A Case of Crescentic IgA Nephropathy with Positive ANCA Serology

Korean Journal of Nephrology 2007;26(4):475-479.

Young Eun Park, M.D.1, Young Mi Seol, M.D.1, Hye Jeong Lee, M.D.1, Seoung Jae An, M.D.1, Sang Heon Song, M.D.1, Dong Won Lee, M.D.1, Soo Bong Lee, M.D.1, Ihm Soo Kwak, M.D.1 and Mi Young Seol, M.D2.

Department of Internal Medicine¹
Pathology2 Pusan National University Collage of Medicine, Busan, Korea

혈청 항중성구세포질항체 (ANCA) 양성인 반월상 IgA신증 1예

박영은1

부산대학교 의과대학 내과학교실1, 병리학교실2

Abstract

Immunoglobulin A (IgA) nephropathy in patients with the clinical findings of rapidly progressive glomerulonephritis usually demonstrate crescentic glomerulonephritis (CrGN). It is characterized by mesangial immune complex deposits containing IgA and is rarely associated with ANCA. The following case report illustrates a CrGN with mesangial & capillary wall IgA deposits by immunofluorescence (IF) and mesangial & endocapillary electron dense deposits by electron microscopy (EM) in patient with positive ANCA serology. A 54-year-old male visited due to the discomfort of right flank. Blood pressure was 150/90 mmHg. BUN and serum Creatinine was 40 mg/dL, 4.4 mg/dL respectively. Urinalysis revealed protein 2+, >30 RBC's/ HPF (dysmorphic), 24hr urine protein 1,612 mg/day and creatinine clearance 19 mL/min/1.73m2. Serological P-ANCA was positive by indirect immunofluorescence & ELISA. The histologic findings showed global scleorsis and fibrocelluar crescent without hypercellularity on LM, mesangial IgA deposition on IF, mesangial and subendothelial electrone dense material depositions on EM. After treatment (Methylprednisolon 500 mg/day for 3 days and then prednisolone 60 mg/day), Serum creatinin level, proteinuria and ANCA titer were decreased.

Key Words: Glomerulonephritis, Immunoglobulin A (IgA), Antibodies, Antineutrophil cytoplasmic