Abstract

BackgroundVarious immune-mediated diseases are associated with increased malignancy risks. However, the relationship between Behçet’s disease (BD) and cancer remains unclear. We conducted a nationwide, population-based study to determine the risk of cancer in patients with BD.MethodsUsing National Health Insurance claims records, we collected data from 2402 patients diagnosed with BD between 2013 and 2014. Standardized incidence ratios (SIRs) of overall and site-specific cancers in patients with BD in comparison with the general population were calculated.ResultsThe risks of overall cancer (SIR, 3.54; 95% confidence interval, 2.35–5.11 in men and 2.17; 1.58–2.92 in women) and solid cancer (3.10; 1.94–4.69 in men and 2.13; 1.52–2.90 in women) were greater in patients with BD than in the general population. There were significantly increased risks for these solid cancers: colorectal (4.26; 1.38–9.94), liver (4.00; 1.09–10.25), bone/articular cartilage (55.66; 1.41–310.14), prostate (7.05; 1.45–20.60), and brain/central nervous system (28.32; 3.43–102.31) in men; and the lips/oral cavity/pharynx (13.97, 1.69–50.47), liver (12.78; 5.14–26.33), lungs (4.35; 1.18–11.13), other female genital organs (53.57; 1.36–298.49), and eyes (128.26; 3.24–714.59) in women. Patients with BD had a greater risk of myelodysplastic syndrome (MDS) than the general population did (65.72; 7.96–237.41 in men and 53.86; 11.11–157.40 in women), but not of hematological cancer.ConclusionsCompared to the general population, Korean patients with BD had greater risks of overall cancer, some solid cancers, and MDS, but not of hematological cancer.

Highlights

  • Behcet’s disease (BD) is a chronic, multisystemic, immune-mediated disorder that is characterized by recurrent oral and/or genital ulcers, arthritis, and skin manifestations, as well as ocular, vascular, neurological, or intestinal involvement [1,2]

  • Patients with BD had a greater risk of myelodysplastic syndrome (MDS) than the general population did (65.72; 7.96–237.41 in men and 53.86; 11.11–157.40 in women), but not of hematological cancer

  • Several epidemiological studies have evaluated the relationships between a variety of systemic autoimmune rheumatic diseases and cancer, and the studies have demonstrated that rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren’s syndrome, systemic sclerosis, and dermatomyositis, are associated with an increased risk of malignancy, especially lymphoma [5,6,7,8,9]

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Summary

Introduction

Behcet’s disease (BD) is a chronic, multisystemic, immune-mediated disorder that is characterized by recurrent oral and/or genital ulcers, arthritis, and skin manifestations, as well as ocular, vascular, neurological, or intestinal involvement [1,2]. Autoimmunity and chronic inflammation are associated with malignancy development [3,4,5]. Several epidemiological studies have evaluated the relationships between a variety of systemic autoimmune rheumatic diseases and cancer, and the studies have demonstrated that rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren’s syndrome, systemic sclerosis, and dermatomyositis, are associated with an increased risk of malignancy, especially lymphoma [5,6,7,8,9]. Given that organs commonly involved in BD include the oral cavity, genitalia, skin, and eye and that BD recurrently induces chronic inflammation of these organs, BD may confer a high risk of cancer in these organs. Various immune-mediated diseases are associated with increased malignancy risks. Population-based study to determine the risk of cancer in patients with BD

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