Correlation of Immunological Profile with Phenotype and Disease Outcome in Pemphigus

Abstract

There has been no previous clinical-immunological study of pemphigus in Spain. The aim of this study was to perform a retrospective analysis of pemphigus patients who had been followed for a period of 18 years in our centre. We characterized the autoantibody response, compared diagnostic assays and correlated the immunobiological data with phenotype and prognosis. Clinical, epidemiological and immunopathological data were collected from 40 patients. Patients sera were characterized by indirect immunofluorescence and enzyme-linked immunosorbent assay (ELISA). Epidemiological and clinical findings were comparable to other series. Mortality during follow-up was 0% and 6% in pemphigus foliaceus and vulgaris, respectively. Importantly, higher indirect immunofluorescence titres and anti-desmoglein 3 ELISA values of samples from untreated patients correlated significantly with a potentially worse clinical course. Moreover, there was a positive correlation between indirect immunofluorescence titres and anti-desmoglein 3 ELISA levels in pemphigus vulgaris patients. Based on our findings, initial high anti-desmoglein 3 antibodies in pemphigus patients correlate with a more adverse prognosis, which raises the question as to whether a more aggressive initial therapy is indicated in patients with this immunological pattern.