Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study
IntroductionPemphigus encompasses a group of muco-cutaneous autoimmune bullous diseases characterized by the loss of adhesion between keratinocytes. The disease is associated with increased morbidity and mortality.Materials and MethodsWe characterized clinical patterns, survival, comorbidities, and...
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Frontiers Media S.A.
2022-07-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2022.895490/full |
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| author | Lavinia Quintarelli Alessio Coi Roberto Maglie Alberto Corrà Elena Biancamaria Mariotti Cristina Aimo Valentina Ruffo di Calabria Alice Verdelli Beatrice Bianchi Elena Del Bianco Emiliano Antiga Marzia Caproni |
| author_facet | Lavinia Quintarelli Alessio Coi Roberto Maglie Alberto Corrà Elena Biancamaria Mariotti Cristina Aimo Valentina Ruffo di Calabria Alice Verdelli Beatrice Bianchi Elena Del Bianco Emiliano Antiga Marzia Caproni |
| author_sort | Lavinia Quintarelli |
| collection | DOAJ |
| description | IntroductionPemphigus encompasses a group of muco-cutaneous autoimmune bullous diseases characterized by the loss of adhesion between keratinocytes. The disease is associated with increased morbidity and mortality.Materials and MethodsWe characterized clinical patterns, survival, comorbidities, and drug prescriptions in patients with pemphigus referred to the Section of Dermatology of the University of Florence from January 2010 to December 2021.ResultsA total of 149 patients were identified (female/male sex ratio = 2.0). Median age at diagnosis was 57.7 ± 17.2 years; 108 patients were diagnosed with pemphigus vulgaris (PV) (72.5%) and 35 (23.5%) with pemphigus foliaceus (PF). Paraneoplastic pemphigus (PNP) and IgA-pemphigus accounted for three patients each. The overall survival rate was 86.9%. Accordingly, 14 (9%) patients died during the study period. The average age at death was 77.8 ± 9.3. Age at diagnosis was a risk factor for death in patients with pemphigus. Average concentration of Dsg3-IgG and Dsg1-IgG was 85.6 ± 68.8 and 75.9 ± 68.4, respectively. The most serious comorbid diseases included cerebro- and cardiovascular accidents and malignancies. Regarding the treatment regimen, we found a substantially stable use of systemic steroids in the 2010–2018 period; the prevalence of use of mycophenolic acid increased, whereas that of azathioprine decreased. The use of rituximab showed the highest increase in the 2013–2018 period. Proton-pump inhibitors and antibiotics were the most frequently prescribed non-immunomodulating drugs.ConclusionsIn this large series of the patients, patients with pemphigus showed a high incidence of serious comorbid diseases, highlighting the importance of a multidisciplinary approach for a proper management of the patients. Rituximab was the immunomodulating drug showing the highest increase in use over time, reflecting the growing evidence of its efficacy as a first-line treatment in pemphigus. |
| first_indexed | 2024-04-13T22:19:41Z |
| format | Article |
| id | doaj.art-e736e1f88b4b4fc781657d14ba6b6f94 |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-04-13T22:19:41Z |
| publishDate | 2022-07-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-e736e1f88b4b4fc781657d14ba6b6f942022-12-22T02:27:19ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-07-011310.3389/fimmu.2022.895490895490Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based StudyLavinia Quintarelli0Alessio Coi1Roberto Maglie2Alberto Corrà3Elena Biancamaria Mariotti4Cristina Aimo5Valentina Ruffo di Calabria6Alice Verdelli7Beatrice Bianchi8Elena Del Bianco9Emiliano Antiga10Marzia Caproni11Rare Skin Diseases Unit, Section of Dermatology, Department of Health Sciences, Unità Sanitaria Locale (USL) Toscana Centro, European Reference Network-Skin Member, University of Florence, Florence, ItalyUnit of Epidemiology of Rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, Pisa, ItalyDepartment of Health Sciences, Section of Dermatology, University of Florence, Florence, ItalyDepartment of Health Sciences, Section of Dermatology, University of Florence, Florence, ItalyDepartment of Health Sciences, Section of Dermatology, University of Florence, Florence, ItalyDepartment of Health Sciences, Section of Dermatology, University of Florence, Florence, ItalyDepartment of Health Sciences, Section of Dermatology, University of Florence, Florence, ItalyRare Skin Diseases Unit, Section of Dermatology, Department of Health Sciences, Unità Sanitaria Locale (USL) Toscana Centro, European Reference Network-Skin Member, University of Florence, Florence, ItalyDepartment of Health Sciences, Section of Dermatology, University of Florence, Florence, ItalyDepartment of Health Sciences, Section of Dermatology, University of Florence, Florence, ItalyDepartment of Health Sciences, Section of Dermatology, University of Florence, Florence, ItalyRare Skin Diseases Unit, Section of Dermatology, Department of Health Sciences, Unità Sanitaria Locale (USL) Toscana Centro, European Reference Network-Skin Member, University of Florence, Florence, ItalyIntroductionPemphigus encompasses a group of muco-cutaneous autoimmune bullous diseases characterized by the loss of adhesion between keratinocytes. The disease is associated with increased morbidity and mortality.Materials and MethodsWe characterized clinical patterns, survival, comorbidities, and drug prescriptions in patients with pemphigus referred to the Section of Dermatology of the University of Florence from January 2010 to December 2021.ResultsA total of 149 patients were identified (female/male sex ratio = 2.0). Median age at diagnosis was 57.7 ± 17.2 years; 108 patients were diagnosed with pemphigus vulgaris (PV) (72.5%) and 35 (23.5%) with pemphigus foliaceus (PF). Paraneoplastic pemphigus (PNP) and IgA-pemphigus accounted for three patients each. The overall survival rate was 86.9%. Accordingly, 14 (9%) patients died during the study period. The average age at death was 77.8 ± 9.3. Age at diagnosis was a risk factor for death in patients with pemphigus. Average concentration of Dsg3-IgG and Dsg1-IgG was 85.6 ± 68.8 and 75.9 ± 68.4, respectively. The most serious comorbid diseases included cerebro- and cardiovascular accidents and malignancies. Regarding the treatment regimen, we found a substantially stable use of systemic steroids in the 2010–2018 period; the prevalence of use of mycophenolic acid increased, whereas that of azathioprine decreased. The use of rituximab showed the highest increase in the 2013–2018 period. Proton-pump inhibitors and antibiotics were the most frequently prescribed non-immunomodulating drugs.ConclusionsIn this large series of the patients, patients with pemphigus showed a high incidence of serious comorbid diseases, highlighting the importance of a multidisciplinary approach for a proper management of the patients. Rituximab was the immunomodulating drug showing the highest increase in use over time, reflecting the growing evidence of its efficacy as a first-line treatment in pemphigus.https://www.frontiersin.org/articles/10.3389/fimmu.2022.895490/fullpemphigusepidemiologyrituximabcomorbiditiesdrugs |
| spellingShingle | Lavinia Quintarelli Alessio Coi Roberto Maglie Alberto Corrà Elena Biancamaria Mariotti Cristina Aimo Valentina Ruffo di Calabria Alice Verdelli Beatrice Bianchi Elena Del Bianco Emiliano Antiga Marzia Caproni Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study Frontiers in Immunology pemphigus epidemiology rituximab comorbidities drugs |
| title | Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study |
| title_full | Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study |
| title_fullStr | Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study |
| title_full_unstemmed | Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study |
| title_short | Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study |
| title_sort | clinical patterns survival comorbidities and treatment regimens in 149 patients with pemphigus in tuscany italy a 12 year hospital based study |
| topic | pemphigus epidemiology rituximab comorbidities drugs |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2022.895490/full |
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