Lomeo R M, Cornella R J, Schabel S I, Silver R M
Department of Medicine, Medical University of South Carolina, Charleston 29425.
Am J Med. 1989 Nov;87(5):525-7. doi: 10.1016/s0002-9343(89)80608-4.
The purpose of this work was to report the clinical features of 10 patients with systemic sclerosis presenting with lung disease in the absence of cutaneous involvement (systemic sclerosis sine scleroderma).
The study involved a retrospective review of patients with systemic sclerosis at a tertiary care facility presenting with clinical and/or radiographic evidence of interstitial lung disease in the absence of cutaneous manifestations of scleroderma, and a MEDLINE, computer search for patients who presented with lung disease and later developed taut skin (scleroderma).
Ten patients with systemic sclerosis sine scleroderma presenting with pulmonary signs or symptoms were discovered. A positive antinuclear antibody (seven of eight), esophageal dysmotility (eight of nine), restrictive lung disease (six of eight), Raynaud's phenomenon (five of eight), and abnormal nailfold capillaries (six of six) were present in these patients. Six of these 10 patients developed typical scleroderma from four months to seven years after presentation. Lung pathology was examined in six patients and all had changes consistent with interstitial pneumonitis and fibrosis. Four of the 10 patients had occupations that could have exposed them to potential pulmonary toxins. In contrast to patients with typical scleroderma, all 10 patients we discovered were men. conclusions: Systemic sclerosis should be considered in patients with idiopathic interstitial lung disease even in the absence of classic sclerodermatous cutaneous involvement. An esophagram, antinuclear antibody, and widefield nailfold examination may facilitate the diagnosis.
本研究旨在报告10例无皮肤受累（无硬皮病的系统性硬化症）而出现肺部疾病的系统性硬化症患者的临床特征。
本研究对一家三级医疗机构中出现间质性肺疾病临床和/或影像学证据但无硬皮病皮肤表现的系统性硬化症患者进行了回顾性分析，并通过医学文献数据库（MEDLINE）进行计算机检索，查找出现肺部疾病并随后出现皮肤紧绷（硬皮病）的患者。
发现了10例无硬皮病的系统性硬化症患者出现肺部体征或症状。这些患者中存在抗核抗体阳性（8例中的7例）、食管动力障碍（9例中的8例）、限制性肺病（8例中的6例）、雷诺现象（8例中的5例）以及甲襞毛细血管异常（6例中的6例）。这10例患者中有6例在出现症状后4个月至7年发展为典型硬皮病。对6例患者进行了肺病理检查，所有患者均有符合间质性肺炎和纤维化的改变。10例患者中有4例的职业可能使他们接触到潜在的肺毒素。与典型硬皮病患者不同，我们发现的所有10例患者均为男性。结论：即使没有典型的硬皮病皮肤受累，特发性间质性肺病患者也应考虑系统性硬化症。食管造影、抗核抗体检测和甲襞全面检查可能有助于诊断。
