4A43.0 IgG4 related disease

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2025-01

IgG4 related syndrome (IgG4-related disease: IgG4-RD) is a clinical disease characterised by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. The diagnostic criteria for IgG4 related syndrome have been proposed, and it may be present in a certain population of patients with a wide variety of diseases, including Mikulicz disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudo tumour.

code elsewhere

• Primary cutaneous plasmacytosis  (EK91.2)
• Benign dermal lymphocytic or lymphoplasmacytic infiltrations or proliferations  (EE90-EE91)
• Type 1 IgG4 related autoimmune pancreatitis  (DC33)

postcoordination

synonyms

• IgG4 related disease
• IgG4-related retroperitoneal fibrosis
• IgG4-related mediastinitis
• IgG4-related thyroid disease
• IgG4-related dacryoadenitis and sialadenitis
• IgG4-related hepatopathy
• IgG4-related mesenteritis
• IgG4-related kidney disease
• IgG4-related aortitis
• IgG4-related pachymeningitis
• IgG4-related submandibular gland disease
• IgG4-related ophthalmic disease
• Eosinophilic angiocentric fibrosis
• Cutaneous pseudolymphoma
• IgG4-related sclerosing cholangitis
• Cholangitis of IgG4-related sclerosing disease

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