Pancreas | Painless obstructive jaundice and endocrine failure (secondary diabetes mellitus); frequently associated with IgG4-related sclerosing cholangitis (4) | Type 1 autoimmune pancreatitis* |
Lacrimal and salivary glands | Mikulicz 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 |
Orbits | Proptosis 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 |
Lungs | Dyspnea, 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 gland | Riedel thyroiditis: (10) stony goiter — usually euthyroid or subclinical hypothyroid profile | IgG4-related thyroid disease |
Lymph nodes | Nontender generalized lymphadenopathy or localized disease near other affected organs; often asymptomatic and diagnosed incidentally on imaging | IgG4-related lymphadenopathy |
Arterial system | Aortitis 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 |
Retroperitoneum | Ormond disease: poorly localized lower back pain and chronic renal failure Ureteric obstruction, hydronephrosis and renal failure | IgG4-related retroperitoneal fibrosis |
Kidneys | Diffuse 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 tree | Jaundice, weight loss and abdominal pain that closely mimicks PSC or cholangiocarcinoma; associated cholecystitis is usually asymptomatic | IgG4-related sclerosing cholangitis |
Meninges | Headache, nerve palsies and radiculomyelopathy | IgG4-related pachymeningitis |