Table 1:

Manifestations of IgG4-related disease in different organ systems

Organ systemCommon clinical presentationPreferred name (3)
PancreasPainless obstructive jaundice and endocrine failure (secondary diabetes mellitus); frequently associated with IgG4-related sclerosing cholangitis (4)Type 1 autoimmune pancreatitis*
Lacrimal and salivary glandsMikulicz disease: (5) bilateral dacryoadenitis, and enlargement of the parotid and submandibular glands with associated xerophthalmia and xerostomia
Küttner tumour/chronic sclerosing sialadenitis: (6) hard indurated masses of submandibular (or parotid) glands; associated xerostomia is common
IgG4-related dacryoadenitis and IgG4-related sialadenitis
OrbitsProptosis due to dacryoadenitis, local myositis or orbital pseudotumours; scleritis, uveitis and locoregional neuronal damage can occur from mass effect (7), (8)IgG4-related ophthalmic disease
LungsDyspnea, wheeze, cough
Mild stridulous symptoms can be associated with upper respiratory tract (e.g., pharynx, trachea) inflammation; often diagnosed incidentally on imaging (9)
IgG4-related lung disease
Thyroid glandRiedel thyroiditis: (10) stony goiter — usually euthyroid or subclinical hypothyroid profileIgG4-related thyroid disease
Lymph nodesNontender generalized lymphadenopathy or localized disease near other affected organs; often asymptomatic and diagnosed incidentally on imagingIgG4-related lymphadenopathy
Arterial systemAortitis of either the thoracic or abdominal aorta; aneurysmal disease can present with pain or vascular insufficiency but can also be acute and catastrophic. (11) Coronary arteritis may be associated with ischemic heart disease (12)IgG4-related aortitis or periaortitis
RetroperitoneumOrmond disease: poorly localized lower back pain and chronic renal failure Ureteric obstruction, hydronephrosis and renal failureIgG4-related retroperitoneal fibrosis
KidneysDiffuse renal enlargement and chronic renal failure; (13) commonly, tubulointerstitial nephritis associated with a profound hypocomplementemia
Proteinuria due to coexistent glomerulonephritis (membranous) is common
IgG4-related kidney disease
Biliary treeJaundice, weight loss and abdominal pain that closely mimicks PSC or cholangiocarcinoma; associated cholecystitis is usually asymptomaticIgG4-related sclerosing cholangitis
MeningesHeadache, nerve palsies and radiculomyelopathyIgG4-related pachymeningitis
  • Note: PSC = primary sclerosing cholangitis. Other involvement includes IgG4-related skin disease, prostatitis, mastitis, mesenteritis, mediastinitis and hypophysitis.

  • * Should not be confused with type 2 autoimmune pancreatitis. Type 2 autoimmune pancreatitis is also called idiopathic duct centric pancreatitis and is not related to IgG4-RD, affects younger patients with no sex preponderance, (14) is less common than type 1 but is also treated with steroids.