title: Clinical significance of complement as a biomarker of disease activity in 4 cases of IgG4-related disease with retroperitoneal fibrosis.
abstract: Hypocomplementaemia is frequently observed in IgG4-related diseases, however the clinical significance is unclear. We describe herein the clinical courses of 4 patients with IgG4-related disease with hypocomplementaemia. Our cases showed autoimmune pancreatitis, retroperitoneal fibrosis, Mikulicz's disease, interstitial lung disease, lymphadenopathy and mesenteric fibrosis around the aorta. A decrease in serum complement preceded deterioration of the disease and clinical improvement was observed in accordance with normalisation of serum complement. These clinical courses suggest that serum complement is a biomarker of the disease activity.
authors.affiliation: Department of Medicine and Rheumatology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan, Department of Medicine and Rheumatology, Tokyo Medical and Dental University, Tokyo, Japan. kihara.rheu@tmd.ac.jp.
authors.forename: Mari
authors.initials: M
authors.lastname: Kihara
authors.forename: Takahiko
authors.initials: T
authors.lastname: Sugihara
authors.forename: Tadashi
authors.initials: T
authors.lastname: Hosoya
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Miyasaka
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D003165:Complement System Proteins / Q000378:metabolism*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D008206:Lymphatic Diseases / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D008882:Mikulicz' Disease / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D010195:Pancreatitis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D011237:Predictive Value of Tests; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction; D012185:Retroperitoneal Fibrosis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D003165:Complement System Proteins; D011239:Prednisolone