52. Analysis of flares in a single centre cohort of Eosinophilic Granulomatosis with Polyangiitis patients
Creators: name: Garcés1, Roberto Ríos
name: Prieto-González1, Sergio
name: Hernández-Rodríguez1, José
name: Cid1, María C.
name: Espígol-Frigolé1, Georgina
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background:</strong> Eosinophilic granulomatosis with polyangiitis (EGPA) is a heterogeneous disease with a variable, relapsing course. Real-world data regarding specific characteristics of flares in these patients are scarce. We aim to describe and analyse the flares presented in a single-centre cohort of EGPA patients. <strong>Methods</strong>: Medical charts of EGPA patients diagnosed since 2008 were reviewed to describe demographics, clinical characteristics at diagnosis and at flare, number of flares, disease activity at flare, treatment of the flares, damage accrual, and laboratory results. The definition of flare was the same used in the MIRRA trial<sup>1</sup>. <strong>Results: </strong>A total of 38 EGPA patients were diagnosed since 2008 and followed regularly at our department. All the patients fulfilled both the ACR/EULAR newly released classification criteria and the MIRRA trial classification criteria for EGPA. Nineteen of them were male and 19 were female, with a mean age of 54.6 years. Baseline characteristics, along with an in-depth description of flares are summarized in table 1. A total of 190 flares were registered in 31 of 38 patients during a cumulative follow-up of 256.77 years, while in the remaining 7 patients without flares the cumulative follow-up accounted for a total of 28.55 years. Thus, the yearly rate of flares was 0.74. The main clinical symptoms at flare were asthmatic (77% of flares), followed by ear, nose and throat (ENT) symptoms (34% rhinitis, 24% sinusitis) being those that may be attributable to vasculitis (alveolar haemorrhage, peripheral nervous system involvement, nephritis, skin purpura, or arthritis) the least frequent (less than 5%), together with constitutional symptoms (around 5%). Lab tests could be requested in a minority of cases when the patients were symptomatic, relying the diagnosis of flare on the clinical judgement of the treating physician. When eosinophils (requested only in 50 flares), CRP (52 flares) and ESR (30 flares) were evaluated, in almost half of the tests (26 flares for eosinophils; 27 flares for CRP; 20 flares for ESR) the results were within normal ranges. ANCA were the least requested test (only in 27 flares), being positive by immunofluorescence also in half of the tests (14 flares). The mean dose of prednisone at the moment of flare was 8.95 mg/day. All the flares required an increase in the dose of prednisone, with a mean increase of 20.69 mg/day of prednisone, reaching mean doses up to 29.59 mg/day to treat the flare. <strong>Conclusions:</strong> The main characteristics of the flares of a well-defined cohort of EGPA patients were described. Asthma was the most common cause of flare (77% of flares), followed by ENT symptoms, being those attributable to vasculitis the least common. Lab test regarding eosinophils, CRP, ESR or ANCA were normal/negative in up to half of the tests. Flares occurred already at medium doses of prednisone and required high increases of prednisone doses for its treatment, which was the main treatment. <strong>Disclosures:</strong> Maria C Cid has received consulting fees from GSK, Abbvie and Janssen, a research grant from Kiniksa Pharmaceuticals and lecturing fees from Vifor and GSK. Georgina Espígol-Frigolé has received consulting fees from Janssen.