Bruno Labeille

TL;DR: Data from this trial suggest that first-line use of rituximab plus short-termprednisone for patients with pemphigus is more effective than using prednisone alone, with fewer adverse events.

TL;DR: This prospective study is the first assessing the incidence and distribution of autoimmune subepidermal bullous disorders that systematically included immunoelectron microscopic data and is large enough to establish bullous pemphigoid as the major autoimmune sub epidermolysis bullosa acquisita disease for the purpose of therapeutic trials.

TL;DR: The presence of 3 of the 4 significant criteria allows the diagnosis of bullous pemphigoid with good validity for both clinical practice and therapeutic trials.

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.