快樂小藥師 Im pharmacist nichts glücklich

這期的NEJM新資訊，希望可以給天皰瘡的病患一線希望
作者：Pascal Joly, M.D., Ph.D., Hugo Mouquet, Ph.D., Jean-Claude Roujeau, M.D.,...等
Abstr act
 

Background

The combination of multiple cycles of rituximab and intravenous immune globulins has been reported to be effective in patients with severe pemphigus. The aim of this study was to assess the efficacy of a single cycle of rituximab in severe types of pemphigus.

Methods

We studied 21 patients with pemphigus whose disease had not responded to an 8-week course of 1.5 mg of prednisone per kilogram of body weight per day (corticosteroid-refractory disease), who had had at least two relapses despite doses of prednisone higher than 20 mg per day (corticosteroid-dependent disease), or who had severe contraindications to corticosteroids. The patients were treated with four weekly infu-sions of 375 mg of rituximab per square meter of body-surface area. The primary end point was complete remission 3 months after the end of rituximab treatment; complete remission was defined as epithelialization of all skin and mucosal lesions.

Results

Eighteen of 21 patients (86%; 95% confidence interval, 64 to 97%) had a complete re-mission at 3 months. The disease relapsed in nine patients after a mean of 18.9±7.9 months. After a median follow-up of 34 months, 18 patients (86%) were free of dis-ease, including 8 who were not receiving corticosteroids; the mean prednisone dose decreased from 94.0±10.2 to 12.0±7.5 mg per day (P = 0.04) in patients with cortico-steroid-refractory disease and from 29.1±12.4 to 10.9±16.5 mg per day (P = 0.007) in patients with corticosteroid-dependent disease. Pyelonephritis developed in one pa-tient 12 months after rituximab treatment, and one patient died of septicemia 18 months after rituximab treatment. These patients had a profound decrease in the num-ber of circulating B lymphocytes but normal serum levels of IgG.

Conclusions

A single cycle of rituximab is an effective treatment for pemphigus. Because of its po-tentially severe side effects, its use should be limited to the most severe types of the disease. (ClinicalTrials.gov number, NCT00213512.)

相關資訊請參閱：http://blog.pixnet.net/mulicia/post/4097693
http://www.badongo.com/file/4029231