Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose XXXX in early @entity7087 trial.

@entity1 @entity7242 @entity1289 @entity229 @entity1283 @entity2361 @entity2631 @entity425 @entity1159 @entity7087
OBJECTIVE: The reported frequency of @entity2631 M01-R renal crisis ( @entity7242 ) in @entity7087 (SSc; @entity2631 ) is 15-20%. Early use of @entity1289 ( @entity1289 ) inhibitors has markedly improved outcome. The present analysis reexamines the prognostic factors for and outcome of @entity7242 in a prospective cohort of @entity1 with early diffuse SSc. METHODS: We retrospectively evaluated the cohort of SSc @entity1 who participated in the High-Dose Versus Low-Dose @entity2361 in Early Diffuse SSc trial. @entity1 with @entity7087 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 @entity1 entered the observation period a mean +/- SD of 0.8 +/- 0.3 years after onset of SSc. @entity7242 occurred in 18 @entity1 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 @entity1 died (mean +/- SD 0.6 +/- 0.9 years after @entity7242 onset). Baseline characteristics that predicted @entity7242 included a modified Rodnan skin thickness score of >or=20 (P < 0.01), enlarged cardiac silhouette on radiograph (P = 0.04), @entity1159 (wrist, elbow, knee) (P = 0.008), and @entity229 use at entry (P = 0.01). Baseline characteristics that did not predict @entity7242 included age, sex, race, Health Assessment Questionnaire score, fist closure, handspread, lung involvement, @entity425 , 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 @entity7242 . CONCLUSION: @entity7242 occurred in 13% of @entity1 soon (mean 11 months) after entry into the cohort. Predictors of @entity7242 identified in this study included higher than average skin score, @entity229 use at study entry, @entity1159 , and @entity1283 . Our data suggest, however, that low-dose @entity229 alone was not associated with the onset of @entity7242 , except in the appropriate clinical setting. Although @entity1289 inhibitors and dialysis are now readily available, @entity7242 continues to be associated with poor survival (in this study, 50% of @entity1 with @entity7242 died).
