Abstract
BackgroundPemphigus 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.MethodsIn 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.ResultsIn 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.ConclusionsEstimated 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.
Highlights
Pemphigus is a potentially fatal disease if left untreated
Estimated cut-off values based on the anti-Dsg1 level, Pemphigus Disease Area Index (PDAI), and autoimmune bullous skin disorder intensity score (ABSIS) scoring systems could be used to classify patients into different severity grades for better management and prognosis
physician global assessment (PGA) score was correlated with all the other scores and values significantly, except anti-Dsg3; but negatively correlated with autoimmune bullous disease quality of life (ABQoL) (r = − 0.486, Pvalue < 0.001)
Summary
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 nonpharmaceutical treatments. The autoantibodies against desmosomal cadherins desmogleins, including anti-desmoglein 1 (anti-Dsg1) and anti-Dsg are widely debated as the main factors in disease development These autoantibodies result in acantholysis, blisters, and erosions of the skin and the mucous membrane of the mouth, nose, throat, eyes, or genitals [2]. Categorizing patients based on severity could be useful for better comparison of different studies and treatment responses and more precise designing of clinical trials and patient recruitment. It could be utilized in monitoring the disease progress, clinical assessment of relapses, and evaluating the effectiveness of treatment. All the aforementioned notions necessitate a universal, reliable and valid tool for categorizing patients based on their severity
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