Skip to main content


Table 2 Organ manifestations of IgG4-related disease

From: IgG4-related disease: a systematic review of this unrecognized disease in pediatrics

Pancreas Autoimmune pancreatitis type 1 Lymph nodes Ig4-related lymphadenopathy of several lymph nodes
Liver and bile duct IgG4-related sclerosing cholangitis IgG4-related cholecystitis IgG4-related hepatopathy Other abdominal manifestations Inflammatory pseudotumors Retroperitoneal fibrosis Small bowel obstruction caused by peritoneal IgG4-RD IgG4-RD of stomach with chronic ulcer IgG4-related esophagitis
Kidneys Interstitial nephritis Glomerular lesions such as membranous nephropathy Skin manifestation Erythematous, subcutaneous papules or nodules of IgG4 origin
Urological manifestation IgG4-related prostatitis Ureteral IgG4-RD Testicular inflammation as a manifestation of IgG4-RD Orbital and ophthalmic manifestation Inflammatory pseudotumors of orbit Scleritis Retinopathy due to IgG4-RD with hypergammaglobulinemic hyperviscosity Trigeminal and orbital nerve compression Nasolacrimal duct obstruction
Pulmonary manifestation Interstitial lung disease/interstitial pneumonia Bronchial damage/asthma-like clinical presentation Plural manifestation of IgG4-disease Pulmonary arterial hypertension Cardiovascular manifestation IgG4-related periaortitis IgG4-related aortitis Pericarditis IgG4-related coronary artery disease
Thyroid Riedel’s thyroiditis Fibrosing Hashimoto thyroiditis Salivary and lacrimal gland IgG4-RD Mikulicz’s Küttner's tumor or IgG4-related submandibular gland disease
Nervous system Infundibular hypophyistis Hypertrophic pachymeningitis IgG4-related hypophysitis Intracerebral inflammatory pseudotumors Neuropathy Other manifestations IgG4-related fibrosing mediastinitis IgG4-related myositis Multifocal fibrosclerosis Increased risk of malignancy: lung, colon and especially MALT lymphoma.