Table 1 Common manifestations of IgG4-RD by organ system
| Gastrointestinal |
Autoimmune pancreatitis Sclerosing cholangitis Sclerosing mesenteritis |
| Head and neck |
Eosinophilic angiocentric fibrosis (puffy, fibroinflammatory lesions of orbits and upper respiratory tract) Orbital pseudotumor Riedel’s thyroiditis Mikulicz’s disease (enlargement of lacrimal, salivary, and parotid glands) Kuttner’s tumor (salivary gland enlargement) |
| Allergy/respiratory |
Asthma, atopy, allergy Tracheal stenosis Chronic sinusitis Pleural and pulmonary nodules, interstitial lung disease Fibrosing mediastinitis |
| Systemic | Multifocal fibrosclerosis (orbits, thyroid, retroperitoneum, mediastinum) |
| Large vessels |
Inflammatory aortic aneurysm Periaoritis and periartertitis |
| Renal/retroperitoneum |
Retroperitoneal fibrosis (RPF) Intrinsic renal disease (IgG4-related kidney disease): – Tubulointerstitial nephritis (IgG4-TIN); often with hypocomplementemia – Membranous glomerulonephropathy (IgG4-MGN); anti-PLA2R negative Renal pyelitis |
| Nervous system |
Hypertrophic pachymeningitis Hypophysitis Peri-neural masses |
| Blood and bone marrow |
Eosinophilia Polyclonal hypergammaglobulinemia with elevation in IgG4 and other immunoglobulins |