Open AccessGlomerulonephritis associated with complete deficiency of the fourth component of complement response to intravenous immunoglobulin
Author(s) -
Welch Thomas R.,
McAdams A. James,
Beischel Linda S.
Publication year - 1995
Publication title -
arthritis & rheumatism
Language(s) - English
Resource type - Journals
eISSN - 1529-0131
pISSN - 0004-3591
DOI - 10.1002/art.1780380923
Subject(s) - medicine , glomerulonephritis , complement deficiency , immunology , immunosuppression , renal biopsy , lupus nephritis , biopsy , rash , immunopathology , mesangial proliferative glomerulonephritis , gastroenterology , antibody , complement system , kidney , disease
Abstract A 15‐year‐old girl with complete C4 deficiency and a lupus‐like disorder developed evidence of nephritis after 4 years of followup. Renal biopsy demonstrated an immune complex glomerulonephritis, with deposits in the capillary loops, the paramesangium, and the mesangial matrix. Renal function was normal. The patient was treated with monthly infusions of intravenous immunoglobulin for 6 months. The treatment was well tolerated, and resulted in resolution of the rash and hematuria. Followup biopsy showed less proliferation and fewer loop deposits. In light of the serious risk of infections that is associated with complement deficiency, approaches to glomerulonephritis that do not include immunosuppression should be considered.