Clinical Characteristics Of Eosinophilic Granulomatosis With Polyangiitis: A Single-Center Retrospective Analysis On 52 Cases Of Chinese Patients

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and heterogeneous systemic vasculitis. Different patients or the same patient in different stages show different manifestations, which may lead to misdiagnosis and delay treatment. Objectives: To analyze the clinical characteristics in Chinese patients with EGPA. Methods: EGPA patients who fulfilled the 1990 ACR classification criteria were included between December 2003 and April 2020. The demographic and clinical characteristics were collected and analyzed retrospectively. Results: There were 52 EGPA patients recruited, 34 (65.4%) patients were males and the median age at onset was 47(38~55) years. The median duration from disease onset to diagnosis was 30(4~96) months. For initial symptoms, respiratory manifestations (61.5%) were the most common, including 42.3% patients beginning with asthma, followed by 21.2% with nose/paranasal sinuses manifestations. Respiratory medicine (53.8%) were the most common department at first visit, followed by rheumatology medicine (11.5%, Figure 1A). There were 44.2% EGPA patients definitely diagnosed at the department of rheumatology or after consultation by rheumatologists. During the whole disease process, the most common clinical manifestations were asthma (88.5%), then nose/ paranasal sinuses (84.6%), pulmonary (76.9%) and nervous system (61.5%) manifestations, followed by constitutional symptom (44.2%), heart (36.5%) and skin (23.1%) involvement. Only 9.6% patients had gastrointestinal tract involvement and 3.8% had renal involvement (Figure 1B). There were 46 (88.5%) patients showing the ratio of peripheral blood eosinophils >10% at diagnosis. Among the rest 6 patients, 3 had higher eosinophil ratio before diagnosis, while the other 3 patients had been treated with glucocorticoid before diagnosis, of whom 2 patients showed pathological eosinophil infiltration in lung or paranasal sinuses mucosa, respectively. There were 8(15.4%) patients with positive ANCA. Compared with EGPA patients with negative ANCA, they had lower incidence of asthma (62.5% vs. 93.2%), but higher incidence of constitutional symptoms (87.5% vs. 36.4%), arthralgia (50.0% vs. 6.8%) and renal involvement (25.0% vs. 0.0%), higher peripheral eosinophil count [2.06(0.80~4.51) ×109/L vs. 1.81(0.93~3.32) ×109/L], ESR [20(7~77) mm/h vs. 18(9~42) mm/h] and CRP [18.5(3.2~65.9) mg/L vs. 3.3(3.2~13.0) mg/L], higher Birmingham vasculitis activity score [17(10~22) vs. 13(9~15)] and vasculitis damage index [3(1~3) vs. 2(1~2), all P Conclusion: EGPA patients may have no asthma especially those with positive ANCA. Multi-disciplinary collaboration especially based on rheumatologists and pulmonologists should be emphasized for early identification and prompt treatment. Disclosure of Interests: None declared