p-ANCA가 동시에 검출된 항사구체 기저막 항체 질환 2예

Abstract

Anti-glomerular basement membrane antibody (anti-GBM Ab) disease is characterized by circulating antibodies to the glomerular basement membrane and the deposition of IgG or, rarely, IgA along the glomerular basement membrane. This disease accounts for 10-20% of crescentic glomerulonephritis. We report two patients with anti-GBM Ab disease who were positive for perinuclear-anti-neutrophil cytoplasmic antibody (p-ANCA). Percutaneous renal biopsies showed many crescent formations and linear deposits of IgG along the glomerular basement membrane. Serologic tests for p-ANCA were positive. They were treated with steroid pulse and cyclophosphamide and one patient also underwent plasma exchange therapy. Despite immunosuppressive therapy, their renal functions did not improve and both required regular hemodialysis. (Korean J Med 2012;83:514-519) Keywords: Anti-glomerular basement membrane antibody disease; Glomerulonephritis; Perinuclear-anti-neutrophil cytoplasmic antibody

저자들은 폐출혈이 동반되지 않는 항사구체 기저막 항체 질환 환자에서 동시에 p-ANCA가 검출된 증례 2예를 경험하여 이를 문헌고찰과 함께 보고하는 바이다. 중심 단어: 항사구체 기저막 항체 질환; 반월상 사구체 신염; 항호중구세포질 항체