AB0093 CLINICAL AND SEROLOGICAL CHARACTERISTICS OF SYSTEMIC SCLEROSIS: EXPERIENCE FROM A TERTIARY CARE CENTER IN PAKISTAN

Abstract

Background: Systemic sclerosis (SS) is less studied Connective tissue disease in our population. It is characterized by different manifestations which if left undiagnosed and untreated lead to serious complications. The hallmark of this disease is fibrosis of various organs including skin and also involving pulmonary, gastrointestinal and cardiovascular system. We aim to evaluate the clinical and serological characteristics of SS in our population. In addition, we evaluated the prevalence of Fibromyalgia and Depression in patients with SS. Objectives: We aimed to determine Clinical and Serological Characteristics of Systemic Sclerosis visiting our department of Rheumatology, Fatima Memorial hospital, Lahore. Methods: It is a cross-sectional study of 38 patients visiting outdoor and indoor in Rheumatology department of Fatima Memorial Hospital from September 2019 to January 2020. All systemic sclerosis patients with age 16 years or above were included in this study. After taking informed consent, we filled the performas to record all the required information. We evaluated the clinical, serological and radiological features of Systemic Sclerosis. Results: A total of 38 patients were included in the study with mean age was 34.47 ± 1.53 years. Female gender comprised of 32(84.2%), with male to female ratio of 1:5.3. Thirty-four (89.5%) had developed Raynaud phenomenon and sclerodactyly was found in 34(89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Skin tightness proximal to elbow was present in 9 (23.7%). Microstomia (decreased mouth opening) was present in 32(84.2%) patients. Twenty-eight (73.7%) patients had arthritis.Respiratory symptoms comprised of shortness of breath in 36(94.7%), palpitation in 24 (63.2%) and chestpain in 7(18.4%). Gastrointestinal (GI) symptoms comprised of regurgitation in 31 (81.5%) and dysphagia in 14 (36.8%). Interstitial lung disease was present in 30 (78.9%) patients, with higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients, with significantly higher prevalence in diffuse disease (57.1%), that was secondary to interstitial lung disease and in limited disease it was found in (11.8%) (p<0.01). Thirty (78.9%) patients were found to have restricted disease on pulmonary function tests. Obstetric history showed a higher prevalence of primary infertility in at least 6 (15.8%) patients, with significantly higher prevalence in limited systemic sclerosis disease as compared to diffuse disease (23.5% vs 9.5%, and p=0.05). Fibromyalgia diagnosed as per ACR criteria was present in 7 (18.4%) patients, and depression assessed by Hospital Anxiety and depression (HADS) score was present in 10 (26.3%) patients. Anti nucleic acid antibody (ANA) was found positive in 30 (78.9%) patients. Anti Scl-70 antibodies were in 24 (63.2%) patients, with significant association with diffuse disease (85% vs 35.3% and p<0.01), while anti centromere antibodies were present in 20 (52.6%) patients; significantly higher in limited disease (94.2% vs 19.0%, and p<0.01). Conclusion: Scleroderma is a very important, autoimmune multisystem disease. It has female preponderence. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of variable course and disease severity. Interstitial lung disease and pulmonary hypertension were the most important complication found in our patients which has poor prognosis. So, It is imperative to early diagnose and treat the disease manifesations to prevent future complications.