The prevalence of oral candidiasis is higher in psoriatic patients and is associated with disease severity. It is not exactly clear whether oral candidiasis can aggravate psoriasis or if psoriasis can predispose patients to oral candidiasis.
Background
Autoimmune bullous diseases are a group of rare, chronic, blistering diseases, with pemphigus vulgaris (PV) being the most common type in Iran. Skin and mucosal involvement and therapy may have a dismal impact on the quality of life of affected patients.
Objective
We aimed to assess the validity and reliability of the Farsi (Persian) version of the Autoimmune Bullous Quality of Life (ABQoL) index in Iranian patients with PV.
Methods
Forward and backward translation procedures were used to prepare the Persian version of the ABQoL questionnaire. A total of 180 patients were asked to complete the questionnaires, and 24 cases repeated the test after 2 weeks. For validity and reliability, an exploratory factor analysis was performed along with a parallel analysis to determine the number of factors. The multi-trait, multi-method matrix assessed convergent and discriminant validity. To evaluate internal reliability and reliability over time, Cronbach's alpha and intraclass correlation coefficients were reported.
Results
Two factors explained a total variance of 54.9% in the data. The first and second factors comprised questions 1–3, 5, 7, 9, and 12–17 (symptoms-social) and questions 4, 6, 8, 10, and 11 (mucosal-psychological), respectively. The internal reliability of the Persian version of the ABQoL index was also acceptable, with a Cronbach's alpha of .855 for total items, .918 for the first factor, and .6 for the second factor. Our results suggest an acceptable convergent and discriminant validity of the Persian version of the ABQoL questionnaire.
Conclusion
The Persian version of ABQoL index is a valid and reliable tool to monitor quality of life in patients with PV. Further studies are needed to assess the sensitivity of this instrument to changes in different disease activity and correlation with more general tools for the measurement of quality of life.
BackgroundPemphigus vulgaris (PV) is a blistering, life-threatening autoimmune disease. Ethylenediaminetetraacetic acid (EDTA)-treated desmoglein (Dsg) enzyme-linked immunosorbent assay (ELISA) has recently been suggested to detect nonpathogenic antibodies. Rituximab (RTX) is now considered a first-line treatment for PV.ObjectiveThe primary and secondary aims were to evaluate anti-Dsg and EDTA-treated anti-Dsg ELISA and clinical response before and 3 months after RTX in treatment-naïve and previously treated patients, respectively. In addition, we compared the short-term efficacy of RTX between these groups.MethodsSeventy-five patients with PV who received RTX (500 mg weekly for 4 weeks or 1000 mg 2 weeks apart) and prednisolone were followed for 3 months. Thirty-seven treatment-naïve newly diagnosed (group A) and 38 relapsed patients (group B) were included. Disease activity was scored with the Pemphigus Disease Area Index (PDAI). Clinical response was also assessed. Serum samples were collected at two points and examined for anti-Dsg1/3 and EDTA-treated anti-Dsg1/3. Conformational anti-Dsg values were calculated by subtracting EDTA-treated from conventional anti-Dsg values.ResultsThe correlation of conventional and conformational anti-Dsg values was perfect (correlation coefficient > 0.98; p < .001) at every time point for both anti-Dsgs. There was no difference with regard to PDAI and anti-Dsg values between the two groups at baseline. The frequency of responders was significantly higher in group A (100%) than in group B (89%; p = .006). Three patients relapsed, and five patients had persistent disease activity in group B. After 3 months, conventional and conformational anti-Dsg values were significantly higher in group B compared with group A (anti-Dsg3: p = .017 and .021, respectively; anti-Dsg1: p = .014 and .016, respectively). Total and scalp PDAI were significantly lower in group A than in group B (p = .042 and .016, respectively).ConclusionEDTA-treated anti-Dsg ELISA had no added value. Using RTX as first-line treatment in patients with PV appears to be associated with better clinical response and immunologic profile than delayed treatment in the short term.
Summary
Background
A role for nondesmoglein antigens in the pathogenesis of pemphigus vulgaris (PV) has been suggested in several studies. Acetylcholine receptors (AchR), are one of the most important groups of these antigens. However, the exact role of both antimuscarinic (m) and nicotinic (n) AchR antibodies (Abs) is still controversial.
Aim
To evaluate anti‐desmoglein (Dsg)1, Dsg 3 and anti‐γ/ε nAchR Abs values in patients with PV before and 3 months after rituximab (RTX) treatment, and to assess their correlation with disease severity.
Methods
In total, 75 patients with PV (26 men, 49 women) who were planned to receive RTX were enrolled. Disease activity was assessed by using the Pemphigus Disease Area Index (PDAI). Using ELISA, anti‐Dsg1,3 and anti‐γ/ε nAchR Abs were determined at baseline and 3 months after RTX treatment.
Results
At baseline, 53.33% patients had positive values for anti‐Dsg1, 89.33% for anti‐Dsg3 and 13.33% for anti‐γ/ε nAchR Abs. All patients with positive anti‐γ/ε nAchR Abs had the mucocutaneous phenotype. PDAI, anti‐Dsg1,3 and anti‐γ/ε nAchR values were dramatically decreased 3 months after RTX infusion (P < 0.001). There was a significant positive correlation between disease activity and anti‐γ/ε nAchR values at baseline (P = 0.04), whereas no significant correlation was observed between anti‐Dsg1,3 and anti‐γ/ε nAchR values at baseline and 3 months after RTX infusion.
Conclusion
The reduction in anti‐γ/ε nAchR Abs with clinical improvement in this study may suggest a synergic role for anti‐γ/ε nAchR Abs with anti‐Dsg1,3 Abs, or it could be an epiphenomenon.
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.
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