title: [From 'malignancy' to IgG4-related systemic disease].
abstract: IgG4-related systemic disease is a new clinical entity with a large variety of clinical symptoms that can affect almost all organs. The best known manifestations are retroperitoneal fibrosis and autoimmune pancreatitis. We present 3 patients aged 71, 83 and 70 years, with malaise, fatigue and swellings suggestive of a malignancy. However, histopathology of these swellings showed infiltration with plasma cells. Increased serum IgG4-levels confirmed the diagnosis 'IgG4-related systemic disease'. All patients responded well to treatment with glucocorticoids. IgG4-related systemic disease is often mistaken for malignancy because of similar presenting symptoms. The diagnosis can easily be confirmed by high serum protein levels, high serum IgG4-levels and infiltrates of IgG4-positive plasma cells. Response to treatment with glucocorticoids is good, as is the prognosis. IgG4-related systemic disease should be part of the differential diagnosis when patients present with malaise, high protein-levels and multi-organ involvement. Rapid diagnosis can prevent unnecessary surgical procedures for malignancy.
authors: Universitair Medisch Centrum Groningen, Afd. Nefrologie, Groningen, the Netherlands. a.b.kramer@int.umcg.nl
authors: Andrea B
authors: AB
authors: Kramer
authors: Helma R
authors: HR
authors: Lebbink
authors: Marcory C R F
authors: MC
authors: van Dijk
authors: Casper F M
authors: CF
authors: Franssen
authors: Coen A
authors: CA
authors: Stegeman
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D003937:Diagnosis, Differential; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D007074:Immunoglobulin G / Q000032:analysis*; D008297:Male; D009369:Neoplasms / Q000175:diagnosis; D010950:Plasma Cells / Q000276:immunology* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G