Patients with pemphigus are at an increased risk of developing rheumatoid arthritis: a large-scale cohort study

Khalaf Kridin,Arnon D Cohen,Virginia A Jones,Kyle T Amber,Christoph M Hammers,Payal M Patel,Giovanni Damiani,Shira Zelber-Sagi

doi:10.1007/s12026-020-09160-6

Abstract

Data regarding the association between pemphigus and rheumatoid arthritis (RA) is inconclusive and yet to be firmly established. In the current study, we aimed to evaluate the risk of developing RA during the course of pemphigus. A large-scale population-based longitudinal cohort study was conducted to evaluate the hazard ratio (HR) of RA among 1985 patients with pemphigus relative to 9874 age-, sex-, and ethnicity-matched control subjects. A multivariate Cox regression model was utilized. The incidence of RA was 1.07 (95% CI, 0.62–1.72) and 0.36 (95% CI, 0.24–0.52) per 1000 person-years among patients with pemphigus and controls, respectively. The lifetime prevalence of RA was 2.3% (95% CI, 1.7–3.1%) among cases and 1.8% (95% CI, 1.5–2.0%) among controls. Patients with pemphigus were more than twice as likely to develop RA as compared to control subjects (adjusted HR, 2.54; 95% confidence interval [CI], 1.31–4.92). The increased risk was robust to a sensitivity analysis that included only cases managed by pemphigus-related systemic medications (adjusted HR, 2.56; 95% CI, 1.30–5.05). In conclusion, pemphigus is associated with an increased risk of RA. Physicians treating patients with pemphigus should be aware of this possible association. Further research is required to better understand the mechanism underlying this association.

Highlights

Pemphigus encompasses a group of rare, potentially lethal, mucocutaneous autoimmune bullous dermatoses [1]
The current retrospective cohort study demonstrates that pemphigus patients are at more than twofold increased risk of developing rheumatoid arthritis (RA) as compared to control subjects (HR, 2.54)
The results revealed that the risk of RA was elevated in females (HR, 3.34), a finding that accords with the female preponderance observed in several autoimmune diseases [17, 18]

Summary

Introduction

Pemphigus encompasses a group of rare, potentially lethal, mucocutaneous autoimmune bullous dermatoses [1]. These intraepidermal bullous diseases manifest with vesicles and erosions on the epithelium of mucous membranes and skin, often causing a substantial impairment of quality of life and. 20161 Milan, Italy 5 Clalit Health Services, Tel-Aviv, Israel elevated morbidity and mortality [2, 3]. It is well studied that autoimmune disease, such as pemphigus, is likely to coexist within individuals and their relatives [6,7,8]. Several studies have substantiated the concept of autoimmune diathesis in pemphigus with an array of autoimmune diseases [1]

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