INCIDENCE OF CANCER IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: A SINGLE CENTER STUDY
- Roberto Padoan (Italy)
Abstract
Background and Aims
Patients with ANCA-associated vasculitis (AAV) exhibit higher rates of malignancy than general population, but data on eosinophilic granulomatosis with polyangiitis (EGPA) are still lacking. The aim of the study was to investigate the incidence of malignancies in EGPA patients and to examine the effect of immunosuppressive therapy on malignancy risk in these patients.
Methods
The study population included 72 EGPA patients (61% female, 48% MPO-ANCA positive, 55 [42-62] years old at diagnosis), diagnosed between 1993 and 2018. Demographic, clinical and laboratory data, and immunosuppressive drugs were assessed. Incidence rates from the Italian Cancer Registry were used to compare malignancy incidence in the AAV cohort and to obtain age- and sex-standardized incidence ratios (SIR).
Results
During the 285 person-years observation period, we found 13 cancers in 8 of the 72 patients, during a median follow-up of 47.5 [16-108] months. The SIR (95% CI) malignancy risk was 2.37 (1.02-4.66) for cancers at all sites, and 2.90 (1.25-5.71) for all cancers excluding non-melanoma skin cancers. Median latency from EGPA onset and first cancer diagnosis was 2.5 (1-6.5) years, with 62.5% of patients developing cancer within 1 and 5 years. Comparing patients who developed malignancies with those who did not, no significant difference was noted regarding sex, ANCA status, age at diagnosis, clinical manifestations, BVAS, FFS, environmental exposure, smoking habit and cancer familiarity. Type of treatment and cumulative doses of cyclophosphamide were not associated with higher incidence of cancers.
Conclusions
EGPA patients have a persistent increased risk for overall malignancy compared to general population, irrespective of type of treatment.