Long-term evaluation of pemphigus vulgaris: A retrospective consideration of 98 patients treated in an oral medicine unit in north-west Italy.


Journal

Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology
ISSN: 1600-0714
Titre abrégé: J Oral Pathol Med
Pays: Denmark
ID NLM: 8911934

Informations de publication

Date de publication:
May 2019
Historique:
received: 05 03 2019
accepted: 06 03 2019
pubmed: 13 3 2019
medline: 8 1 2020
entrez: 13 3 2019
Statut: ppublish

Résumé

Despite the frequency of oral involvement, there are unexpectedly few studies of either on the oral manifestations of pemphigus or their long-term management, and diagnostic delay in Dentistry is frequent. We have examined outcome of patients presenting with predominantly oral pemphigus vulgaris (PV). Ninety-eight subjects were followed up for 85.12 months and treated with systemic steroids: 48 of them received adjunctive therapy with azathioprine, 16 with rituximab, 13 with mycophenolate mofetil, three with immunoglobulin and one with dapsone. Clinical remission was achieved in 80 patients (84.21%); 39 of them were off therapy and 41 on therapy. Fifteen patients were not in remission, having been under systemic therapy for 72.16 months. Sixty-nine patients developed detectable adverse effects. Two fatal outcomes were recorded. Each additional year of steroid therapy ensured 47% chance of developing 1 or 2 side effects, and 64% chance of developing more than 3 (ORs 1.47, CI 1.162-1.903; ORs 1.64, CI 1.107-2.130, respectively). In one of the largest available cohort with the longest follow-up ever reported, we observed that the management remains need-based and patient-specific, still relying on systemic corticosteroids.

Sections du résumé

BACKGROUND BACKGROUND
Despite the frequency of oral involvement, there are unexpectedly few studies of either on the oral manifestations of pemphigus or their long-term management, and diagnostic delay in Dentistry is frequent.
METHODS METHODS
We have examined outcome of patients presenting with predominantly oral pemphigus vulgaris (PV). Ninety-eight subjects were followed up for 85.12 months and treated with systemic steroids: 48 of them received adjunctive therapy with azathioprine, 16 with rituximab, 13 with mycophenolate mofetil, three with immunoglobulin and one with dapsone.
RESULTS RESULTS
Clinical remission was achieved in 80 patients (84.21%); 39 of them were off therapy and 41 on therapy. Fifteen patients were not in remission, having been under systemic therapy for 72.16 months. Sixty-nine patients developed detectable adverse effects. Two fatal outcomes were recorded. Each additional year of steroid therapy ensured 47% chance of developing 1 or 2 side effects, and 64% chance of developing more than 3 (ORs 1.47, CI 1.162-1.903; ORs 1.64, CI 1.107-2.130, respectively).
CONCLUSION CONCLUSIONS
In one of the largest available cohort with the longest follow-up ever reported, we observed that the management remains need-based and patient-specific, still relying on systemic corticosteroids.

Identifiants

pubmed: 30860627
doi: 10.1111/jop.12847
doi:

Substances chimiques

Adrenal Cortex Hormones 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

406-412

Informations de copyright

© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Auteurs

Paolo G Arduino (PG)

Department of Surgical Sciences, CIR-Dental School, University of Turin, Turin, Italy.

Roberto Broccoletti (R)

Department of Surgical Sciences, CIR-Dental School, University of Turin, Turin, Italy.

Mario Carbone (M)

Department of Surgical Sciences, CIR-Dental School, University of Turin, Turin, Italy.

Alessio Gambino (A)

Dimeas, Politecnico of Turin, Turin, Italy.

Veronica Sciannameo (V)

Unit of Epidemiology, Regional Health Service ASL TO3, Grugliasco, Italy.

Davide Conrotto (D)

Department of Surgical Sciences, CIR-Dental School, University of Turin, Turin, Italy.

Marco Cabras (M)

Department of Surgical Sciences, CIR-Dental School, University of Turin, Turin, Italy.

Savino Sciascia (S)

Center of Research of Immunopathology and Rare Diseases-Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.

Fulvio Ricceri (F)

Unit of Epidemiology, Regional Health Service ASL TO3, Grugliasco, Italy.

Simone Baldovino (S)

Center of Research of Immunopathology and Rare Diseases-Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.

Marco Carrozzo (M)

Department of Oral Medicine, School of Dental Sciences, University of Newcastle upon Tyne, Newcastle upon Tyne, UK.

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Classifications MeSH