Clinical and serological predictors of relapse in pemphigus: a study of 143 patients.

Abstract

SummaryBackgroundPemphigus is an autoimmune bullous disease mediated by autoantibodies targeting epithelial cell–cell adhesion molecules. Predictors of relapse have not yet been clearly identified.AimsTo identify factors at diagnosis and during follow‐up that could be predictors of relapse.MethodsClinical and immunopathological data at diagnosis, clinical remission and first relapse from patients with pemphigus vulgaris or foliaceus and at least a 36‐month follow‐up were collected retrospectively. Based on the autoantibody profile at diagnosis, three serological patient subsets were devised: (i) anti‐desmoglein (Dsg)1‐positive and anti‐Dsg3‐negative; (iii) anti‐Dsg1‐negative and anti‐Dsg3‐positive; and (iii) anti‐Dsg1‐positive and anti‐Dsg3‐positive.ResultsData from 143 patients were collected. No significant differences were found between relapsers (n = 90) and nonrelapsers (n = 53) for time to remission or for anti‐Dsg1 and anti‐Dsg3 titres at diagnosis and remission. In the analysis of all patients, a higher risk of relapse was found for a body surface area (BSA) score of 3 compared with BSA < 3 (OR = 3.30, 95% CI 1.17–9.28; P = 0.02) and for a positive titre of either anti‐Dsg1 or anti‐Dsg3 autoantibodies at remission compared with both being negative (OR = 2.42, 95% CI 1.21–4.85, P = 0.01). In patients who were anti‐Dsg3‐positive and anti‐Dsg1‐negative at diagnosis, failure to achieve anti‐Dsg3 negativity at clinical remission was a significant predictor of relapse (OR = 7.89, 95% CI 2.06–30.21; P < 0.01). Similarly, failure to achieve anti‐Dsg1 negativity at clinical remission was a significant predictor of relapse in patients with both anti‐Dsg1 and anti‐Dsg3 positivity at diagnosis (OR = 5.74, 95% CI 1.15–28.61; P = 0.03), but not in those who were anti‐Dsg1‐positive/anti‐Dsg3‐negative at diagnosis (OR = 1.08, 95% CI 0.27–4.30; P = 0.91).ConclusionRegardless of pemphigus subtype, autoantibody titre negativity at clinical remission in patients classified based on their anti‐Dsg1 and anti‐Dsg3 profile at diagnosis and BSA were useful tools in predicting relapse.