Cox D, Conant E, Earle L, Newman J, Kahaleh B, Jimenez S, Varga J
Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA 19107-5541, USA.
J Rheumatol. 1995 May;22(5):881-5.
To describe the clinical, radiologic, and pathologic features of coexistent systemic sclerosis (SSc) and sarcoidosis, 2 conditions of unknown cause associated with altered cellular immune response.
We reviewed clinical information, results from laboratory and radiologic studies, and lung or lymph node biopsy samples of 7 patients with concurrent SSc and sarcoidosis evaluated at 2 academic referral centers between 1989 and 1993.
Each patient fulfilled American College of Rheumatology criteria for the classification of SSc. SSc and sarcoidosis developed simultaneously in 4 patients, whereas in 3 others sarcoidosis was diagnosed more than 6 years after the onset of SSc. The onset of sarcoidosis was characterized by fever, weight loss, or increasing respiratory symptoms. Each patient had radiographic evidence of intrathoracic lymphadenopathy and/or interstitial lung disease. Examination of lung or lymph node biopsies demonstrated noncaseating granulomas. Treatment with corticosteroids was associated with improved lung function.
Since sarcoidosis coexists with SSc more frequently than previously suggested, it should be considered in patients with SSc presenting with new pulmonary symptoms. Recognizing sarcoidosis in patients with SSc is important, since these patients may benefit from corticosteroid therapy.
描述系统性硬化症（SSc）与结节病并存的临床、放射学及病理学特征，这两种病因不明的疾病均与细胞免疫反应改变相关。
我们回顾了1989年至1993年间在2个学术转诊中心评估的7例同时患有SSc和结节病患者的临床信息、实验室及放射学研究结果，以及肺或淋巴结活检样本。
每位患者均符合美国风湿病学会的SSc分类标准。4例患者SSc和结节病同时发病，而另外3例患者结节病在SSc发病6年多后才被诊断。结节病的发病以发热、体重减轻或呼吸道症状加重为特征。每位患者均有胸内淋巴结肿大和/或间质性肺病的放射学证据。肺或淋巴结活检检查显示为非干酪样肉芽肿。使用皮质类固醇治疗可改善肺功能。
由于结节病与SSc并存的情况比之前认为的更常见，对于出现新的肺部症状的SSc患者应考虑此病。认识到SSc患者中的结节病很重要，因为这些患者可能从皮质类固醇治疗中获益。
