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Table 1 Nomenclature for manifestations in different organ systems [adapted from Stone JH et al. A&R [[10]]]

From: IgG4- related disease: an orphan disease with many faces

Pancreas

IgG4-related pancreatitis (Type 1 autoimmune pancreatitis)

Bile ducts, gallbladder and liver

IgG4-related sclerosing cholangitis

 

IgG4-related cholecystitis

 

IgG4-related hepatopathy

Thyroid gland

IgG4-related thyroid disease

Salivary and lacrimal glands

IgG4-related sialadenitis

 

IgG4-related parotitis

 

IgG4-related submandibular gland disease

 

IgG4-related dacryoadenitis

Orbits

IgG4-related ophthalmic disease

 

IgG4-related orbital inflammatory pseudotumor

 

IgG4-related pan-orbital inflammation

 

IgG4-related orbital myositis

Retroperitoneal fibrosis, arteries

IgG4-related retroperitoneal fibrosis

 

IgG4-related aortitis/periaortitis

 

IgG4-related periarteritis

Intrapulmonary, mediastinal and pleural involvement

IgG4-related lung disease

 

IgG4-related mediastinitis

 

IgG4-related pleuritis

Lymph nodes

IgG4-related lymphadenopathy

Kidney

IgG4-related kidney disease

 

tubulointerstitial nephritis secondary to IgG4-related disease

 

membranous glomerulonephritis secondary to IgG4-related disease

 

IgG4-related renal pyelitis

Miscellaneous

IgG4-related perineural disease*

 

IgG4-related pachymeningitis

 

IgG4-related hypophysitis

 

IgG4-related mesenteritis

 

IgG4-related mastitis

 

IgG4-related prostatitis

 

IgG4-related epididymo-orchitis*

 

IgG4-related paratesticular pseudotumor*

 

IgG4-related skin disease

 

IgG4-related pericarditis