Severe organ involvement in systemic sclerosis with diffuse scleroderma

Objective, To determine the natural history and timing of severe involvemen t of the kidney, heart, lung, gastrointestinal (GI) tract, and skin in pati ents with systemic sclerosis (SSc) and diffuse cutaneous involvement, Methods. This study used the Pittsburgh Scleroderma Databank and included p atients with diffuse scleroderma who were seen between January 1, 1972 and December 31, 1995, Patients had frequent follow-ups, and a 95% accountabili ty for these patients was maintained. Severe organ involvement was defined as the presence of any of the following: 1) in the kidney, scleroderma "ren al crisis"; 2) in the heart, cardiomyopathy, symptomatic pericarditis, or a n arrhythmia requiring treatment; 3) in the lung, pulmonary fibrosis on che st radiograph and a forced vital capacity of <55% of predicted; 4) in the G I tract, malabsorption, repeated episodes of pseudoobstruction, or severe p roblems requiring hyperalimentation; and 5) in the skin, a modified Rodnan skin score >40, The timing from disease onset to survival for each case of severe organ involvement was determined, Results. Of the 953 patients with diffuse scleroderma, kidney involvement d eveloped in 177 (19%), heart involvement in 143 (15%), lung involvement in 151 (16%), GI tract involvement in 74 (8%), and skin involvement in 233 (24 %), Severe skin and kidney involvement occurred during the first 3 years in 70% of those who ever developed these problems throughout a mean of 10 yea rs of followup, Severe heart, lung, and GI tract involvement developed duri ng the first 3 years in 45-55% of those who were ever affected. The surviva l of patients with severe organ involvement was poor. The 9-year cumulative survival rate of all patients with severe organ involvement was 38%, compa red with 72% in patients without such involvement (P < 0.0001). Conclusion. This study demonstrates that severe organ involvement in SSc pa tients with diffuse scleroderma most often occurs early in the course of th e disease. Survival for patients with severe organ involvement is markedly reduced, Patients should therefore be monitored very closely during the fir st 3 years of disease for signs and symptoms that may signal the subsequent development of severe organ damage. Potential disease-modifying therapies must be initiated early to modify the natural history of SSc and to improve survival. Patients who survive the first few years without developing seve re organ involvement are less likely to develop such life-threatening invol vement later in the disease course.