Behera Ajoy K, Sharma Pratibha, Tg Ranganath, Kumar Vikas, Pati Saroj K, Sinha Kulshreshth
Pulmonary Medicine, All India Institute of Medical Sciences, Raipur, Raipur, IND.
Microbiology, Shri Balaji Institute of Medical Science, Raipur, IND.
Cureus. 2024 Jul 28;16(7):e65579. doi: 10.7759/cureus.65579. eCollection 2024 Jul.
Systemic sclerosis (SSc) is a multisystem autoimmune disorder characterized by dysregulated innate and adaptive immunity. Interstitial lung disease (ILD) is a common and serious complication of SSc, often leading to significant morbidity and mortality. Consistent demographic characteristics that aid in the early diagnosis of ILD in SSc are lacking. This study aims to identify clinical and demographic parameters associated with ILD in SSc patients and assess the safety and tolerability of nintedanib with other immunosuppressants.
This study is a subgroup analysis of data from the ILD clinic at All India Institute of Medical Sciences Raipur, collected between January 2022 and January 2024. We assessed the clinical and demographic profiles, high-resolution computed tomography thorax patterns, autoantibody profiles, lung function, and treatments used in the patients.
We enrolled 57 patients with SSc-associated ILD. The mean age of the participants was 39.0 ± 11.1 years, with 53 (92.9%) being women. The mean body mass index was 20.4 ± 4.32 kg/m². Dyspnea was the most common symptom, followed by skin tightening and cough. Antinuclear antibody tests were positive in 92.9% of patients, and anti-Scl-70 antibodies were positive in 57.9%. Rheumatoid arthritis-SSc overlap was observed in 15.8% of patients. The mean predicted forced vital capacity was 46.5 ± 19.9%, the mean predicted total lung capacity was 64.5 ± 20.4%, and the mean predicted diffusing capacity for carbon monoxide was 46.2 ± 15.7%. The mean six-minute walk distance was 360.3 ± 81.2 meters, and the mean King's Brief Interstitial Lung Disease score was 63.9 ± 10.7. Common radiological abnormalities included ground-glass opacities in 57.8%, traction bronchiectasis in 43.8%, and honeycombing in 28.07%. The predominant ILD pattern was nonspecific interstitial pneumonia. Patients received a combination of prednisolone (5 mg/day) with mycophenolate mofetil (63.2%), hydroxychloroquine (17.5%), cyclophosphamide (12.3%), and methotrexate (7.02%). Nintedanib, the only antifibrotic used, was administered to 17 (29.8%) patients.
ILD is relatively common in SSc, particularly in patients with diffuse cutaneous SSc and those with anti-topoisomerase antibodies. Female patients comprised the predominant population in this study. Patients tolerated mycophenolate mofetil and cyclophosphamide well. Nintedanib was the only antifibrotic used, and all patients tolerated the combination of antifibrotics and immunosuppressants well. Early diagnosis is crucial to slow disease progression and preserve lung function. Our results highlight the need for vigilant screening in high-risk groups and suggest that MMF, cyclophosphamide, and nintedanib can be safely incorporated into treatment regimens, offering a potential strategy to improve patient outcomes.
系统性硬化症（SSc）是一种多系统自身免疫性疾病，其特征为先天性和适应性免疫失调。间质性肺疾病（ILD）是SSc常见且严重的并发症，常导致显著的发病率和死亡率。目前缺乏有助于早期诊断SSc患者ILD的一致人口统计学特征。本研究旨在确定与SSc患者ILD相关的临床和人口统计学参数，并评估尼达尼布与其他免疫抑制剂联合使用的安全性和耐受性。
本研究是对印度赖布尔全印医学科学研究所ILD诊所2022年1月至2024年1月收集的数据进行的亚组分析。我们评估了患者的临床和人口统计学资料、胸部高分辨率计算机断层扫描模式、自身抗体谱、肺功能及所用治疗方法。
我们纳入了57例SSc相关ILD患者。参与者的平均年龄为39.0±11.1岁，其中53例（92.9%）为女性。平均体重指数为20.4±4.32kg/m²。呼吸困难是最常见的症状，其次是皮肤紧绷和咳嗽。92.9%的患者抗核抗体检测呈阳性，57.9%的患者抗Scl-70抗体呈阳性。15.8%的患者观察到类风湿关节炎-SSc重叠。预计用力肺活量平均为46.5±19.9%，预计总肺容量平均为64.5±20.4%，预计一氧化碳弥散量平均为46.2±15.7%。六分钟步行距离平均为360.3±81.2米，金氏简易间质性肺疾病评分平均为63.9±10.7。常见的放射学异常包括57.8%的磨玻璃影、43.8%的牵拉性支气管扩张和28.07%的蜂窝状改变。主要的ILD模式为非特异性间质性肺炎。患者接受了泼尼松龙（5mg/天）联合霉酚酸酯（63.2%）、羟氯喹（17.5%）、环磷酰胺（12.3%）和甲氨蝶呤（7.）的治疗。唯一使用的抗纤维化药物尼达尼布用于17例（29.8%）患者。
ILD在SSc中相对常见，尤其是在弥漫性皮肤型SSc患者和抗拓扑异构酶抗体阳性患者中。女性患者在本研究中占主要人群。患者对霉酚酸酯和环磷酰胺耐受性良好。尼达尼布是唯一使用的抗纤维化药物，所有患者对抗纤维化药物和免疫抑制剂联合治疗耐受性良好。早期诊断对于减缓疾病进展和保护肺功能至关重要。我们的结果强调了对高危人群进行警惕筛查的必要性，并表明霉酚酸酯、环磷酰胺和尼达尼布可以安全地纳入治疗方案，为改善患者预后提供了一种潜在策略。
