IgG4 related disease

Description

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.

Index terms

IgG4 related disease
IgG4-related retroperitoneal fibrosis
IgG4-related mediastinitis

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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

Exclusions from above levels

02 – Neoplasms
18 – Complications of pregnancy, childbirth and the puerperium
20 – Developmental anomalies

Foundation children elsewhere

EK91.2 – Primary cutaneous plasmacytosis

Sibling codes

4A43.1 – Mikulicz disease
4A43.2 – Sjögren syndrome
4A43.3 – Mixed connective tissue disease
4A43.4 – Diffuse eosinophilic fasciitis
4A43.Y – Other specified overlap non-organ specific systemic autoimmune disease
4A43.Z – Undifferentiated non-organ specific systemic autoimmune disease

Foundation entity

Allowed postcoordination axes

hasManifestation

Part of: 4A43: Overlap or undifferentiated nonorgan specific systemic autoimmune disease › 04: Diseases of the immune system