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<0.05]. There were 21.2% EGPA patients showing poor prognostic factors according to five-factor score (FFS) and 34.6% patients according to the revised FFS.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.Figure 1.Department at first visit (A) and clinical manifestations during the whole disease process of 52 Chinese EGPA patients(B).Disclosure of Interests:None declared
Addison’s disease presenting with perimyocarditis