Clinical analysis of 43 patients with eosinophilic granulomatosis with polyangiitis

Abstract

To describe the clinical features of eosinophilic granulomatosis with polyangiitis (EGPA). Patients who fulfilled the criteria for EGPA managed at the Beijing Chaoyang Hospital of Capital Medical University between May 2005 and Feb 2014 were retrospectively investigated. Patients' characteristics were compared according to antineutrophil cytoplasmic antibody (ANCA) status.Patients were followed up in outpatient service or by telephone to evaluate the treatment result. There were 43 patients included, 24 males and 19 females. Mean age at onset was (53.0±15.0) years old. The most common onset symptom of EGPA was asthma in 30 (69.8%) patients. Asthma was also the most common symptom in 42 (97.7%) patients, followed by sinusitis and rhinitis in 40(93.0%) patients and peripheral neuropathy in 26 (60.5%) patients. ANCA positive was found in 13 (30.2%) patients, mainly anti-myeloperoxidase antibodies. High resolution computed tomography scanning (HRCT) of the chest was performed in all the patients, and the main anomalies included ground-glass opacities in 34 (79.1%) patients and bronchial wall thickening and/or bronchial dilatation in 22 (51.2%) patients. Pulmonary function tests available at diagnosis in 33 patients mainly demonstrated an obstructive ventilatory defect. There were totally 33 patients who underwent biopsy, 21 (63.6%) patients had histologic findings supporting a diagnosis of EGPA, including eosinophil infiltration in 21 patients, vasculitis in 5 patients, but no granuloma. ANCA-positive patients were more likely to have fever and renal disease, more honeycombing pattern in the HRCT image and more significantly decreased pulmonary diffusion function, while ANCA-negative patients were more likely to have significant obstructive ventilatory defect. 41 patients received oral corticosteroids, among them, 19 patients additionally received immunosuppressants. 35 Patients were followed for a median of 30 (13, 46) months, 3 patients died (mortality 8.57%), among the other 32 patients, asthma was still the most common symptom in 16 (50%) patients at their last visit, and prednisone administration had to be continued for 23 (71.9%) patients. The clinical manifestations of EGPA may vary widely, and it involves different organs. Physicians should be alert to EGPA when seeing patients with refractory asthma and eosinophilia.