Risk factors for lethal outcome in patients with pemphigus: a retrospective cohort study

Background: Although pemphigus is associated with increased mortality, little is known regarding factors that influence prognosis. Objective: To identify prognostic factors for mortality at five-and 10-year periods after an initial diagnosis of pemphigus. Materials & methods: A retrospective cohort study was performed. Data were collected for all patients with a new diagnosis of pemphigus between 1990 and 2011, who were actively followed for at least five years at our centre. The endpoint was overall survival at five and 10 years after pemphigus diagnosis. Based on the test sample, associations between clinical variables and overall survival were assessed using univariate and multivariate analyses. Results: A total of 184 patients were included in the study (mean age: 52.2 +/- 16.1). The major risk factors for lethal outcome at both five and 10 years after diagnosis were age >= 65 years at diagnosis (with a multivariate hazard ratio [HR] of 5.9 and 4.2 at five and 10 years, respectively) and neurological disease at diagnosis (with a multivariate HR of 5.4 and 5.1 at 5 and 10 years, respectively). Diabetes mellitus was an independent risk factor for mortality at five years (multivariate HR: 3.8). Two risk factors were independently associated with lethal outcome at 10 years: serum albumin levels <3.5 g/dL (multivariate HR: 3.3) and lung disease at diagnosis (multivariate HR: 3.8). Mucosal involvement in patients with pemphigus vulgaris was not associated with increased mortality. Conclusions: Prognosis of patients with pemphigus is influenced by older age, hypoalbuminaemia, diabetes mellitus, and neurological and respiratory comorbidities at diagnosis.