MPO-ANCA Associated Rapidly Progressive Glomerulonephritis in A Patient with Mixed Connective Tissue Disease

Korean Journal of Nephrology 2010;29(6):776-781.

Hyun-Seok Hong, M.D.1, Ji-Hoon Park, M.D.1, Ju-Hyun Lee, M.D.1, Joo-Hark Yi, M.D.1, Sang-Woong Han, M.D.1, Moon-Hyang Park, M.D.2 and Ho-Jung Kim, M.D.1

Department of Internal Medicine¹
Hanyang University Guri Hospital, Guri, Korea Department of Pathology²
Hanyang University College of Medicine, Seoul, Korea

증례 : 복합교원성질환과 동반되어 나타난 MPO-ANCA 관련 급속진행 사구체신염 1예

홍현석1, 박지훈1, 이주현1, 이주학1, 한상웅1, 박문향2, 김호중1

한양대학교 구리병원 내과학교실1, 한양대학교 의과대학 병리학교실2

Abstract

Mixed connective tissue disease (MCTD) has the clinical feature of other collagen vascular diseases. According to several reports recently published, MCTD sometimes involved kidney as benign course. ANCA associated glomerulonephritis occurred in systemic autoimmune disease such as systemic sclerosis or rheumatoid arthritis, not in MCTD. In this case, as we experienced that MPO-ANCA associated glomerulonephritis arose and proceeded to rapid progressive glomerulonephritis (RPGN) in women with MCTD, we report it with review of reference. A 60-year-old woman was admitted with uremia and joint pain. In physical finding and serum test, she was diagnosed with MCTD according to Khan`s criteria. Also MPO antibody and ANCA were noted in specific immunological test and the histologic findings showed crescentic glomerulonephritis. So we diagnosed RPGN induced by MPO-ANCA associated glomerulonephritis. She was treated with immunosuppressive treatment and uremia was improved. RPGN induced by MPO-ANCA associated glomerulonephritis should be ruled out by prompt renal biopsy and require proper treatment.

Key Words: ANCA, Glomerulonephritis, Mixed connective tissue disease