AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2016 Issue 4; ICD-10-CM NEW/REVISED CODES

Autoinflammatory Syndromes

Category M04, Autoinflammatory syndromes, has been created and new codes were developed to uniquely identify periodic fever syndromes (M04.1), cryopyrin-associated periodic syndromes (M04.2), other autoinflammatory syndromes (M04.8), and autoinflammatory syndrome, unspecified (M04.9).  Cryopyrin is an important mediator of inflammation, via activating interleukin 1 (IL-1). Excessive activation of IL-1 can lead to an inflammatory response, which can be harmful. This is the key to the inflammation in cryopyrin-associated periodic syndromes (CAPS), as well as certain other autoinflammatory disorders. CAPS can cause end organ damage due to chronic inflammation. Some patients with CAPS may develop hearing...

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