AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2023 Issue 4; New/Revised ICD-10-CM Codes

Immunoglobulin G4-Related Disease

A new code was created to identify immunoglobulin (Ig) G4-related disease (D89.84). Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibroinflammatory immune-mediated condition of unknown origin that is comprised of a collection of disorders that share specific serological, pathological, and clinical features. IgG4-RD has a propensity for tumor formation with IgG4 rich plasma cells and elevation of serum IgG4. If not diagnosed, IgG4-RD can lead to impaired organ function. Any organ or tissue may be affected. For example, IgG4-related ophthalmic disease may present as myositis, dacryoadenitis, or involve other orbital tissue while cranial neuropathy may manifest as pachymeningitis...

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