Laryngeal Mucous Membrane Pemphigoid: A Systematic Review and Pooled-Data Analysis

Objectives/Hypothesis: To perform a systematic pooled-data analysis of literature data involving laryngeal mucous membrane pemphigoid (MMP). Study Design: A systematic review and pooled-data analysis. Methods: We conducted a systematic literature search of MEDLINE, EMBASE, Cochrane Central Register of Clinical Trials, Cochrane Database of Systematic Reviews, clinicaltrials.gov, and the National Guideline Clearinghouse databases without language restriction for studies including combinations of relevant terms. All authors independently screened the abstracts of the search results, identified articles eligible for review, and critically appraised the full-text studies. Pooled-data analyses and Kaplan-Meier survival analyses were conducted using SPSS version 16.0 software (SPSS Inc., Chicago, IL). Results: Of the 2,524 citations reviewed, the included articles consisted of 63 case reports and 10 case series reporting on 141 patients with laryngeal MMP. No clinical trials or comparative trials were found. The overall calculated prevalence of laryngeal MMP was 12.2% (95% confidence interval [CI], 11.5-12.9%) of cases of MMP or one in 10 million persons in the general population. Mean age at laryngeal MMP onset was 59.7 years (95% CI, 57.9-61.1 years), and the supraglottis was the most commonly affected site (84.8%, 95% CI, 82.5-87.2%). Distribution among genders was equivalent (P = .655). The presence of antiepiligrin autoantibodies was associated with increased laryngeal involvement (Odds ratio 7.9, 95% CI, 3.9-16.0). The overall 5-year relative survival rate was 92.4% (standard error, 8.4) with a follow-up range of 1 to 221 months. Standard medical therapy alone occasionally improved the condition; however, relapses were frequent, and 10.5% eventually required tracheostomy. Laryngeal surgical interventions seemed to be effective in severe cases. Conclusions: Laryngeal MMP is a rare condition that can be life threatening without proper treatment and frequent follow-up.