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 |