Demographic characteristics and clinical manifestations of interstitial lung disease with systemic sclerosis in eastern part of Libya

Fathi Elbraky, Khaled D. Alsaeiti, Fathiyah M Aboulqasim, Saleh Alawgali
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Abstract

Introduction: Pulmonary involvement, such as Interstitial Lung Disease (ILD) and Pulmonary Hypertension (PH ), accounts for significant morbidity and is the leading cause of Systemic sclerosis (SSc)-related morbidity and mortality. The aim of the current study was to study the frequency of occurrence of ILD in SSc and to describe the clinical and radiological picture of pulmonary involvement in SSc. Patients and Methods: Forty patients attending different rheumatology clinics in eastern part of Libya between January 2018 and September 2020 were included. Basic details including age, gender, disease duration, detailed history, and clinical examination were noted. Autoimmune profiling included rheumatoid factor, antinuclear antibodies, and anti-Scl-70 antibodies. Pulmonary function test, chest X-ray (CXR), and high-resolution computed tomography of the chest (HRCT) in all patients were recorded. Data presented either as frequencies and percentages or as means ± standard deviation. Results: The male: female ratio was 1:9 with a mean age of 37.5 ± 9.6 years and duration of illness 6 ± 4 years. diffuse cutaneous SSc was seen in 62.5% of the patients. 77.5% of the participants had bilateral crepitation and 57.5% had loud P2. Presenting complaints included gastrointestinal reflux in 72.5%, digitalis ulcerations in 40%, and synovitis/arthritis of all patients. Other comorbidities included congestive heart failure in 12.5%, PH in 15%, and renal impairments in 7.5% of all patients. Anti-Scl-70 antibody was the most common in all patients (45%), followed by anti-centromere Ab (25%), anti-U3 RNP (10%), and anti-U1 RNP (5%). 72.5% of the participants had reticulonodular shadows on CXR. HRCT showed honeycombing as the predominant finding (37.5%). Echocardiograms showed that 15% of all patients have signs of PH. Duration of disease, dyspnea, cough, bilateral crepitations, and CXR were found to be significantly associated with extensive ILD (P < 0.05). Conclusion: ILD is a serious complication of SSc, it is more common among patients with dcSSc. Chest HRCT is very sensitive to detect ILD. A significant association was found in Libyan patients between the severity of ILD and the duration of disease, dyspnea, cough, bilateral crepts, and CXR.
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利比亚东部间质性肺病伴系统性硬化症的人口学特征和临床表现
肺受累,如间质性肺疾病(ILD)和肺动脉高压(PH),占显著发病率和系统性硬化症(SSc)相关发病率和死亡率的主要原因。本研究的目的是研究SSc中ILD的发生频率,并描述SSc肺部受累的临床和影像学表现。患者和方法:纳入了2018年1月至2020年9月期间在利比亚东部不同风湿病诊所就诊的40名患者。记录基本细节,包括年龄、性别、病程、详细病史和临床检查。自身免疫分析包括类风湿因子、抗核抗体和抗scl -70抗体。记录所有患者的肺功能检查、胸部x线(CXR)和高分辨率胸部计算机断层扫描(HRCT)。数据以频率和百分比或平均值±标准差表示。结果:男女比例为1:9,平均年龄37.5±9.6岁,病程6±4年。62.5%的患者可见弥漫性皮肤SSc。77.5%的参与者有双侧震颤,57.5%的参与者有响亮的P2。主诉包括72.5%的胃肠道反流、40%的洋地黄溃疡和所有患者的滑膜炎/关节炎。其他合并症包括12.5%的充血性心力衰竭,15%的PH和7.5%的肾损害。抗scl -70抗体在所有患者中最常见(45%),其次是抗着丝粒Ab(25%),抗u3 RNP(10%)和抗u1 RNP(5%)。72.5%的参与者在CXR上有网状结节影。HRCT主要表现为蜂窝状病变(37.5%)。超声心动图显示15%的患者有ph体征。病程、呼吸困难、咳嗽、双侧心悸和CXR与广泛的ILD显著相关(P < 0.05)。结论:ILD是SSc的严重并发症,在dcSSc患者中更为常见。胸部HRCT对ILD检测非常敏感。在利比亚患者中,发现ILD的严重程度与病程、呼吸困难、咳嗽、双侧爬行和CXR之间存在显著关联。
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