Persistent Hematuria After Induction of Remission in Wegener Granulomatosis A Therapeutic Dilemma
Persistent Hematuria After Induction of Remission in Wegener Granulomatosis A Therapeutic Dilemma
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DOI：
10.1097/md.0b013e3181c101cc
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发表时间：
2009-11-01
期刊：
影响因子：
1.6
通讯作者：
Hoffman, Gary S.
中科院分区：
文献类型：
--
作者：
Magrey, Marina N.;Villa-Forte, Alexandra;Hoffman, Gary S.
韦格纳肉芽肿病（WG）是一种全身性疾病，通常与免疫介导的肾小球肾炎（GN）有关。肾脏疾病最常表现为镜下血尿伴或不伴红细胞或混合细胞管型、蛋白尿和血清肌酐浓度升高，我们进行了目前的研究，以确定持续性血尿，在明显的临床缓解的情况下，是否可能反映肾小球损伤，而不是活动性肾脏疾病。我们对82例新发WG患者的资料进行了回顾性分析，其中25例在就诊时患有GN，25例GN患者中有20例获得了持续缓解（持续时间>6个月）。在活动性疾病的初始阶段，中位峰值血清肌酐为1.9 mg/dL（范围，0.6-13.6 mg/dL）。中位缓解时间为4个月（范围，2-13个月）。有效治疗后，中位肌酐为1.1 mg/dL（范围：0.4-1.8 mg/dL）。20例患者中有10例患者的血尿时间超过6个月。在该亚组中，5名患者随后在11个月的中位时间段内尿正常化，5名患者在38个月的中位随访期内未达到正常尿沉渣。因此，25例WG和GN患者中有10例持续血尿，尽管明显延长临床缓解。WG和GN患者可能会达到持久的缓解，允许停药，尽管持续的显微镜下血尿，有或没有红细胞管型，可能持续数月甚至数年。对此类患者继续使用积极的免疫抑制治疗是不明智的，可能导致环磷酰胺或高剂量皮质类固醇的不可逆甚至危及生命的副作用。
Wegener granulomatosis (WG) is a systemic disease that is often associated with an immune-mediated form of glomerulonephritis (GN). Renal disease most often manifests as microscopic hematuria with or without red blood cell or mixed cellular casts, proteinuria, and an elevated serum creatinine concentration.We conducted the current study to determine whether persistent hematuria, in the setting of apparent clinical remission, may reflect glomerular injury and not active renal disease. We performed a retrospective analysis of data from 82 patients with new-onset WG, of whom 25 had GN at presentation.Twenty of 25 patients with GN achieved sustained remissions (>6 consecutive months' duration). During initial periods of active disease the median peak serum creatinine was 1.9 mg/dL (range, 0.6-13.6 mg/dL). The median time to remission was 4 months (range, 2-13 mo). After effective therapy, median creatinine was 1.1 mg/dL (range, 0.4-1.8 mg/dL). Ten of 20 patients had prolonged hematuria over a period of >6 months. Within this subset, 5 subsequently normalized urine over a median period of I I months and 5 did not achieve normal urine sediment over a median follow-up of 38 months. Thus, 10 of 25 patients with WG and GN had sustained hematuria in spite of apparent prolonged clinical remission.Patients with WG and GN may achieve enduring remissions that allow withdrawal of medications in spite of continued microscopic hematuria with or without red blood cell casts that may persist for months or even years. Continued use of aggressive immunosuppressive therapies in such patients would be ill-advised and could lead to irreversible and even life-threatening side effects from cyclophosphamide or high-dose corticosteroids.