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Infections, age and medications linked to death in patients with ANCA-associated vasculitis
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Infections in patients with ANCA-associated vasculitis are a cause of mortality and are associated with leukopenia, increased age and high doses of certain medications, according to study data presented at the European League Against Rheumatism Annual European Congress of Rheumatology.
The retrospective study included 129 patients with ANCA-associated vasculitis (AAV). Of the patients studied, 46.5% were men, and mean age at the time of diagnosis was 54.6 years. Thirty-nine percent of the patients had granulomatosis with polyangiitis (GPA), 38% had microscopic polyangiitis (MPA) and 23% had eosinophilic GPA (EGPA). ANCAs were positive in 111 patients, with 30% of the patients presenting with C-ANCA and 56% with P-ANCA.
Common initial symptoms included toxic syndrome in 77.5% of patients; fever in 66%; cough and dyspnea with lung infiltrates or nodules in 57% and alveolar hemorrhage in 19%; peripheral or central neurologic involvement in 49%; ear, nose and throat involvement in 47%; and acute renal failure in 45%.
Oral glucocorticoids were the most commonly prescribed medications, received by 99% of patients, followed by intravenous glucocorticoids in 64%, cyclophosphamide in 43%, pulse cyclophosphamide in 49% and azathioprine in 25%.
Twenty-four patients underwent dialysis, and in 63% of patients, trimethoprim-sulfamethoxazole was used as a prophylactic. Relapse occurred in 89 patients during follow-up at a rate of 1.7 per patient, according to the researchers.
Bacterial infections occurred in 63 patients (49%) and included bronchitis in 41% of patients, pneumonia in 24%, urinary tract infections in 11%, sepsis in 9% and abdominal infections in 5% of patients.
Tuberculosis was present in two patients and opportunistic infections presented in some patients, including systemic mycosis in eight patients, cytomegalovirus in three patients, Pneumocystis jirovecii in three patients and Clostridium difficile in four patients, among others.
Bacterial infections occurred more frequently in patients with leukopenia, patients who were treated with glucocorticoids at high doses and in patients who received cyclophosphamide at cumulative doses above 15 g.
Early-stage death occurred in 34 patients (26%) in an average of 113.6 months due to refractory disease, whereas later-stage death was caused by infection in 42% of patients, malignancies in 9% and ischemic events in 6% of patients.
Bacterial infections, renal failure, urinary infection, sepsis and age older than 65 years at diagnosis were predictive of lower life expectancy, according to the researchers. – by Shirley Pulawski
Reference:
Vives EG, et al. Paper #AB0639. Presented at: European League Against Rheumatism Annual European Congress of Rheumatology; June 10-13, 2015; Rome.
Disclosure: The researchers report no relevant financial disclosures.