A nationwide survey of rapidly progressive glomerulonephritis in Japan: etiology, prognosis and treatment diversity
Tóm tắt
The etiology, prevalence, and prognosis of rapidly progressive glomerulonephritis (RPGN) including renal vasculitis vary among races and periods. To improve the prognosis of Japanese RPGN patients, we conducted a nationwide survey of RPGN in the nephrology departments of 351 tertiary hospitals, and found 1772 patients with RPGN (Group A: diagnosed between 1989 and 1998, 884 cases; Group B: diagnosed between 1999 and 2001, 321 cases; and Group C: diagnosed between 2002 and 2007, 567 cases). ANCA subclasses, renal biopsy findings, treatment, outcome and cause of death were recorded. The most frequent primary disease was renal-limited vasculitis (RLV) (42.1%); the second was microscopic polyangiitis (MPA) (19.4%); the third was anti-GBM-associated RPGN (6.1%). MPO-ANCA was positive in 88.1% of RLV patients and 91.8% of MPA patients. The proportion of primary renal diseases of RPGN was constant during those periods. The most frequent cause of death was infectious complications. The serum creatinine at presentation and the initial dose of oral prednisolone decreased significantly in Groups B and C compared to Group A. However, both patient and renal survival rates improved significantly in Groups B and C (survival rate after six months in Group A: 79.2%, Group B: 80.1%, and Group C: 86.1%. Six-month renal survival in Group A: 73.3%, Group B: 81.3%, and Group C: 81.8%). Early diagnosis was the most important factor for improving the prognosis of RPGN patients. To avoid early death due to opportunistic infection in older patients, a milder immunosuppressive treatment such as an initial oral prednisolone dose reduction with or without immunosuppressant is recommended.
Tài liệu tham khảo
Simon P, Ramee MP, Autuly V, Laruelle E, Charasse C, Cam G, et al. Epidemiology of primary glomerular diseases in a French region. Variations according to period of age. Kidney Int. 1994;46:1192–8.
Levy JB, Winearls CG. Rapidly progressive glomerulonephritis: what should be first-line therapy? Nephron. 1994;67:402–7.
Fuiano G, Cameron JS, Raftery M, Hratley BH, Williams DG, Ogg CS. Improved prognosis of renal microscopic polyarteritis in recent years. Nephrol Dial Transplant. 1988;3:383–91.
Haas M, Spargo BH, Wit EJ, Meehan SM. Etiologies and outcome of acute renal insufficiency in older adults: a renal biopsy study of 259 cases. Am J Kidney Dis. 2000;35:433–47.
Hogan SL, Nachman PH, Wilkman AS, Jennette JC, Falk RJ. Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol. 1996;7:23–32.
Satchell SC, Nicholls AJ, D’Souza RJ, Beaman M. Renal vasculitis: increasingly a disease of the elderly? Nephron Clin Pract. 2004;97:C142–6.
Sakai H, Kurokawa K, Koyama A, Arimura Y, Kida H, Shigematsu S, et al. Clinical guildeline for rapidly progressive glomerulonephritis in Japan. Jpn J Nephrol. 2002;44:55–82.
Glassock RJ, Cohen RH, Adler SG. Rapidly progressive glomerulonephritis. In: Brenner BM, editor. The kidney. Philadelphia: Saunders Co., 1996:1402–1421.
Couser WG. Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy. Am J Kidney Dis. 1988;11:449–64.
Davies DJ, Moran JE, Niall JF, Ryan GB. Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? Br Med J (Clin Res Ed). 1982;285:606.
Gallagher H, Kwan JTC, Jayne DR. Pulmonary renal syndrome: a 4-year, single-center experience. Am J Kidney Dis. 2002;39:42–7.
Falk RJ, Jennette JC. Anti-neutrophic cytoplasmic autoantibodies with specificity for myeloperoxydase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med. 1988;318:1651–7.
Jennette JC. Rapidly progressive crescentic glomerulonephritis. Kidney Int. 2003;63:1164–77.
Franssen C, Stegeman C, Kallenberg C, Gans R, Jong P, Hoornije S, et al. Antipreteinase 3- and antimyeloperoxidase-associated vasculitis. Kidney Int. 2000;57:2195–206.
Yashiro M, Muso E, Itoh-Ihara T, Oyama A, Hashimoto K, Kawamura T, et al. Significantly high regional morbidity of MPO-ANCA-related angitis and/or nephritis with respiratory tract involvement after the 1995 great earthquake in Kobe (Japan). Am J Kidney Dis. 2000;35:889–95.
Wichmann I, Sanchez-Roman J, Morales J, Castillo MJ, Ocana C, Nunez-Roldan A. Antimyeloperoxidase antibodies in individuals with occupational exposure to silica. Ann Rheum Dis. 1996;55:205–7.
Fujimoto S, Uezono S, Hisanaga S, Fukudome K, Kobayashi S, Suzuki K, et al. Incidence of ANCA-associated primary renal vasculitis in the Miyazaki Prefecture: the first population-based, retrospective, epidemiologic survey in Japan. Clin J Am Soc Nephrol. 2006;1:1016–22.
Gencik M, Meller S, Borgmann S, Sitter T, Menezes Saecker AM, Fricke H, et al. The association of CD18 alleles with anti-myeloperoxidase subtypes of ANCA-associated systemic vasculitides. Clin Immunol. 2000;94:9–12.
Tsuchiya N, Kobayashi S, Kawasaki A, Kyogoku C, Arimura Y, Yoshida M, et al. Genetic background of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis: association of HLA-DRB1*0901 with microscopic polyangiitis. J Rheumatol. 2003;30:1534–40.
Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg–Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001;44:666–75.
Booth AD, Almond MK, Burns A, Ellis P, Gaskin G, Neild GH, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis. 2003;41:776–84.
Yamagata K, Hirayama K, Mase K, Yamaguchi N, Kobayashi M, Takahashi H, et al. Apheresis for MPO-ANCA-associated RPGN-indications and efficacy: lessons learned from Japan nationwide survey of RPGN. J Clin Apher. 2005;20:244–51.
Vizjak A, Rott T, Koselj-Kajtna M, Rozman B, Kaplan-Pavlovcic S, Ferluga D. Histologic and immunohistologic study and clinical presentation of ANCA-associated glomerulonephritis with correlation to ANCA antigen specificity. Am J Kidney Dis. 2003;41:539–49.
Hauer HA, Bajema IM, van Houwelingen HC, Ferrario F, Noel LH, Waldherr R, et al. Renal histology in ANCA-associated vasculitis: differences between diagnostic and serologic subgroups. Kidney Int. 2002;61:80–9.
Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. Q J Med. 1994;87:671–8.
Green H, Paul M, Vidal L, Leibovici L. Prophylaxis for Pneumocystis pneumonia (PCP) in non-HIV immunocompromised patients. Cochrane Database Syst Rev 2007;CD005590.
Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniene J, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349:36–44.
Nowack R, Gobel U, Klooker P, Hergesell O, Andrassy K, van der Woude FJ. Mycophenolate mofetil for maintenance therapy of Wegener’s granulomatosis and microscopic polyangiitis: a pilot study in 11 patients with renal involvement. J Am Soc Nephrol. 1999;10:1965–71.
Hirayama K, Kobayashi M, Hashimoto Y, Usui J, Shimizu Y, Hirayama A, et al. Treatment with the purine synthesis inhibitor mizoribine for ANCA-associated renal vasculitis. Am J Kidney Dis. 2004;44:57–63.