Eosinophilic granulomatosis with polyangiitis: 30 cases report

Abstract

Objective: To improve the understanding of Eosinophilic granulomatosis with polyangiitis (EGPA). Methods: A total of 30 EGPA patients from the China-Japan Friendship Hospital between August 2005 and April 2017 were included, and a systematic retrospective study about clinical manifestations, laboratory findings, imaging features, pathology and so on was conducted. Results: There were 9 males and 21 females. Mean age was (44.5±13.9) years at diagnosis. Among these patients, 26 (86.7%) had asthma, duration was 45.0 (20.0, 78.7) months, and 15 (50.0%) had asthma as their first symptom. Rhinitis/nasosinusitis occurred in 27 (90.0%), skin lesions in 13 (43.3%), neuropathy in 10 (33.3%), heart disease in 8 (26.7%), renal involvement in 3 (10.0%) and gastro-intestinal manifestations in 3 (10.0%) of the patients. Peripheral blood eosinophil>10% accounted for 23/30 (76.7%). There were 3 patients (10.0%) who had positive anti-neutrophil antibody, and the pattern by immunofluorescence was mainly perinuclear. Migratory infiltrates often observed on the chest radiograph, and the main presentation was ground-glass opacity, amounting for 21/30 (70.0%). The pathological manifestations were extravascular eosinophilic tissue infiltration, necrotizing vasculitis, and extravascular granulomas, and the latter two of which were fewer. Conclusion: The manifestations of EGPA are individual, complex, with a lack of pathognomonic symptoms, and female predominated.