World Workshop on Oral Medicine VI: a systematic review of the treatment of mucocutaneous pemphigus vulgaris.
OBJECTIVE
To determine the efficacy and safety of interventions for pemphigus vulgaris (PV).


METHODS
We conducted a systematic review from 2003 to 2013 according to the Cochrane Collaboration methodology. Randomized controlled trials (RCTs) or controlled clinical trials (CCTs) and observational studies were conducted along with diagnosis confirmed by clinical, histopathologic, and immunofluorescence criteria. Primary outcomes were disease remission and mortality; several relevant secondary outcomes were also included.


RESULTS
Fourteen RCTs or CCTs and 110 observational studies were included in the final analyses. RCTs or CCTs demonstrated considerable heterogeneity in outcome measures, and all had a high risk of bias for at least 1 of 8 domains. Of the studies, 96.8% (120) described the use of oral corticosteroids. Azathioprine and mycophenolate-mofetil were the most commonly cited treatments. An increasing number of studies described biologic therapies (rituximab, intravenous immunoglobulin [IVIg]). Evidence supporting recent comprehensive treatment guidelines was reviewed.


CONCLUSIONS
We found persisting wide variations in treatment practice and inadequate quality of research supporting optimal PV treatment.
Consultant and Honorary Clinical Teaching Fellow in Oral Medicine and Facial Pain, Eastman Dental Hospital, London, UK.
R
R
McMillan
SPR Oral Medicine, University Dental Hospital of Manchester, Manchester, UK.
J
J
Taylor
SPR Oral Medicine, Eastman Dental Hospital, London, UK.
M
M
Shephard
Center for Blistering Diseases, Boston, Massachusetts, USA.
R
R
Ahmed
Professor of Oral Medicine, School of Dental Sciences, University of Newcastle upon Tyne, Framlington Place, Newcastle upon Tyne, UK.
M
M
Carrozzo
Reader/Honorary Consultant in Dermatology in relation to Oral Disease, Department of Oral Medicine and St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
J
J
Setterfield
Professor, Dermatology, School of Medicine, University of California, Irvine, California, USA.
S
S
Grando
Head of the Oral Medicine Complex Unit, Department of Neurosciences, Reproductive and Odontostomatological Sciences: Head and Neck Clinical Section, "Federico II" University, Naples, Italy.
M
M
Mignogna
Oral Medicine Resident, Harvard School of Dental Medicine, Boston, Massachusetts, USA.
M
M
Kuten-Shorrer
Orofacial Pain Resident, Orofacial Pain Center, University of Kentucky College of Dentistry, Lexington, Kentucky, USA.
T
T
Musbah
Oral Medicine Section, Department of Surgical Sciences, Lingotto Dental School, University of Turin, Turin, Italy.
A
A
Elia
Research Librarian, Adjunct Assistant Professor, Department of Epidemiology and Health Promotion, New York University College of Dentistry, New York, New York, USA.
R
R
McGowan
Clinical Professor, Department of Oral and Maxillofacial Pathology, Radiology and Medicine, New York University College of Dentistry, New York, New York, USA.
A R
AR
Kerr
Professor Emeritus, Department of Oral Medicine, School of Dental Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
M S
MS
Greenberg
Consultant in Oral Medicine/Honorary Senior Clinical Lecturer, Divisional Clinical Director, Eastman Dental Hospital, London, UK.
T
T
Hodgson
Associate Professor, Department of Oral and Maxillofacial Pathology, Radiology and Medicine, New York University College of Dentistry, New York, New York, USA. Electronic address: ds62@nyu.edu.
D
D
Sirois
D006801:Humans; D009059:Mouth Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D010392:Pemphigus / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D012074:Remission Induction