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Table 1 Common manifestations of IgG4-RD by organ system

From: Recognizing IgG4-related tubulointerstitial nephritis

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