Retrospective evaluation of treatments and clinical responses of patients with systemic sclerosis with lung involvement

Introduction and Aim: Systemic sclerosis (SSc) is a systemic, autoimmune connective tissue disease because of unknown reasons and characterized by excessive collagen production, fibroblast activation and microvasculopathy. Lung involvement in the disease is one of the important internal organ involvements. After gastrointestinal system involvement, the most frequently involved visceral organ is the lung. Although its frequency varies according to the diagnostic method used, Interstitial lung disease (ILD) affects approximately 50% of patients with systemic sclerosis. ILD and pulmonary hypertension (PAH) have been reported as the leading cause of disease- related death in SSc patients in recent years. In our study, it was aimed to evaluate the treatment of lung involvement of the disease, to compare the advantages of immunosuppressive drugs used in the treatment on pulmonary involvement. Method: Systemic sclerosis patients with pulmonary involvement followed by the Rheumatology Department of Cukurova University Medicine Facultuy Balcalı Hospital were included in our study. 88 patients with lung involvement were included in the study. Presence of interstitial lung findings in thorax HRCT was accepted as lung involvement criterion. Routine biochemistry, sedimentation, CRP, complete blood count, serological immunological tests, vitamin D levels, pulmonary function test results, echocardiographic results of the patients were recorded retrospectively from patient files. The analyzes were performed by dividing patients into 3 groups as those receiving MMF, cyclophosphamide and azathioprine. Results: The mean age of the patients was 56.3±12.7 years, 80 (90.9%) of them were female. Lung pattern distribution in patients; 56 (63.6%) had NSIP, 29 (32.9%) had UIP, 2 (2.3%) had fibrotic NSIP, 1 (1.1%) had probable UIP. While the mean PAP was >40 in 26 patients (29.5%) with ECHO, the diagnosis of PAH was confirmed by right heart catheterization in 19 (21.6%) patients. While no significant difference was found in the PFT values of the patients who received azathioprine and MMF treatments, before and after the treatment however the post-treatment FEV1/FVC and DLCO values were found to be lower than the pre-treatment values in the patients who received cyclophosphamide treatment (p<0.05). It was determined that 11 (12.5%) of the patients died during follow-up. When the causes of death were examined, it was determined that 3 patients died due to COVID-19 infection, 4 patients died due to pulmonary hypertension, and 4 patients died due to lung involvement. Discussion and Conclusion: Systemic sclerosis is a disease with a high mortality rate. Especially since pulmonary involvement plays a major role in mortality, diagnosis, screening and treatment are very important in terms of pulmonary. In our study, we found that in cyclophosphamide, group, the reduces in the respiratory function tests were more prominent than the micophenolate patient group and azathiopurine patient group thıs may be due to the higher percentage of more aggresive and therapy resistan involvement pattern in cyclophosphamide group We think that there should be more detailed treatment algorithms for SSc -ILD, therefore, many randomized controlled studies should be conducted on this subject. Keywords: İnterstitial lung disease, Pulmonary hypertension, Systemic sclerosis