P128 Clinical and immunological effects of treatment with S2K guidelines (2020) on 72 patients with pemphigus: a pre–post study

Abstract

Pemphigus is a rare, potentially fatal autoimmune blistering disorder. Although systemic corticosteroids have improved survival, they are associated with significant long-term adverse effects. The 2020 European Academy of Dermatology and Venereology S2K guidelines provide evidence-based management; however, data evaluating outcomes using these recommendations are limited, prompting this study to assess their efficacy and safety. We aimed to study the effect of treatment following the updated S2K European guidelines in pemphigus using the Pemphigus Disease Area Index (PDAI) and anti-desmoglein (Dsg)1 and anti-Dsg3 antibody levels. Adverse effects of the treatment were monitored. This was a pre–post quasiexperimental study conducted over 18 months, with a sample size of 72. PDAI was recorded at baseline, month 3 and month 6. Anti-Dsg1 and ant-Dsg3 antibodies were measured at baseline and at month 6. Adverse effects were monitored at baseline and on follow-up. The mean age of the patients was 43.4 years (SD 13.2), and the male-to-female ratio was 0.89 : 1 (34 male and 38 female). Pemphigus vulgaris (PV) constituted the most cases (85%), followed by pemphigus foliaceous (9%), pemphigus vegetans (3%) and pemphigus erythematosus (3%). At baseline, 53% of patients had mild, 39% moderate and 8% severe disease. Mild PV showed a PDAI reduction of 42.8% with steroids plus ­s­teroid-­sparing agents and 56% with steroids plus rituximab at 3 months, sustained at 6 months. Moderate PV improved by 22% and 28.8%, respectively; severe PV (rituximab group) showed 42.5% reduction. Pemphigus foliaceous and pemphigus erythematosus showed substantial improvement with steroid–dapsone or rituximab. Steroids plus rituximab led to greater Dsg1 reduction in mild PV (69% vs. 26%). Dsg1 correlated well with disease severity; Dsg3 showed variable correlation. Infusion reactions occurred in 16% of rituximab-treated patients; metabolic complications and infections were noted. In the present study, the clinical and immunological efficacy of the treatment was measured based on PDAI and anti-Dsg1 and anti-Dsg3 antibody levels. There was a significant reduction in all parameters at month 6 post-treatment compared with baseline, with discordance in rare variants. Minor adverse effects were observed with the treatment.