ANCA-related crescentic glomerulonephritis in a patient with systemic sclerosis.
- Author:
Jung Seok KWON
1
;
Young Kook SHIN
;
Jin Ho KWAK
;
Sung Hoon PARK
;
Seong Kyu KIM
;
Jung Yoon CHOE
Author Information
1. Department of Internal Medicine, School of Medicine, Catholic University of Daegu, Daegu, Korea. jychoe@cu.ac.kr
- Publication Type:Case Report
- Keywords:
Systemic sclerosis;
ANCA;
Crescentic glomerulonephritis;
Renal crisis;
Pulse therapy
- MeSH:
Antibodies, Antineutrophil Cytoplasmic;
Azotemia;
Biopsy;
Chymosin;
Cytoplasm;
Female;
Glomerulonephritis;
Humans;
Hypertension;
Hypertension, Malignant;
Hypertensive Retinopathy;
Isonipecotic Acids;
Middle Aged;
Plasma;
Proteinuria;
Renal Insufficiency;
Renin;
Scleroderma, Systemic
- From:Korean Journal of Medicine
2009;76(Suppl 1):S154-S157
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The renal manifestations of systemic sclerosis include proteinuria, hypertension, azotemia, and renal crisis. Two types of scleroderma renal crisis (SRC) are recognized. Typical SRC is a syndrome consisting of acute-onset malignant hypertension accompanied by rapidly progressive renal failure, hypertensive retinopathy, and elevated plasma renin activity. The other type is normotensive renal failure, which is generally accompanied by antineutrophil cytoplasmic autoantibody (ANCA)-positive crescentic glomerulonephritis. A 51-year-old woman with scleroderma without marked dermatological change developed ANCA-related renal failure. She had neither malignant hypertension nor an elevated plasma rennin concentration. Renal biopsy showed crescentic glomerulonephritis (pauci-immune type), and the myeloperoxidase-specific ANCA (MPO-ANCA) titer was elevated at 1015 AAU. She was cured using steroid pulse therapy, combined with an angiotensin-converting-enzyme inhibitor and angiotensin-II receptor blocker
