AB0579 COMPARISON OF CLINICAL CHARACTERISTICS OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS BETWEEN RHEUMATOLOGY AND RESPIRATORY MEDICINE: A SINGLE CENTER, RETROSPECTIVE STUDY
Authors: Le-Feng Chen, LI Qianhua, Dong-Hui Zheng, Lie Dai
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare but potentially life-threatening systemic necrotizing vasculitis. Multiple organs may be involved and patients initial consulting different departments may have different clinical manifestations. <h3>Objectives:</h3> To explore the clinical characteristic of EGPA diagnosed in different departments in our hospital. <h3>Methods:</h3> A retrospective study of EGPA patients in Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China. All patients fulfill the ACR 1990 classification criteria or the EULAR/ACR 2017 draft classification criteria. Demographic and clinical data of EGPA patients were collected and compared between different departments. <h3>Results:</h3> 1) There were 45 EGPA patients diagnosed between December 2003 and January 2019. There were 29(64.4%) male, with median onset age 46(36∼56) years, median time required for diagnosis 24(3∼96) months. There were 8(17.8%) patients had history of drug allergy. There were 31(68.9%) patients fulfill the ACR 1990 classification criteria and 40(88.9%) fulfill the EULAR/ACR 2017 draft classification criteria. 2) The main clinical manifestations included asthma-like symptoms (82.2%), limb numbness (37.8%), rash (22.2%), fever (20.0%), gastrointestinal symptom (11.1%) and arthralgias (8.9%). There were 91.1% (41/45) patients had serum eosinophil &gt;10%, 77.8% (35/45) serum eosinophil &gt;1.0×10<sup>9</sup>/L, 77.5% (31/40) elevated serum IgE, 7.8% (3/44) positive MPO-ANCA or p-ANCA and 18.4% (7/38) positive ANA. 3) There were 21(46.7%) patients diagnosed at rheumatology, 22(48.9%) at respiratory medicine, 1(2.2%) at dermatology and 1(2.2%) at pediatrics. Compared with patients diagnosed at respiratory medicine, patients diagnosed at rheumatology had a higher frequency of fever (33.3% vs. 0), rash (33.3% vs. 4.5%), arthralgias (19.0% vs. 0) and limb numbness (57.1% vs. 22.7%), but lower frequency of asthma-like symptoms (66.7% vs. 100%; all <i>P</i>&lt;0.05). There were more patients diagnosed at rheumatology received treatment of glucocorticoid (100% vs. 72.7%), immunosuppressive agents (85.7% vs. 4.5%) and intravenous immunoglobin (28.6% vs. 0) than those diagnosed at respiratory medicine (all <i>P</i>&lt;0.01). Patients diagnosed at rheumatology also received higher initial dose of glucocorticoid than those diagnosed at respiratory medicine [50(50∼100) mg/d vs. 28(10∼40) mg/d of prednisone dosage, <i>P</i>&lt;0.001]. 4) Among the 21 patients diagnosed at rheumatology, 14(66.7%) had asthma-like symptoms, who showed significant longer time required for diagnosis than those without asthma-like symptoms [36(24∼120) months vs. 2(2∼13) months, <i>P</i>=0.020], but showed no significant difference of onset age between these two groups (<i>P</i>&gt;0.05). 5) The median revisited Five-Factor Score (FFS) was 0(0∼1). There were 17(37.8%) patients had FFS≥1, who had older diagnostic age than those with FFS=0 [65(46∼68) years vs. 48(42∼53) years, <i>P</i>&lt;0.05], but with no significant difference of onset age or time required for diagnosis. Patients with FFS≥1 had higher level of serum IgE [1280(515∼4895) IU/mL vs. 243(72∼587) IU/mL] than those with FFS=0 (<i>P</i>&lt;0.05). <h3>Conclusion:</h3> Clinical manifestations of EGPA were different between rheumatology and respiratory medicine. Rheumatologist may be more aggressive treating EGPA patients with glucocorticoid and immunosuppressive agents than respiratory physician. Multidisciplinary team is needed for the diagnosis and management of EGPA. <h3>Acknowledgement:</h3> This work was supported by Guangdong Medical Scientific Research Foundation (grant no. A2017093) to Le-Feng Chen. <h3>Disclosure of Interests:</h3> None declared