Estimated cut-off values for pemphigus severity classification according to pemphigus disease area index (PDAI), autoimmune bullous skin disorder intensity score (ABSIS), and anti-desmoglein 1 autoantibodies

Abstract

Background Pemphigus is a potentially fatal disease if left untreated. Valid scoring systems and defined cut-off values for classification of patients would help with better management through specified pharmaceutical and non-pharmaceutical treatments. Methods In this study, pemphigus patients who were receiving immunosuppressive treatments and had recent disease relapse were recruited for examination of pemphigus disease area index(PDAI), autoimmune bullous skin disorder intensity score (ABSIS), physician global assessment (PGA), autoimmune bullous disease quality of life (ABQoL), anti-desmoglein 1 (anti-Dsg1), and anti-Dsg3 autoantibody titers from December-2017 to February-2018. Cut-off values were estimated using model-based clustering classification and the 25th and 75th percentiles approach, performed separately for the exclusive cutaneous, exclusive mucosal, and mucocutaneous groups. Results In the 109 included patients, the 25th and 75th percentiles cut-offs were 6.2 and 27 for PDAI score, and 4 and 29.5 for ABSIS score. The model-based analysis resulted in two groups (cut-point:15) for PDAI score, and three groups (cut-points:6.4 and 31.5) for ABSIS score. The groups were significantly different for the PDAI, ABSIS, PGA, and ABQoL values. Based on anti-Dsg1 autoantibody values, the model-based analysis cut-point was 128 and the 25th and 75th percentiles cut-offs were 98 and 182. Anti-Dsg3 autoantibody values did not differentiate between pemphigus severity classes. Conclusions Estimated cut-off values based on the anti-Dsg1 level, PDAI, and ABSIS scoring systems could be used to classify patients into different severity grades for better management and prognosis.

Immunosuppressive treatment for autoimmune blistering diseases does not impair production of neutralizing antibodies after “booster” dose of COVID‐19 vaccine

“…Nine patients had been diagnosed with bullous pemphigoid and eight with pemphigus vulgaris (all of them confirmed by direct immunofluorescence). All patients had been receiving immunosuppressive therapy for at least 6 months continuously (mean duration of immunosuppressive therapy 9.8 months), with good control of the disease (ABSIS < 6.4) 10 . Immunosuppressive therapy was defined as >0.5 mg/kg of prednisone (or equivalent dosage of other steroids) and/or >50 mg/day of azathioprine, >7.5 mg/week of methotrexate, >1000 mg/day of mycophenolate.…”

“…All patients and all controls received a third dose of COVID‐19 vaccine Comirnaty – BioNTech/Pfizer 5–6 months before the evaluation, while on immunosuppressive therapy, with good control of the disease (ABSIS < 6.4) 10 . No patient and no control had been diagnosed with SARS‐COV‐2 infection previously.…”

“…All patients had been receiving immunosuppressive therapy for at least 6 months continuously (mean duration of immunosuppressive therapy 9.8 months), with good control of the disease (ABSIS < 6.4). 10 Immunosuppressive therapy was defined as >0.5 mg/kg of prednisone (or equivalent dosage of other steroids) and/or >50 mg/day of azathioprine, >7.5 mg/week of methotrexate, All patients and all controls received a third dose of COVID-19 vaccine Comirnaty -BioNTech/Pfizer 5-6 months before the evaluation, while on immunosuppressive therapy, with good control of the disease (ABSIS < 6.4). 10 No patient and no control had been diagnosed with SARS-COV-2 infection previously.…”

“…10 Immunosuppressive therapy was defined as >0.5 mg/kg of prednisone (or equivalent dosage of other steroids) and/or >50 mg/day of azathioprine, >7.5 mg/week of methotrexate, All patients and all controls received a third dose of COVID-19 vaccine Comirnaty -BioNTech/Pfizer 5-6 months before the evaluation, while on immunosuppressive therapy, with good control of the disease (ABSIS < 6.4). 10 No patient and no control had been diagnosed with SARS-COV-2 infection previously. All patients, as well as all controls, were found to have NAbs titers well above the positivity threshold provided by the manufacturer's kit.…”