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[系统性硬化症与结节病:3例新病例]

[Systemic scleroderma and sarcoidosis: 3 new cases].

作者信息

Bandt M D, Meyer O, Masson C, Peroux-Goumy L, Audran M, Kahn M F

机构信息

Service de Rhumatologie, Hôpital Bichat, Paris.

出版信息

Ann Med Interne (Paris). 1996;147(8):590-4.

PMID:9137689
Abstract

We observed 3 patients with successive scleroderma (SS) and (what is considered to be) sarcoidosis (SA). The diagnosis SS included in the 3 patients: Raynaud's syndrome with pulpal necrosis and capillaritis, sclerodactylia and acro-osteolysis, multiple joint pain and FAN+. Also observed were: esophagus involvement (n = 3), pulmonary artery hypertension (n = 1), telangiectasia (n = 2) and anti-Scl 70 (n = 2). Initially, all patients had restrictive pulmonary disease. SS was diagnosed 5 to 9 years prior to SA in 2 patients. Diagnosis of SA was based on the following arguments: Loëfgren's syndrome with erythema nodosa (n = 1), parotiditis (n = 2), sicca syndrome (n = 2), myalgia (n = 2), joint involvement (n = 2), non-infectious pluropericarditis (n = 2), epitheloid and giant cell granulomas without caseous necrosis (lung = 3, liver = 1, lymph nodes = 1, salivary glands = 1, synovia = 1), negative search for bacilli, elevated conversion enzyme (n = 1) and, in each case, by the lack of any other cause. One patient died from lung cancer and another from respiratory failure. Nome of the patients had primary biliary cirhosis. This rare association between SS and SA demonstrates the confluent limits of certain systemic diseases and raises a difficult problem to differentiate pulmonary involvement in these diseases. The gravity of this localization and the poor sensitivy to corticosteroids.

摘要

我们观察到3例患有连续性硬皮病(SS)以及(被认为是)结节病(SA)的患者。这3例患者的SS诊断包括:伴有指端坏死和毛细血管炎的雷诺综合征、硬指和肢端骨质溶解、多关节疼痛以及FAN+。还观察到:食管受累(n = 3)、肺动脉高压(n = 1)、毛细血管扩张(n = 2)以及抗Scl 70(n = 2)。最初,所有患者均患有限制性肺病。2例患者在SA诊断前5至9年被诊断为SS。SA的诊断基于以下依据:伴有结节性红斑的 Löfgren综合征(n = 1)、腮腺炎(n = 2)、干燥综合征(n = 2)、肌痛(n = 2)、关节受累(n = 2)、非感染性多浆膜炎(n = 2)、无干酪样坏死的上皮样和巨细胞肉芽肿(肺 = 3、肝 = 1、淋巴结 = 1、唾液腺 = 1、滑膜 = 1)、未检出杆菌、转换酶升高(n = 1),并且在每种情况下均无任何其他病因。1例患者死于肺癌,另1例死于呼吸衰竭。所有患者均无原发性胆汁性肝硬化。SS与SA之间这种罕见的关联表明了某些全身性疾病的融合界限,并提出了一个难以区分这些疾病肺部受累情况的难题。这种定位的严重性以及对皮质类固醇的敏感性较差。

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