Prevalence of Nasopharyngeal Carcinoma in Patients with Dermatomyositis: A Systematic Review and Meta-Analysis

SIMPLE SUMMARY: This first systematic review and meta-analysis on the prevalence of nasopharyngeal carcinoma in patients suffering dermatomyositis was necessitated by the absence of a true and reliable prevalence estimate necessary to adequately inform medical preparedness and decisions. Following a careful review of literature and data analyses, a prevalence of 3.3% was found. It is hoped that a clear knowledge of the actual prevalence of nasopharyngeal carcinoma in dermatomyositis patients would not only help sensitize clinicians and patients about the frequency of these disease conditions but would also enhance the adoption of precautions essential to mitigate their co-occurrence in patients. ABSTRACT: For more than 50 years, nasopharyngeal carcinoma (NPC) has been associated with dermatomyositis (DM), a rare idiopathic inflammatory disorder that mainly affects the skin and muscles. Although the association between these rare diseases is well-documented, the actual prevalence of NPC in DM patients remains unknown. Here, a systematic review and meta-analysis of published data was conducted in accordance with the guidelines of Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA). Electronic databases including PubMed, Scopus, ScienceDirect, and Google Scholar were searched without year or language restrictions for studies reporting the occurrence of NPC in DM patients. The study protocol was lodged with PROSPERO (CRD42021225335). A total of 95 studies covering 303 cases of NPC among 16,010 DM patients was included. Summary estimates were calculated using the random-effects model. The pooled prevalence of NPC in DM was 3.3% (95% CI, 2.5–4.3). When stratified according to study location, higher prevalence estimates were obtained for Hong Kong (36.5%), Malaysia (27.7%), and Singapore (11.9%). There was a predominance of cases among male DM patients compared with females, and most patients were aged 40 and above. Many of the NPC cases were found to be diagnosed after the diagnosis of DM. It is therefore pertinent to screen for NPC in DM patients, especially among older DM patients in the Asian region.