Autoinflammatory syndromes (M04)
The M04 ICD-10 codes cover a group of conditions known as autoinflammatory syndromes, which are disorders characterized by episodes of inflammation without the usual triggers like infections or autoimmunity. These codes classify specific syndromes involving periodic fevers, cold-induced symptoms, and other complex inflammatory disorders.
Within this range, M04.1 refers to periodic fever syndromes, also known colloquially as familial Mediterranean fever, PFAPA syndrome, or TNF receptor-associated periodic fever syndrome (TRAPS). These conditions feature recurrent fever episodes and inflammation. Code M04.2 covers cryopyrin-associated periodic syndromes, including familial cold urticaria and chronic infantile neurological, cutaneous, and articular syndrome, where symptoms are triggered by cold exposure. The M04.8 category includes other autoinflammatory syndromes such as Blau syndrome, VEXAS syndrome, and Majeed syndrome, encompassing diverse hereditary and monogenic inflammatory disorders. Lastly, M04.9 is used for unspecified autoinflammatory syndromes when a precise diagnosis within these categories is not established. These codes help clearly identify and track various autoinflammatory diseases for accurate diagnosis and management.
Instructional Notations
Type 2 Excludes
A type 2 excludes note represents "Not included here". An excludes2 note indicates that the condition excluded is not part of the condition represented by the code, but a patient may have both conditions at the same time. When an Excludes2 note appears under a code, it is acceptable to use both the code and the excluded code together, when appropriate.
- Crohn's disease K50
Clinical Terms
The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.
Familial Mediterranean Fever
A group of HEREDITARY AUTOINFLAMMATION DISEASES, characterized by recurrent fever, abdominal pain, headache, rash, PLEURISY; and ARTHRITIS. ORCHITIS; benign MENINGITIS; and AMYLOIDOSIS may also occur. Homozygous or compound heterozygous mutations in marenostrin gene encoding PYRIN result in autosomal recessive transmission; simple heterozygous, autosomal dominant form of the disease also exists with mutations in the same gene.
Macrophage Activation Syndrome
A serious complication of childhood systemic inflammatory disorders that is thought to be caused by excessive activation and proliferation of T-LYMPHOCYTES and MACROPHAGES. It is seen predominantly in children with systemic onset JUVENILE IDIOPATHIC ARTHRITIS.
