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Autoimmune mucocutaneous blistering diseases in the south of Italy: a 25‐year retrospective study on 169 patients
Author(s) -
Calabria Elena,
Fortuna Giulio,
Aria Massimo,
Mignogna Michele D.
Publication year - 2020
Publication title -
journal of oral pathology and medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.887
H-Index - 83
eISSN - 1600-0714
pISSN - 0904-2512
DOI - 10.1111/jop.13059
Subject(s) - medicine , mucocutaneous zone , retrospective cohort study , single center , pemphigus vulgaris , pemphigoid , pemphigus , therapeutic effect , disease , gastroenterology , bullous pemphigoid , dermatology , immunology , antibody
Background Autoimmune mucocutaneous blistering diseases (AMBDs) represent a heterogeneous group of organ‐specific and potentially life‐threatening diseases. We sought to determine the relationship between clinical remission and therapeutic regimens with clinical type and phenotype of AMBDs, as well as clinical outcomes achieved based on different therapeutic regimens. Methods A retrospective single‐center study on 169 AMBDs patients, including pemphigus vulgaris (PV), mucous membrane pemphigoid (MMP), paraneoplastic autoimmune multiorgan syndrome (PAMS), and lichen planus pemphigoides (LPP), was performed from 1994 to 2019 in an oral medicine tertiary center, where we collected sociodemographic data, clinical type and phenotype, prescribed therapies, and related outcomes. Results The mean age of AMBDs patients was 55.0 ± 16.4 years. They were followed for a mean of 8.4 ± 5.8 years. The majority of these patients (62.1%) were successfully managed with conventional immunosuppressive therapy (CIST) alone. However, 37.9% of patients required additional biological treatments, either because they were non‐responders or developed severe side effects from CIST, or because of the rapid and severe progression of the disease. Overall, complete clinical remission was achieved in 92.3% of patients. A statistically significant difference was noted between the frequency distribution of AMBDs patients among different therapeutic regimens ( P = .002), of different clinical phenotype and type of AMBDs patients and clinical remission ( P = .012 and P = .005, respectively). No difference was reported regarding clinical outcomes and different therapeutic regimens. Conclusions AMBDs' management may be challenging, nonetheless CIST and biologic regimens introduced, when needed as reliable alternatives to CIST, result in a very high percentage of CCR.