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

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
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
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
IgG4-related coronary artery disease
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
Other manifestations
IgG4-related fibrosing mediastinitis
IgG4-related myositis
Multifocal fibrosclerosis
Increased risk of malignancy: lung, colon and especially MALT lymphoma.