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抗中性粒细胞胞浆抗体阳性的伴有快速进展性肾小球肾炎的变应性肉芽肿性血管炎

[The Churg-Strauss syndrome with rapidly progressive glomerulonephritis positive for antineutrophil cytoplasmic antibodies].

作者信息

Tesar V, Rychlík I, Bartůnková J, Stejskalová A, Honsová E, Lachmanová J

机构信息

Interní klinika 1. LF UK, Praha.

出版信息

Cas Lek Cesk. 1993 Nov 22;132(22):691-4.

PMID:8293437
Abstract

Renal failure developed in a patient treated for worsening spastic dyspnoea, high erythrocyte sedimentation rate and enlarged peribronchial lymph nodes by the antituberculotic regimen. Renal biopsy disclosed rapidly progressive glomerulonephritis with 95% crescents, granulomatous periglomerulonephritis vasculitis and eosinophilic interstitial infiltrates. On the basis of the positivity of antineutrophil cytoplasmic antibodies (ANCA), eosinophilia and profound ventilatory impairment the diagnosis of Churg-Strauss syndrome was established. The patient was treated by plasma exchanges and combined immunosuppression with the profound effect on erythrocyte sedimentation rate, eosinophilia a negativization of ANCA and preservation of at least minimal renal function. Further therapy was complicated by steroid diabetes, repeated leucopenia and exacerbation of spastic bronchitis and eventually by the massive gastrointestinal haemorrhage from asymptomatic gastric ulcer. There were no signs of inflammation in renal autopsy specimens with prevailing glomerulosclerosis a periglomerular fibrosis. Renal impairment is rare in Churg-Strauss syndrome and it is only exceptionally the cause of renal failure.

摘要

一名因痉挛性呼吸困难加重、红细胞沉降率升高和支气管周围淋巴结肿大而接受抗结核治疗的患者出现了肾衰竭。肾活检显示为快速进展性肾小球肾炎,伴有95%的新月体、肉芽肿性肾小球周围肾炎血管炎和嗜酸性间质浸润。基于抗中性粒细胞胞浆抗体(ANCA)阳性、嗜酸性粒细胞增多和严重的通气功能障碍,确诊为Churg-Strauss综合征。该患者接受了血浆置换和联合免疫抑制治疗,对红细胞沉降率、嗜酸性粒细胞增多、ANCA转阴以及至少保留最低限度的肾功能产生了显著效果。进一步治疗因类固醇糖尿病、反复白细胞减少、痉挛性支气管炎加重而变得复杂,最终因无症状胃溃疡导致大量胃肠道出血。肾尸检标本中没有炎症迹象,主要表现为肾小球硬化和肾小球周围纤维化。Churg-Strauss综合征中肾功能损害罕见,仅在极少数情况下是肾衰竭的原因。

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