Robust staging of disease activity for pemphigus vulgaris

In this study a robust and simple classification into different stages of pemphigus, based on a set of therapeutic benchmarks including a definition of disease activity, is proposed. This staging method was validated against ELISA titres for autoantibodies against desmoglein-1 and -3. The proposed staging method was applied a priori to five newly diagnosed pemphigus patients in the course of their disease. The ELISA titres for desmoglein-1 and -3, as well as the indirect immunofluorescence titres were cross tabulated against the a priori defined disease stages. ELISA titres of desmogleins seemed to show a good correlation with our proposed staging system, better than the indirect immunofluorescence titres. However, due to the small sample size, we could not perform a statistical test. We conclude that the proposed staging system for disease activity in pemphigus vulgaris is useful for international uniform monitoring pemphigus vulgaris. Submitted for publication