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. |