A study on clinico immuno pathological correlation of skin and pulmonary involvement in systemic sclerosis

INTRODUCTION: Systemic sclerosis is a chronic autoimmune connective tissue disease of unknown etiology involving multiple systems. It is characterized by significant dysfunction of the microvasculature, immune system and connective tissue. The currently used classification of Systemic Sclerosis is by the extent of skin involvement1. The extent of skin disease correlates with the disease course. Though many internal organs are involved, lung involvement is the major cause of morbidity and mortality in SSc. While some studies regard skin involvement as a surrogate marker for pulmonary involvement, there are studies that have shown improvement of sclerosis occurring spontaneously or as a result of treatment and therefore it does not reflect the pulmonary fibrosis. In addition several cutaneous features have been found to be associated with clinical and serological manifestations in systemic sclerosis. In a recent study2 elevated serum level of MMP-12 correlated with the severity of skin fibrosis and activity of interstitial lung disease in systemic sclerosis, suggesting the common pathogenesis between them. So the skin can be useful marker for early diagnosis and to assess pulmonary involvement. AIMS AND OBJECTIVES: 1. To study the skin and pulmonary manifestations in Systemic Sclerosis. 2. To study the correlation of the clinical, pathological, immunological features of skin and pulmonary involvement in Systemic Sclerosis. MATERIALS AND METHODS: SUBJECTS: Patients attending the Rheumatology Care Centre (RCC) outpatient and inpatient of Rajiv Gandhi Government General Hospital, Chennai were recruited from the period of June 2011 to February 2013. 55 eligible cases who fulfilled the inclusion criteria were enrolled. All subjects gave a written informed consent to enroll in this study. The Ethical committee approval was obtained. INCLUSION CRITERIA: American College of Rheumatology preliminary classification criteria. Major criteria or two minor criteria for diagnosis. Major criteria: Scleroderma proximal to the metacarpophalangeal joints. Minor Criteria: 1. Sclerodactyly. 2. Digital pitting scars or loss of finger pad substance. 3. Bibasilar pulmonary fibrosis. EXCLUSION CRITERIA Overlap syndrome, mixed connective tissue disease, other scleroderma spectrum disorders. RESULTS: Total number of cases were 55. The mean age was 35.5 years. The range was from 20-56 years. Majority were females 49 (89.1%) while males were 6 (10.9%). The mean disease duration was 3.1 years with range 4 months to 10 years. CONCLUSION: In this study on Systemic sclerosis there was a female gender Predominance (8:1). • The limited cutaneous SSc were more than diffuse cutaneous type in this study. • There was positive correlation between disease duration and PHT. • 43.6% of the study group were in the 7-15 MRSS Range. • Presence of salt and pepper had significant association with MRSS in this study. • Dyspnea was the most common respiratory symptom and it correlated positively with MRSS. • The MRSS was significantly associated with presence of ILD in the study group. • ILD was more common in diffuse cutaneous type and the mean MRSS was significantly associated with ILD in diffuse cutaneous type. • There was no association between MRSS and PHT in this study. • There was significant association between MRSS and the Medsger disease severity of lung. • Digital pitted scars and Raynaud‟s Phenomenon positively correlated with ILD in this study group. • ANA positivity was seen in 80% of the cases