Ghosh Anirban, Banerjee Avinandan, Biswas Amlan Kanti
Indian J Chest Dis Allied Sci. 2014 Oct-Dec;56(4):231-5.
This study was undertaken to find out the characteristics of clinical, radiological and functional changes affecting the respiratory system in patients with systemic sclerosis (SSc) from eastern India, and the association of these characteristics with pulmonary hypertension.
This was a cross-sectional, observational study involving 46 patients. Other than the routine tests, anti-nuclear antibody (ANA), spirometry, diffusing capacity of lung for carbon monoxide (DLCO) measurement, chest radiograph, high-resolution computed tomography (HRCT) of thorax, 6-minute walk test and echocardiography were done.
Out of a total of 46 patients, 27 patients had diffuse cutaneous SSc (dcSSc) and 19 had limited cuteaneous SSc (lcSSc). Eleven patients had pulmonary hypertension. The HRCT revealed diffuse parenchymal lung disease (DPLD) in 32 (65%) cases. The ANA was positive in 83% cases. Anti-Scl70 was found in 41% of patients with dcSSc and anti-centromere antibody was found in 47% of patients with lcSSc. Spirometry revealed restrictive pattern in 30 patients; 9 had obstruction; and the rest were normal. The DLCO was abnormal in 38 patients. A strong correlation was found between reduction in DLCO and pulmonary artery systolic pressure (PASP). Also, a strong association was observed between a drop of > 4% in oxygen saturation on 6-minute walk test and presence of pulmonary arterial hypertension (PAH).
Majority of the patients with SSc had restrictive lung disease with abnormal DLCO and features resembling non-specific interstitial pneumonia. Nucleolar ANA was predominantly found in patients having PAH. Presence of DPLD had a negative association with presence of anti-centromere antibody. Reduction in DLCO and a fall of > 4% in oxygen saturation on 6-minute walk test may be used as predictors of PAH in asymptomatic individuals.
本研究旨在了解印度东部系统性硬化症（SSc）患者影响呼吸系统的临床、放射学和功能变化特征，以及这些特征与肺动脉高压的关联。
这是一项横断面观察性研究，涉及46例患者。除常规检查外，还进行了抗核抗体（ANA）检测、肺功能测定、肺一氧化碳弥散量（DLCO）测量、胸部X线片、胸部高分辨率计算机断层扫描（HRCT）、6分钟步行试验和超声心动图检查。
46例患者中，27例为弥漫性皮肤型SSc（dcSSc），19例为局限性皮肤型SSc（lcSSc）。11例患者患有肺动脉高压。HRCT显示32例（65%）患者存在弥漫性实质性肺疾病（DPLD）。83%的病例ANA呈阳性。41%的dcSSc患者检测到抗Scl70，47%的lcSSc患者检测到抗着丝点抗体。肺功能测定显示30例患者为限制性模式；9例有阻塞性改变；其余正常。38例患者DLCO异常。发现DLCO降低与肺动脉收缩压（PASP）之间存在强相关性。此外，6分钟步行试验中氧饱和度下降>4%与肺动脉高压（PAH）的存在之间存在强关联。
大多数SSc患者患有限制性肺病，DLCO异常，且具有类似非特异性间质性肺炎的特征。核仁型ANA主要见于患有PAH的患者。DPLD的存在与抗着丝点抗体的存在呈负相关。DLCO降低和6分钟步行试验中氧饱和度下降>4%可作为无症状个体PAH的预测指标。
