Christine Pauwels

TL;DR: A single cycle of rituximab is an effective treatment for pemphigus and its use should be limited to the most severe types of the disease.

TL;DR: A strong beneficial effect of the mild regimen was observed in patients with moderate BP, with an almost twofold decrease in the risk of death or life-threatening adverse events relative to the standard regimen.

TL;DR: It is found that rituximab therapy can help pemphigus patients even after 6 years, in part by reshaping the B cell repertoire during reconstitution, allowing for long-term effects in addition to the short-term loss of pathogenic B cells.

TL;DR: The results suggest that some drug therapies may be a risk factor for bullous pemphigoid, and the cause of this association should be further investigated.

Abstract: Objective To identify the prognostic factors of bullous pemphigoid (BP). Design Prospective study of patients with BP included in a randomized, controlled trial. Setting Twenty dermatology departments in France. Patients One hundred seventy patients with BP initially treated with a 40-g/d dosage of clobetasol propionate cream (testing sample) and 171 patients initially treated with oral corticosteroids at a dosage of 0.5 or of 1.0 mg/kg per day, depending on the extent of BP (validation samples). Main Outcome Measures The end point was overall survival during the first year after BP diagnosis. From the testing sample, associations of clinical and biological variables with overall survival were assessed using univariate and multivariate analyses. Selected predictors were included in a prognostic model. To verify that these predictors were not dependent on the treatment used, the model was then validated independently on the 2 series of BP patients treated with oral corticosteroids. Results Median age of the BP patients included in the testing sample was 83 years. The 1-year Kaplan-Meier survival rate was 74%. From univariate analysis, the main deleterious predictors were demographic factors (ie, older age and female sex), associated medical conditions (ie, cardiac insufficiency, history of stroke, and dementia), and low Karnofsky score, which is a measure of the patient’s general condition. No factors directly related to BP, in particular extent of cutaneous lesions, were shown to be related to the patients’ prognosis. From multivariate analysis, only older age (P = .02) and low Karnofsky score (P Conclusions The prognosis of patients with BP is influenced by age and Karnofsky score. These predictors are easy to use and should facilitate the management of BP.