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A retrospective randomly selected cohort study of D‐penicillamine treatment in rapidly progressive diffuse cutaneous systemic sclerosis of recent onset
Author(s) -
Derk C.T.,
Huaman G.,
Jimenez S.A.
Publication year - 2008
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2008.08452.x
Subject(s) - medicine , rheumatology , retrospective cohort study , cohort , family medicine , library science , computer science
Summary Background Several uncontrolled studies in systemic sclerosis have shown that D‐penicillamine may cause improvement in skin sclerosis, decrease the rate of new visceral organ involvement, and improve overall survival. Objectives To undertake a single‐centre retrospective randomly selected cohort study to examine the effects of D‐penicillamine treatment on skin and visceral organ involvement in patients with rapidly progressive systemic sclerosis of recent onset. Methods Eighty‐four patients with diffuse cutaneous systemic sclerosis who had received D‐penicillamine within 24 months of clinically detectable onset of skin sclerosis were randomly selected from the systemic sclerosis cohort followed at the Scleroderma Center of Thomas Jefferson University. Employing a previously described severity scale, disease severity and skin involvement were compared from initiation of D‐penicillamine to end of study and a correlated matched t ‐test was used to establish statistical significance. Results At a mean ± SD duration of D‐penicillamine therapy of 29·2 ± 5·5 months and at a median dose of 750 mg per day statistically significant improvement in skin ( P < 0·01) and cardiac, pulmonary and renal involvement ( P < 0·05) was observed. At last follow‐up, 17 (20%) patients were still receiving D‐penicillamine, 25 (30%) had discontinued it owing to disease improvement, and 18 (21%) had discontinued it owing to side‐effects. Conclusions In a population of patients with diffuse cutaneous systemic sclerosis, with progressive disease of recent onset, D‐penicillamine treatment at a median dose of 750 mg per day caused a statistically significant reduction in skin involvement and improvement of renal, cardiac and pulmonary involvement.