Farrokh Donya, Abbasi Bita, Fallah-Rastegar Yalda, Mirfeizi Zahra
Department of Radiology, Mashhad University of Medical Sciences, Mashhad, Iran.
Department of Rheumatology, Mashhad University of Medical Sciences, Mashhad, Iran.
Tanaffos. 2015;14(3):193-200.
Systemic sclerosis (SS) is a collagen vascular disease of unknown etiology that is characterized by connective tissue abnormalities. This study aimed to evaluate the extra-pulmonary manifestations of SS on chest high resolution computed tomography (HRCT).
The medical records of patients with SS who presented to our hospital in a 10-year period were retrospectively reviewed. Forty patients with SS were included in this study. The extra pulmonary manifestations of SS were evaluated in these patients, including esophageal involvement, pulmonary arterial dilatation, pleural abnormalities, pericardial disease and mediastinal lymph node involvement.
The most common extra-pulmonary manifestation was esophageal dilatation, which was detected in 70% of the cases followed by pleural involvement. Pulmonary arterial dilatation was seen in 20%, pleural involvement in 40%, pericardial involvement in 40% and mediastinal lymphadenopathy in 30%. The most common pleural abnormality was diffuse pleural thickening and the most common pericardial abnormality was pericardial effusion. There was an association between the severity of lung fibrosis with the incidence of esophageal dilatation and pulmonary arterial hypertension (PAH) in our series. Patients with SS and interstitial lung disease (ILD) who had PAH, had more severe lung fibrosis than those without PAH.
Patients with SS may have a variety of extra-pulmonary manifestations, which can be detected using HRCT. Our study evidenced that HRCT was useful for detecting extra-pulmonary findings of SS such as esophageal dysmotility and dilatation, enlargement of main pulmonary artery and PAH, pleuropericardial involvement and mediastinal lymphadenopathy.
系统性硬化症（SS）是一种病因不明的胶原血管疾病，其特征为结缔组织异常。本研究旨在评估胸部高分辨率计算机断层扫描（HRCT）上SS的肺外表现。
回顾性分析我院10年间收治的SS患者的病历。本研究纳入了40例SS患者。对这些患者的SS肺外表现进行评估，包括食管受累、肺动脉扩张、胸膜异常、心包疾病和纵隔淋巴结受累情况。
最常见的肺外表现是食管扩张，70%的病例中可检测到，其次是胸膜受累。肺动脉扩张见于20%的病例，胸膜受累见于40%，心包受累见于40%，纵隔淋巴结肿大见于30%。最常见的胸膜异常是弥漫性胸膜增厚，最常见的心包异常是心包积液。在我们的系列研究中，肺纤维化的严重程度与食管扩张和肺动脉高压（PAH）的发生率之间存在关联。患有PAH的SS和间质性肺病（ILD）患者比未患PAH的患者有更严重的肺纤维化。
SS患者可能有多种肺外表现，可通过HRCT检测到。我们的研究证明，HRCT有助于检测SS的肺外表现，如食管运动障碍和扩张、主肺动脉增粗和PAH、胸膜心包受累及纵隔淋巴结肿大。
