Table 3.

Preferred Nomenclature for Individual Organ Manifestations of IgG4-Related Disease.

Organ System/Tissue	Preferred Name
Pancreas	Type 1 autoimmune pancreatitis (IgG4-related pancreatitis)
Eye	IgG4-related ophthalmic disease is the general term for the peri-ocular manifestations of this disease. There are several subsets, outlined below.
Lacrimal glands	IgG4-related dacryoadenitis
Orbital soft tissue (orbital inflammatory pseudotumor)	IgG4-related orbital inflammation (or IgG4-related orbital inflammatory pseudotumor)
Extra-ocular muscle disease	IgG4-related orbital myositis
Orbit with involvement of multiple anatomic structures	IgG4-related pan-orbital inflammation (includes lacrimal gland disease, extra-ocular muscle involvement, and other potential intra-orbital complications)
Salivary glands (parotid and submandibular glands)	IgG4-related sialadenitis or, more specifically, IgG4-related parotitis or IgG4-related submandibular gland disease
Pachymeninges	IgG4-related pachymeningitis
Hypophysis	IgG4-related hypophysitis
Thyroid (Riedel’s thyroiditis)	IgG4-related thyroiditis
Aorta	IgG4-related aortitis/periaortitis
Arteries	IgG4-related periarteritis
Mediastinum	IgG4-related mediastinitis
Retroperitoneum	IgG4-related retroperitoneal fibrosis
Mesentery	IgG4-related mesenteritis
Skin	IgG4-related skin disease
Lymph node	IgG4-related lymphadenopathy
Bile ducts	IgG4-related sclerosing cholangitis
Gallbladder	IgG4-related cholecystitis
Liver	IgG4-related hepatopathy (refers to liver involvement that is distinct from biliary tract involvement)
Lung	IgG4-related lung disease
Pleura	IgG4-related pleuritis
Pericardium	IgG4-related pericarditis
Kidney	IgG4-related kidney disease. The specific renal complications should be termed tubulointerstitial nephritis secondary to IgG4-RD and membranous glomerulonephritis secondary to IgG4-RD. Involvement of the renal pelvis should be termed IgG4-related renal pyelitis.
Breast	IgG4-related mastitis
Prostate	IgG4-related prostatitis