Predictors and outcomes of scleroderma renal crisis: The high‐dose versus low‐dose D‐penicillamine in early diffuse systemic sclerosis trial

Abstract

Objective. The reported frequency of scleroderma M01-R renal crisis (SRC) in diffuse systemic sclerosis (SSc; scleroderma) is 15-20%. Early use of angiotensinconverting enzyme (ACE) inhibitors has markedly improved outcome. The present analysis reexamines the prognostic factors for and outcome of SRC in a prospective cohort of patients with early diffuse SSc.Methods. We retrospectively evaluated the cohort of SSc patients who participated in the High-Dose Versus Low-Dose D-Penicillamine in Early Diffuse SSc trial. Patients with diffuse cutaneous scleroderma were enrolled if their disease duration was <18 months. Because the trial failed to show a difference between treatment groups, the data were pooled.Results. One hundred thirty-four SSc patients entered the observation period a mean ؎ SD of 0.8 ؎ 0.3 years after onset of SSc. SRC occurred in 18 patients a mean ؎ SD of 0.9 ؎ 1.1 years after entry. During a mean ؎ SD 4.0 ؎ 1.1 years of followup after entry, 9 of the 18 patients died (mean ؎ SD 0.6 ؎ 0.9 years after SRC onset). Baseline characteristics that predicted SRC included a modified Rodnan skin thickness score of >20 (P < 0.01), enlarged cardiac silhouette on radiograph (P ‫؍‬ 0.04), large joint contractures (wrist, elbow, knee) (P ‫؍‬ 0.008), and prednisone use at entry (P ‫؍‬ 0.01). Baseline characteristics that did not predict SRC included age, sex, race, Health Assessment Questionnaire score, fist closure, handspread, lung involvement, muscle weakness, erythrocyte sedimentation rate, and platelet count. In 5 of 10 subjects for whom at least 2 sequential skin scores were available, skin scores increased significantly (P ‫؍‬ 0.012) in the 6 months before onset of SRC.Conclusion. SRC occurred in 13% of patients soon (mean 11 months) after entry into the cohort.

Interstitial Lung Disease in Systemic Sclerosis: Diagnosis and Management

“…In general, these drugs are avoided because of the well known risk of scleroderma renal crisis. This phenomenon has been well documented [53] and has been reported to occur at mean doses as low as 7.4 mg [54]. However, in most clinical trials, use of prednisone was permitted with the drug under investigation.…”

Prevalence of renal involvement in Indian patients with systemic sclerosis

“…GFR was 61-90 ml/min CKD-2 40 (45.97) calculated using Cokcorft-Gault formula in our 30-60 ml/min CKD-3 25 (28.73) CKD-4 1 (1.14) study, which has been found to correlate well < 15 ml/min (or dialysis) CKD-5 Nil with GFR, which is measured using a radioisotope clearance method in a recent one early study from India by Desai et al Renal biopsy has revealed primary nifedipine therapy for the treatment of Significant renal disease occurs mostly in However, these 2 groups were incomparable patients with diffuse cutaneous because of the small numbers of patients in scleroderma. [13] We therefore compared the the group without nifedipine therapy. mean GFR in diffuse SSc patients with limited SSc patients.…”

Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: Application of a latent linear trajectory model

“…For example, investigators in the High-Dose Versus Low-Dose DPenicillamine in Early Diffuse Systemic Sclerosis Trial reported that a high baseline skin score predicted mortality and scleroderma renal crisis in the subsequent 4 years and was associated with baseline heart involvement (3,4). In addition, Steen and Medsger reported that patients who achieved improvement in the skin Supported by the Raynaud's and Scleroderma Association, UK.…”