLanguage\":\"nl-NL\"},\"inLanguage\":\"nl-NL\"},{\"@type\":\"Question\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483392\",\"position\":4,\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483392\",\"name\":\"What is the ICD-9-CM equivalent of M04.1?\",\"answerCount\":1,\"acceptedAnswer\":{\"@type\":\"Answer\",\"text\":\"The primary ICD-9-CM crosswalk for M04.1 is code 277.31 (Familial Mediterranean fever). Because ICD-9-CM had limited specificity for autoinflammatory syndromes, the mapping is approximate. Verify against the CMS General Equivalence Mappings (GEMs) before using this crosswalk for historical claims reconciliation.\",\"inLanguage\":\"nl-NL\"},\"inLanguage\":\"nl-NL\"},{\"@type\":\"Question\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483393\",\"position\":5,\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483393\",\"name\":\"What is the effective date for ICD-10-CM M04.1 in 2026?\",\"answerCount\":1,\"acceptedAnswer\":{\"@type\":\"Answer\",\"text\":\"M04.1 became effective October 1, 2025 under the FY2026 edition of ICD-10-CM, and it remains active through September 30, 2026. The code description has not changed from the FY2025 edition.\",\"inLanguage\":\"nl-NL\"},\"inLanguage\":\"nl-NL\"},{\"@type\":\"Question\",\"@id\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483394\",\"position\":6,\"url\":\"https:\\\/\\\/pabau.com\\\/nl\\\/diagnostic-codes\\\/icd-10-code-m041\\\/#faq-question-1784874483394\",\"name\":\"How do you distinguish M04.1 from other fever diagnosis codes?\",\"answerCount\":1,\"acceptedAnswer\":{\"@type\":\"Answer\",\"text\":\"M04.1 applies only to confirmed hereditary autoinflammatory syndromes with documented genetic or clinical evidence. Nonspecific fever codes (such as R50.9 for fever unspecified) are appropriate when the cause is undetermined. Coders should not assign M04.1 based on symptom presentation alone. The clinical record must confirm the specific syndrome.\",\"inLanguage\":\"nl-NL\"},\"inLanguage\":\"nl-NL\"}]}<\/script>\n<!-- \/ Yoast SEO Premium plugin. -->","yoast_head_json":{"title":"ICD-10 Code M04.1: Periodic fever syndromes (FY2026)","description":"M04.1 is the billable ICD-10 code for periodic fever syndromes: FMF, TRAPS, and HIDS\/MKD, effective FY2026.","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/","og_locale":"nl_NL","og_type":"article","og_title":"ICD-10 Code M04.1: Periodic fever syndromes (FY2026)","og_description":"M04.1 is the billable ICD-10 code for periodic fever syndromes: FMF, TRAPS, and HIDS\/MKD, effective FY2026.","og_url":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/","og_site_name":"Pabau","article_publisher":"https:\/\/www.facebook.com\/Pabau\/","article_published_time":"2026-07-27T11:53:24+00:00","article_modified_time":"2026-08-17T11:48:36+00:00","og_image":[{"width":1200,"height":630,"url":"https:\/\/pabau.com\/wp-content\/uploads\/2026\/07\/icd-10-code-m041.webp","type":"image\/webp"}],"author":"Aleksandar Kochovski","twitter_card":"summary_large_image","twitter_creator":"@pabaucrm","twitter_site":"@pabaucrm","twitter_misc":{"Geschreven door":"Aleksandar Kochovski","Geschatte leestijd":"11 minuten"},"schema":{"@context":"https:\/\/schema.org","@graph":[{"@type":"Article","@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#article","isPartOf":{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/"},"author":{"name":"Aleksandar Kochovski","@id":"https:\/\/pabau.com\/nl\/#\/schema\/person\/73b672a49e20bfba8161e3c17ee3dda2"},"headline":"ICD-10 Code M04.1: Periodic fever syndromes (FY2026)","datePublished":"2026-07-27T11:53:24+00:00","dateModified":"2026-08-17T11:48:36+00:00","mainEntityOfPage":{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/"},"wordCount":2252,"publisher":{"@id":"https:\/\/pabau.com\/nl\/#organization"},"image":{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#primaryimage"},"thumbnailUrl":"https:\/\/pabau.com\/wp-content\/uploads\/2026\/07\/icd-10-code-m041.webp","articleSection":["Diagnostic Codes","ICD-10-CM"],"inLanguage":"nl-NL"},{"@type":["WebPage","FAQPage"],"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/","url":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/","name":"ICD-10 Code M04.1: Periodic fever syndromes (FY2026)","isPartOf":{"@id":"https:\/\/pabau.com\/nl\/#website"},"primaryImageOfPage":{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#primaryimage"},"image":{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#primaryimage"},"thumbnailUrl":"https:\/\/pabau.com\/wp-content\/uploads\/2026\/07\/icd-10-code-m041.webp","datePublished":"2026-07-27T11:53:24+00:00","dateModified":"2026-08-17T11:48:36+00:00","description":"M04.1 is the billable ICD-10 code for periodic fever syndromes: FMF, TRAPS, and HIDS\/MKD, effective FY2026.","mainEntity":[{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483389"},{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483390"},{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483391"},{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483392"},{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483393"},{"@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483394"}],"inLanguage":"nl-NL","potentialAction":[{"@type":"ReadAction","target":["https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/"]}]},{"@type":"ImageObject","inLanguage":"nl-NL","@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#primaryimage","url":"https:\/\/pabau.com\/wp-content\/uploads\/2026\/07\/icd-10-code-m041.webp","contentUrl":"https:\/\/pabau.com\/wp-content\/uploads\/2026\/07\/icd-10-code-m041.webp","width":1200,"height":630},{"@type":"WebSite","@id":"https:\/\/pabau.com\/nl\/#website","url":"https:\/\/pabau.com\/nl\/","name":"Pabau","description":"Clinic Software for your Business | Go Paperless Today","publisher":{"@id":"https:\/\/pabau.com\/nl\/#organization"},"potentialAction":[{"@type":"SearchAction","target":{"@type":"EntryPoint","urlTemplate":"https:\/\/pabau.com\/nl\/?s={search_term_string}"},"query-input":{"@type":"PropertyValueSpecification","valueRequired":true,"valueName":"search_term_string"}}],"inLanguage":"nl-NL"},{"@type":"Organization","@id":"https:\/\/pabau.com\/nl\/#organization","name":"Pabau","url":"https:\/\/pabau.com\/nl\/","logo":{"@type":"ImageObject","inLanguage":"nl-NL","@id":"https:\/\/pabau.com\/nl\/#\/schema\/logo\/image\/","url":"https:\/\/pabau.com\/wp-content\/uploads\/2022\/05\/Logo-4-e1654525062364.png","contentUrl":"https:\/\/pabau.com\/wp-content\/uploads\/2022\/05\/Logo-4-e1654525062364.png","width":736,"height":195,"caption":"Pabau"},"image":{"@id":"https:\/\/pabau.com\/nl\/#\/schema\/logo\/image\/"},"sameAs":["https:\/\/www.facebook.com\/Pabau\/","https:\/\/x.com\/pabaucrm","https:\/\/www.linkedin.com\/company\/pabau-ltd\/","https:\/\/www.instagram.com\/pabausoftware\/"],"description":"Pabau is an all-in-one healthcare practice management software platform designed for clinics, medspas, therapists, and medical professionals. It combines scheduling, electronic health records, clinical forms, payments, marketing automation, reporting, and patient engagement tools into one secure cloud system to help practices streamline operations and grow their business.","foundingDate":"2011-10-20","numberOfEmployees":{"@type":"QuantitativeValue","minValue":"201","maxValue":"500"}},{"@type":"Person","@id":"https:\/\/pabau.com\/nl\/#\/schema\/person\/73b672a49e20bfba8161e3c17ee3dda2","name":"Aleksandar Kochovski","image":{"@type":"ImageObject","inLanguage":"nl-NL","@id":"https:\/\/pabau.com\/wp-content\/uploads\/2026\/06\/cropped-aleksandar-kochovski-profile-picture-96x96.jpeg","url":"https:\/\/pabau.com\/wp-content\/uploads\/2026\/06\/cropped-aleksandar-kochovski-profile-picture-96x96.jpeg","contentUrl":"https:\/\/pabau.com\/wp-content\/uploads\/2026\/06\/cropped-aleksandar-kochovski-profile-picture-96x96.jpeg","caption":"Aleksandar Kochovski"},"description":"Aleksandar is a content strategist and editor working in tech publishing, with a background in higher education. He specializes in topics around compliance in software solutions. When he's not writing at Pabau, you can find him tending to his bonsai collection or hitting the gym.","url":"https:\/\/pabau.com\/nl\/blog\/author\/aleksandar-kochovski\/"},{"@type":"Question","@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483389","position":1,"url":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483389","name":"What is ICD-10 Code M04.1?","answerCount":1,"acceptedAnswer":{"@type":"Answer","text":"ICD-10 Code M04.1 is a billable, specific ICD-10-CM diagnosis code for periodic fever syndromes. It covers hereditary autoinflammatory diseases including familial Mediterranean fever (FMF), TNF receptor-associated periodic syndrome (TRAPS), and hyperimmunoglobin D syndrome \/ mevalonate kinase deficiency (HIDS\/MKD), all of which cause recurrent episodes of fever and systemic inflammation driven by innate immune dysregulation.","inLanguage":"nl-NL"},"inLanguage":"nl-NL"},{"@type":"Question","@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483390","position":2,"url":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483390","name":"Is ICD-10 Code M04.1 billable?","answerCount":1,"acceptedAnswer":{"@type":"Answer","text":"Yes. M04.1 is a billable\/specific ICD-10-CM code and can be used as a primary or secondary diagnosis on claims. It is valid for the FY2026 edition, effective October 1, 2025. The parent code M04 is not billable and cannot be submitted on a claim.","inLanguage":"nl-NL"},"inLanguage":"nl-NL"},{"@type":"Question","@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483391","position":3,"url":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483391","name":"What conditions are included under ICD-10 Code M04.1?","answerCount":1,"acceptedAnswer":{"@type":"Answer","text":"The Applicable To note for M04.1 includes three conditions: familial Mediterranean fever (FMF), TNF receptor-associated periodic syndrome (TRAPS), and hyperimmunoglobin D syndrome \/ mevalonate kinase deficiency (HIDS\/MKD, two names for one condition). CAPS is coded separately under M04.2, and PFAPA syndrome is coded under M04.8.","inLanguage":"nl-NL"},"inLanguage":"nl-NL"},{"@type":"Question","@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483392","position":4,"url":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483392","name":"What is the ICD-9-CM equivalent of M04.1?","answerCount":1,"acceptedAnswer":{"@type":"Answer","text":"The primary ICD-9-CM crosswalk for M04.1 is code 277.31 (Familial Mediterranean fever). Because ICD-9-CM had limited specificity for autoinflammatory syndromes, the mapping is approximate. Verify against the CMS General Equivalence Mappings (GEMs) before using this crosswalk for historical claims reconciliation.","inLanguage":"nl-NL"},"inLanguage":"nl-NL"},{"@type":"Question","@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483393","position":5,"url":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483393","name":"What is the effective date for ICD-10-CM M04.1 in 2026?","answerCount":1,"acceptedAnswer":{"@type":"Answer","text":"M04.1 became effective October 1, 2025 under the FY2026 edition of ICD-10-CM, and it remains active through September 30, 2026. The code description has not changed from the FY2025 edition.","inLanguage":"nl-NL"},"inLanguage":"nl-NL"},{"@type":"Question","@id":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483394","position":6,"url":"https:\/\/pabau.com\/nl\/diagnostic-codes\/icd-10-code-m041\/#faq-question-1784874483394","name":"How do you distinguish M04.1 from other fever diagnosis codes?","answerCount":1,"acceptedAnswer":{"@type":"Answer","text":"M04.1 applies only to confirmed hereditary autoinflammatory syndromes with documented genetic or clinical evidence. Nonspecific fever codes (such as R50.9 for fever unspecified) are appropriate when the cause is undetermined. Coders should not assign M04.1 based on symptom presentation alone. The clinical record must confirm the specific syndrome.","inLanguage":"nl-NL"},"inLanguage":"nl-NL"}]}},"yoast":{"focus_keyword":"ICD-10 Code M04.1","seo_title":"ICD-10 Code M04.1: Periodic fever syndromes (FY2026)","meta_description":"M04.1 is the billable ICD-10 code for periodic fever syndromes: FMF, TRAPS, and HIDS\/MKD, effective FY2026.","content_score":"60","is_cornerstone":"","related_keyphrases":[{"keyword":"autoinflammatory syndromes ICD-10","score":67},{"keyword":"familial Mediterranean fever ICD-10","score":67},{"keyword":"hereditary periodic fever ICD-10","score":61}]},"_links":{"self":[{"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/posts\/163324","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/users\/81"}],"replies":[{"embeddable":true,"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/comments?post=163324"}],"version-history":[{"count":5,"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/posts\/163324\/revisions"}],"predecessor-version":[{"id":182180,"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/posts\/163324\/revisions\/182180"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/media\/163322"}],"wp:attachment":[{"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/media?parent=163324"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/categories?post=163324"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/pabau.com\/nl\/wp-json\/wp\/v2\/tags?post=163324"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}