Manifestations of IgG4-related disease in different organ systems
| Organ system | Common clinical presentation | Preferred name3 |
|---|---|---|
| Pancreas | Painless obstructive jaundice and endocrine failure (secondary diabetes mellitus); frequently associated with IgG4-related sclerosing cholangitis4 | 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 effect7,8 | 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 imaging9 | 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 disease12 | 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 |
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.