Department of Internal Medicine, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Alexandria University, Alexandria, Egypt.
Department of Clinical and Chemical Pathology, Faculty of Medicine, Alexandria University, Alexandria, Egypt.
Curr Rheumatol Rev. 2023;19(2):189-196. doi: 10.2174/1573397118666220818095927.
Pulmonary involvement is the most common leading cause of morbidity and mortality associated with systemic sclerosis. Therefore, identifying the various patterns of pulmonary affection is crucial in the clinical management of these patients. In the current study, we aim to investigate the patterns of interstitial lung disease (ILD) associated with SSc patients (SSc- ILD) and their relation to serologic markers and clinical parameters.
A cross-sectional study was undertaken on thirty-four adult SSc patients who met the 2013 ACR/EULAR criteria for SSc and Forty healthy controls of matched age and sex. The patients were subjected to history taking, clinical examination, skin assessment using the modified Rodnan Skin Score (mRSS), chest x-ray (CXR), pulmonary function test (PFTs), and high resolution computed tomography of the chest (HRCT). Routine laboratory tests were conducted in addition to immunologic tests and an enzyme-linked immunosorbent assay (ELISA) to determine the IL-33 level.
ILD was found in 23 SSc patients (67.6%); 20 patients had diffuse type while 3 patients had limited type. Non-specific interstitial pneumonia (NSIP) was found in 56.5%, usual interstitial pneumonia (UIP) was found in 21.7%, pleuroparenchymal fibroelastosis (PPFE) was found in 8.7%, and organizing pneumonia (OP) with the mixed pattern was found in 13% of SSc patients. Additionally, the mean IL-33 level in SSc patients was 98±12.7 compared to 66.2±10.6 in the control group (p < 0.001), with ILD patients having a significantly higher level (101.7±13.4) than those without (90.4±6.2), and a strong positive correlation with mRSS.
Even in asymptomatic patients with SSc, ILD is prevalent, with NSIP being the most common pattern. IL-33 could be considered a potential biomarker for predicting the presence of ILD in SSc patients.
肺部受累是系统性硬化症（SSc）相关发病率和死亡率的最常见主要原因。因此，确定各种肺部受累的模式对于这些患者的临床管理至关重要。在目前的研究中，我们旨在研究与 SSc 患者（SSc-ILD）相关的间质性肺病（ILD）的模式及其与血清标志物和临床参数的关系。
对 34 名符合 2013 年 ACR/EULAR SSc 标准的成年 SSc 患者和 40 名年龄和性别匹配的健康对照组进行了横断面研究。对患者进行了病史采集、临床检查、使用改良罗德纳皮肤评分（mRSS）进行皮肤评估、胸部 X 线（CXR）、肺功能检查（PFTs）和胸部高分辨率计算机断层扫描（HRCT）。除了进行常规实验室检查外，还进行了免疫检查和酶联免疫吸附试验（ELISA）以确定 IL-33 水平。
ILD 在 23 名 SSc 患者中发现（67.6%）；20 名患者为弥漫性，3 名患者为局限性。非特异性间质性肺炎（NSIP）占 56.5%，寻常性间质性肺炎（UIP）占 21.7%，胸膜肺弹性纤维增生症（PPFE）占 8.7%，机化性肺炎（OP）混合模式占 13%。此外，SSc 患者的平均 IL-33 水平为 98±12.7，而对照组为 66.2±10.6（p<0.001），ILD 患者的水平显著更高（101.7±13.4）比没有ILD 的患者（90.4±6.2），与 mRSS 呈强正相关。
即使在无症状的 SSc 患者中，ILD 也很常见，其中 NSIP 是最常见的模式。IL-33 可以被认为是预测 SSc 患者存在 ILD 的潜在生物标志物。
