Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis.
Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis.
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发表时间：
1996
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通讯作者：
P. Nachman;S. Hogan;J. Jennette;R. Falk
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作者：
P. Nachman;S. Hogan;J. Jennette;R. Falk
在这项研究中，评估了 107 例与抗中性粒细胞胞质自身抗体相关的显微镜下多血管炎以及坏死性和新月体性肾小球肾炎患者的缓解率、复发率和治疗抵抗率。韦格纳肉芽肿病患者被排除在外。确定了评估缓解、复发和耐药疾病的前瞻性标准。 107 名患者中有 97 名接受了皮质类固醇治疗 (N = 25) 或环磷酰胺联合皮质类固醇治疗 (N = 72)。在这些患者中，75 例 (77.3%) 进入缓解（完全缓解，N = 61；治疗缓解，N = 14）。在 75 名缓解者中，32 名患者 (43%) 保持长期缓解，平均随访时间为 44 +/- 29 个月； 15 名患者 (20%) 进展为 ESRD，且在治疗结束后平均 21.4 +/- 22.8 个月内没有复发迹象； 6名患者死亡。 75 名最初对治疗有反应的患者中，有 22 名 (29%) 在治疗结束后 18 个月内出现复发，并且通常影响与初次就诊时相同的器官系统。皮质类固醇治疗的患者和环磷酰胺治疗的患者之间的缓解率存在显着差异（56% vs 84.7%，P = 0.003），并且环磷酰胺治疗的患者复发的风险比皮质类固醇治疗的患者低三倍（0.31，95% Cl =（0.12，0.84））。 77%（22 名患者中的 17 名）的治疗抵抗发生在患有暴发性疾病或晚期和严重肾脏疾病的患者中。结论是，大多数患有显微镜下多血管炎或坏死性和新月体性肾小球肾炎的患者通过治疗均可获得缓解。 29% 的患者会出现复发，并且通常对再治疗有反应。使用环磷酰胺和皮质类固醇进行初始治疗，而不是单独使用皮质类固醇，可以降低复发频率。即使就诊时需要透析的患者也可能从治疗中受益，然而，直到疾病过程危及生命才接受治疗的患者可能会在诱导治疗完成之前死亡，这表明仍然需要早期诊断和治疗。
In this study, the rate of remission, relapse, and treatment resistance in 107 patients with microscopic polyangiitis and necrotizing and crescentic glomerulonephritis associated with antineutrophil cytoplasmic autoantibodies were assessed. Patients with Wegener's granulomatosis were excluded. Prospective criteria were identified to assess remission, relapse, and resistant disease. Ninety-seven of the 107 patients received treatment with corticosteroids (N = 25) or with cyclophosphamide and corticosteroids (N = 72). Of these patients, 75 (77.3%) went into remission (complete remission, N = 61; remission on therapy, N = 14). Of the 75 responders, 32 patients (43%) remained in long-term remission, for a mean follow-up of 44 +/- 29 months; 15 patients (20%) progressed to ESRD without signs of relapse, for a mean of 21.4 +/- 22.8 months after the end of treatment; 6 patients died. Twenty-two of the 75 patients who initially responded to treatment (29%) suffered a relapse that occurred within 18 months of the end of therapy and usually affected the same organ systems as on initial presentation. There was a significant difference in the remission rate between the corticosteroid-treated patients and the cyclophosphamide-treated patients (56% versus 84.7%, P = 0.003), and the cyclophosphamide-treated patients had three times less risk of experiencing a relapse than did corticosteroid-treated patients (0.31, 95% Cl = (0.12, 0.84)). Seventy-seven percent (17 of 22 patients) of treatment resistance occurred in patients who presented with fulminant disease or advanced and severe renal disease. It was concluded that most patients with microscopic polyangiitis or necrotizing and crescentic glomerulonephritis achieve remission with therapy. Relapses occur in 29% of patients and generally respond to retreatment. Initial treatment with cyclophosphamide and corticosteroids rather than corticosteroids alone results in a lower frequency of relapse. Even patients who require dialysis at presentation may benefit from treatment, however, patients who are not treated until the disease process is life-threatening may die before induction therapy is complete, indicating the continued need for early diagnosis and therapy.