Table 1.
Organs affected by IgG4-related systemic disease
| Organ or Site | Clinicopathologic Features | References |
|---|---|---|
| Pancreas | Lymphoplasmacytic sclerosing pancreatitis (type 1 autoimmune pancreatitis) Idiopathic, duct-centric, chronic pancreatitis or granular epithelial lesion (type 2 autoimmune pancreatitis) | 10, 12, 13,16, 17, 21,27, 38, 41, 49, 50 |
| Bile duct | Sclerosing cholangitis or IgG4-associated cholangitis Inflammatory pseudotumor | 46, 53, 54, 55, 56 |
| Liver | Sclerosing cholangitis involving intrahepatic ducts Portal inflammation, with or without interface hepatitis Large bile-duct obstruction Portal sclerosis Lobular hepatitis Canalicular cholestasis Inflammatory pseudotumor | 35, 60-64 |
| Gallbladder | Diffuse, acalculous, lymphoplasmacytic cholecystitis | 65-68 |
| Gastrointestinal tract | Increased IgG4-positive cells in mucosa Inflammatory bowel disease | 13, 18, 23, 52 |
| Salivary and lacrimal glands | Küttner tumor (chronic sclerosing sialadenitis) Mikulicz disease Chronic, sclerosing dacryoadenitis | 69-74 |
| Kidney | Tubulointerstitial nephritis Membranous glomerulopathy, with IgG4 immune complex deposits in tubular basement membrane | 78-84 |
| Retroperitoneum and mesentery | Retroperitoneal fibrosis Sclerosing mesenteritis | 85-91 |
| Thyroid | HypothyroidismRiedel thyroiditis | 92-94 |
| Breast | Inflammatory pseudotumor | 36 |
| Lung | Interstitial pneumonia Inflammatory pseudotumor | 97-101 |
| Aorta | Inflammatory abdominal aortic aneurysm | 31, 32 |
| Orbit | Inflammatory pseudotumor | 30 |
| Mediastinum | Sclerosing mediastinitis | 29 |
| Pituitary gland | Hypophysitis Inflammatory pseudotumor | 33 |
| Prostate | IgG4-associated prostatitis | 34 |
| Lymph nodes | Castleman disease-like lymphadenopathy Lymphadenopathy with follicular hyperplasia Lymphadenopathy with interfollicular expansion by immunoblasts and plasma cells | 100-103 |