Abstract | BACKGROUND: 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.
|
Authors | C T Derk, G Huaman, S A Jimenez |
Journal | The British journal of dermatology
(Br J Dermatol)
Vol. 158
Issue 5
Pg. 1063-8
(May 2008)
ISSN: 0007-0963 [Print] England |
PMID | 18284395
(Publication Type: Journal Article, Research Support, N.I.H., Extramural)
|
Chemical References |
- Antirheumatic Agents
- Penicillamine
|
Topics |
- Antirheumatic Agents
(therapeutic use)
- Cohort Studies
- Dose-Response Relationship, Drug
- Female
- Humans
- Male
- Middle Aged
- Penicillamine
(therapeutic use)
- Retrospective Studies
- Scleroderma, Diffuse
(drug therapy)
- Skin
(drug effects)
|