Immunoglobulin G4-related kidney diseases: An updated review

Table 4 Clinical presentation of immunoglobulin G4-related disease per site of involvement

Organ system	Nomenclature	Clinical features
Orbit	IgG4-related ophthalmic disease IgG4-related orbital inflammatory pseudo-tumor IgG4-related pan-orbital inflammation IgG4-related orbital myositis	Swelling of orbital tissue and proptosis
Lacrimal gland	IgG4-related dacryadenitis	Bilateral swelling of the glands and impaired production of secretion
Salivary gland	IgG4-related sialoadenitis IgG4-related parotitis IgG4-related submandibular gland disease	Bilateral swelling of the glands and impaired production of secretion
Thyroid	IgG4-related thyroid disease	Hypothyroidism, neck pain, dysphagia, dyspnea
Liver	IgG4-related hepatopathy	Jaundice, right upper quadrant mass
Biliary tract and gall bladder	IgG4-related sclerosing cholangitis IgG4-related cholecystitis	Jaundice, pruritus, cholestasis
Blood vessels	IgG4-related aortitis/periaortitis IgG4-related periarteritis	Chest pain, dyspnea
Retroperitoneal fibrosis	IgG4-related retroperitoneal fibrosis	Flank pain, obstructive symptoms, peripheral edema
Kidneys	IgG4-related kidney disease Tubulo-interstitial nephritis secondary to IgG4-related disease	Hematuria, proteinuria, hypocomplementemia, chronic renal failure
Skin	IgG4-related skin disease	Papulonodular lesions, plaques, purpura