ارزیابی شدت بیماری در 40 بیمار مبتلا به اسکلرودرمی

The aim of this study was test a new developed severity for individual organ system involvment in 40 patients with systemic sclerosis (SSc, scleroderma). In this study used from a new developed disease severity scale published by an international study group for determination of severity grade in 9 organ system, general, skin, peripheral vascular, joint/tendon, skeletal muscle, gasterointestinal, lung, heart and kidney in 40 patients with systemic sclerosis in firoozgar hospital. Severity scale graded from 0(no documented involvement) to 4(end stage disease). The data required for scale complation are available in data bank of systemic sclerosis (SSc) for all of our patient in firoozgar hospital. The mean age was 40±14.5 years. Female to male ratio 9/1. Mean of total skin score in all patients was 10.7(based on modified rodnan score). Mean duration of first symptoms to time of study was 3.4 years. Normal grade of severity seen different organ system. Severly ill patients contain 1:4 of patients. Sever to end stage disease seen in 2.5% patient with cardiac and 20% of patient with lung involvment. This long term (>5 years) study of disease severity show relativly 1:5 patients developed severe end stage lung disease that approprait management at this time is cardiopulmonary transplant so the center for cardiopulmonary transplanation must be informed to provide facility for these cases.