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[系统性纤维化(奥蒙德病的全身性形式)。1例经环磷酰胺和皮质类固醇治疗后完全缓解的病例报告]

[Systemic fibrosis (generalized form of Ormond's disease). Report of a case which achieved complete remission with cyclophosphamide and corticosteroids].

作者信息

Kohler H P, Laeng R H, Egger C, Streuli R

机构信息

Medizinische Klinik, Regionalspital Langenthal.

出版信息

Schweiz Med Wochenschr. 1995 Nov 4;125(44):2131-6.

PMID:7502012
Abstract

A 51-year-old engineer was admitted with progressive lower back pain which had started 4 months before. We found an elevated ESR and anemia. Chest X-ray showed bilateral polycyclic thickening of the pleura, and abdominal CT examination revealed a paraaortic tumorous lesion and a solid kidney tumor with a diameter of 5 cm on the left side. During the course of the disease we also observed an infiltration in the apex of the upper lobe of the left lung. Histological examination showed fibrotic tissue typical of Ormond's disease in the kidney tumor as well as in the pulmonary infiltrate. We diagnosed a systemic form of retroperitoneal fibrosis. Treatment with cyclophosphamide (combined with prednisone during the first 4 months) resulted in complete remission of the disease.

摘要

一名51岁的工程师因4个月前开始的进行性下背部疼痛入院。我们发现血沉升高和贫血。胸部X线显示双侧胸膜多环增厚,腹部CT检查发现主动脉旁肿瘤性病变以及左侧一个直径5厘米的实性肾肿瘤。在疾病过程中,我们还观察到左肺上叶尖部有浸润。组织学检查显示,肾肿瘤以及肺部浸润中有奥蒙德病典型的纤维化组织。我们诊断为系统性腹膜后纤维化。用环磷酰胺治疗(最初4个月与泼尼松联合使用)使疾病完全缓解。

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