Clinical and cutaneous profile of patients with systemic sclerosis in a tertiary centre in South India

Abstract

Background and Aims: Systemic sclerosis is an autoimmune disease, of which cutaneous sclerosis is a cardinal feature. Other skin manifestations include Raynaud's phenomenon, digital ulcers, gangrene, flexion contractures, and telangiectasias. It has been noted that the clinical and antibody profile varies within ethnic groups. The objective of the study was to study the clinical profile of patients with systemic sclerosis in a tertiary center in South India and to compare to other ethnic groups. Materials and Methods: Thirty-five patients were recruited in the study period between February 2014 and September 2015. A record of the disease details, systemic manifestations, morphology, and distribution of the cutaneous manifestations and investigations were done by the principal investigator. The severity of cutaneous sclerosis was assessed by modified Rodnan Skin Score (mRSS). Results: Our study had a female preponderance with diffuse type of disease in 91% of patients. The common cutaneous presenting complaints were sclerosis (n = 35, 100%), Raynaud's phenomenon (n = 25, 71.4%), and pigmentary disturbances (n = 33, 94.3%). Respiratory system was the most common system involved, with interstitial lung disease seen in 78.1% (n = 25). A significant association (P = 0.026) was noticed between the increase in mRSS and interstitial lung disease. Conclusion: Pigmentary disturbances were higher in our population, whereas Raynaud's phenomenon, digital ulcers, and telangiectasia were less common than in Western population. A significant association (P = 0.026) was found between mRSS and interstitial lung disease.