Abstract
The association between antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and cancer remains poorly understood. In this study, we searched the Korea National Health Insurance Claims Database to obtain data for 2097 AAV patients, and evaluated the risk of cancers in AAV. The standardized incidence ratios (SIRs) of overall and site-specific cancers were estimated in patients with AAV compared to the general population. The overall risk of cancer was significantly higher in patients with AAV (SIR 1.90); this remained true in both males (SIR 1.74) and females (SIR 2.06). For site-specific cancers, the risks of lung (SIR 2.23) and hematological (SIR 11.39) cancers were higher in AAV patients. For males, the risks of gallbladder and hematological cancers were increased, while the risks of bladder and hematological cancers were increased in females. Among AAV subtypes, patients with granulomatosis with polyangiitis had the highest risk of cancers, and cyclophosphamide, azathioprine/mizoribine, and methotrexate ever-users had increased risk of overall cancer. The risks of overall and hematological cancers were elevated in AAV patients younger than 60 years old. Patients with AAV have increased risks of overall, lung, and hematological cancers. Distinct patterns of cancer incidence are present according to age, sex, AAV subtypes, and immunosuppressant usage.
Highlights
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare autoimmune disease (AID) with the hallmarks of chronic inflammation within the blood vessels and the production of ANCAs directed against myeloperoxidase (MPO) and proteinase 3 (PR3) [1]
In terms of the subgroups of AAV, 947 (45.2%) patients were diagnosed with microscopic polyangiitis (MPA), 568 (27.1%) patients were diagnosed with granulomatosis with polyangiitis (GPA), and 582 (27.7%) patients were diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) (Table 1)
We have demonstrated that compared to the general population, AAV patients have an increased overall risk of cancer and increased risks of site-specific lung and hematological cancers
Summary
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare autoimmune disease (AID) with the hallmarks of chronic inflammation within the blood vessels and the production of ANCAs directed against myeloperoxidase (MPO) and proteinase 3 (PR3) [1]. Epidemiologic studies have indicated that AAV mainly affects patients over 50 years old, with an estimated incidence of 10–20/million and a prevalence of over 100/million [3,4,5]. Various comorbidities, such as cancers, infections, cardiovascular disease, and osteoporosis, are prevalent in patients with AIDs [6,7,8]. The long-term administration of immunosuppressive agents that are used for the treatment of AIDs is believed to support the development of cancers [12,13] For this reason, continuous efforts have been made to explore the link between AIDs and cancers
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