신장 조직 검사에서 사구체 면역침착을 보인 베게너 육아종증 1예 = Case Report : A Case of Pa-tient with Wegener`s Granulomatosis Showing Glomerular Immune Deposition in Kidney Biopsy

다국어 초록 (Multilingual Abstract)

Rapidly progressive glomerulonephritis (RPGN) in Wegener`s granulomatosis patients typically has been characterized by pauci-immune glomerulonephritis (PIGN). In some patients, however, significant amount of glomerular immune deposits was detected and reported that they may have poor prognosis. A 30 year-old-female visited due to the skin rash of both lower extremities, arthralgia and nasal stiffness. She had sinusitis, lung opacity, and proteinuria. Serologic PR-3 ANCA was positive and histologic findings of nasal cavity and lung also showed necrotizing vasculitis and granuloma. Thus we could diagnose Wegener`s granulomatosis. However, gross hematuria developed and renal function worsened in spite of treatment with high dose prednisolone and oral cyclophosphamide. Therefore we performed a kidney biopsy. The kidney biopsy showed crescentic glomerulonephritis with Ig A deposition in the mesangium. We experienced a case of Wegener`s granulomatosis patient with significant IgA deposition in glomeruli. We report this case with brief review of the literature.

참고문헌 (Reference)

1 박영은, "혈청 항중성구세포질항체 (ANCA) 양성인 반월상 IgA신증 1예" 대한신장학회 26 (26): 475-479, 2007

2 Fauci AS, "Wegener's granulomatosis: studies in eighteen patients and a review of the literature" 52 : 535-561, 1973

3 Aasarød K, "Wegener's granulomatosis: clinical course in 108 patients with renal involvement" 15 : 611-618, 2000

4 Hoffman GS, "Wegener granulomatosis: an analysis of 158 patients" 116 : 488-498, 1992

5 Cochrane CG, "The role of polymorphonuclear leukocytes in the initiation and cessation of the Arthus vasculitis" 110 : 481-494, 1959

6 Leavitt RY, "The american college of rheumatology 1990 criteria for the classification of Wegener's granulomatosis" 33 : 1101-1107, 1990

7 Couser WG, "Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy" 11 : 449-464, 1988

8 Jennette JC, "Pauci-immne and antineutrophil cytoplasmic autoantibody-mediated cresenteric glomerulonephritis and vasculitis: Heptinstall’s Pathology of the Kidney 6th ed" Linppincott Williams & Wilkins, Inc. 2007

9 Ardiles LG, "Incidence and studies on antigenic specificities of antineutrophil-cytoplasmic autoantibodies (ANCA) in poststreptococcal glomerulonephritis" 47 : 1-5, 1997

10 Neumann I, "Glomerular immune deposits are associated with increased proteinuria in patients with ANCA-associated crescentic nephritis" 18 : 524-531, 2003

11 Yu F, "Clinical and pathological characteristics and outcomes of chinese patients with primary anti-neutrophil cytoplasmic antibodies-associated systemic vasculitis with immune complex deposition in kidney" 12 : 74-80, 2007

12 Ozaki S, "ANCA-associated vasculitis: diagnostic and therapeutic strategy" 56 : 87-96, 2007

13 Weidner S, "ANCA-associated vasculitis with 뭐renal involvement: an outcome analysis" 19 : 1403-1411, 2004