Secondary systemic amyloidosis

This code identifies a systemic disorder characterized by the extracellular deposition of abnormal amyloid proteins in various organs, occurring as a complication of another chronic inflammatory or infectious disease. Unlike primary amyloidosis, secondary amyloidosis (AA amyloidosis) is directly linked to an underlying condition that causes prolonged inflammation. The amyloid deposits can impair organ function, leading to symptoms specific to the affected organs.

Use this code when documentation clearly indicates systemic amyloidosis that has developed as a consequence of a chronic inflammatory process, such as rheumatoid arthritis, inflammatory bowel disease, or chronic infections. The medical record should specify the secondary nature of the amyloidosis and ideally identify the underlying causative condition.

• Always code the underlying chronic inflammatory or infectious disease first, as it is the etiology of the secondary amyloidosis.
• Do not confuse this with primary amyloidosis (E85.0-E85.2, E85.8-E85.9), which is not associated with an underlying chronic inflammatory condition.
• Code any specific organ manifestations or complications of the amyloidosis (e.g., nephropathy, cardiomyopathy) in addition to this code.