ObjectivesThe aim of the prospective study was to compare clinical presentation at diagnosis and long-term outcomes of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) patients according to antineutrophil cytoplasmic antibody (ANCA) status.MethodsEGPA was classified according to the Revised CHCC Nomenclature and ACR1990 criteria. Activity of vasculitis was evaluated using BVAS. A minor relapse was defined as an increase in at least one new or worse minor item and no major BVAS items. A major relapse was an increase in at least one major BVAS item.ResultsWe followed 93 patients with EGPA for a mean±SD of 6.3±6.5 years. Their mean±SD age was 46.6±13.8 years, and 96.8% patients had a history of asthma. The most common EGPA manifestations at diagnosis included ENT manifestations (88.2%), fever (78.5%), peripheral neuropathy (73.1%), lung involvement (59.1%) and skin lesions (49.5%). Thirty seven of 93 patients (39.8%) were ANCA-positive. These patients had significantly more frequent myalgia and mononeuritis multiplex, than the ANCA-negative patients. The difference in occurrence of kidney disease between the two groups did not reach statistical significance. However, all three patients with rapidly progressive kidney failure were ANCA-positive. BVAS at diagnosis and VDI at the end of the follow-up were significantly higher for ANCA-positive patients. The follow up duration was 587.7 patient-years. The incidence of all vasculitis relapses was 14 per 100 patient-years, including major and minor vasculitis relapses (5.3 and 8.7 per 100 patient-years, respectively). The 3- and 5-year relapse-free survival rate was 65.4% (95% CI 54.6–76.2) and 43.1% (95% CI 30.4–55.7), respectively. The frequency of vasculitis relapses was 21.0 per 100 patient-years in the ANCA-positive group versus 19.6 per 100 patient-years in the ANCA-negative group (P=0.4).Table 1.Main clinical characteristics at diagnosis of the 71 patients with EGPA, according to ANCA statusManifestationsANCA+ (n=37)ANCA- (n=34)P
Fever30 (81.1)25 (73.5)0.57Myalgia22 (59.5)10 (29.4)
0.02
ENT33 (89.2)30 (88.2)1.00Lung24 (64.9)19 (55.9)0.47Cutaneous24 (64.9)15 (44.1)0.10Peripheral neuropathy30 (81.1)21 (61.8)0.11Mononeuritis multiplex17 (45.9)6 (17.6)
0.01
Cardiovascular13 (35.1)10 (29.4)0.62Gastrointestinal4 (10.8)4 (11.8)1.00Renal11 (29.7)5 (14.7)0.16BVAS at diagnosis, mean ± SD16.86±6.4412.62±5.52
<0.01
VDI at the end of the follow-up2.43±1.571.76±1.16
<0.01
ConclusionsThe characteristics of EGPA patients differ according to their ANCA status, although long-term outcomes were similar in both groups. EGPA is characterized by relapsing course of disease. The minor vasculitis relapses predominated in the structure of relapses.Disclosure of InterestNone declared
BackgroundMany patients with eosinophilic granulomatosis with polyangiitis (EGPA) lack a “vasculitic” phenotype and/or are ANCA negative. Recently, the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires developed a revised nomenclature for EGPA and proposed to differentiate it from hypereosinophilic asthma with systemic (nonvasculitic) manifestations (HASM).ObjectivesTo evaluate the occurrence of genuine polyangiitis and HASM in a cohort of patients with EGPA.MethodsWe retrospectively studied the medical records of patients with EGPA that fulfilled the classification criteria of the American College of Rheumatology. Patients with genuine vasculitis were identified by at least one of the following criteria:1 definite vasculitis feature as defined: biopsy-proven necrotizing vasculitis of any organ, biopsy-proven necrotizing glomerulonephritis or crescentic glomerulonephritis, alveolar haemorrhage, palpable purpura, myocardial infarction due to proven coronary arteritis;2 strong surrogate of vasculitis as defined: haematuria associated with red casts or ≥10% dysmorphic erythrocytes, or haematuria and 2+proteinuria on urinalysis related to the systemic disease; and any organ manifestation other than ENT or bronchopulmonary manifestation associated with leukocytoclastic capillaritis and/or eosinophilic infiltration of the arterial wall;3 mononeuritis multiplex;4 ANCA with at least one extra-thoracic non-ENTmanifestation of disease.ResultsWe followed 68 patients with EGPA for a mean ±SD of 6.3±6.5 years (587.7 patient-years). There were 19 males and 49 females. Their mean ±SD age was 49.5±13.8 years. In 18 patients (26.5%) with EGPA diagnosis was revised in favour of HASM using the new criteria (table 1). Notably, 19 of 50 patients (38%) with genuine polyangiitis were ANCA-negative but have histological evidence or clinical signs (rapidly progressive glomerulonephritis in 1, mononeuritis multiplex 7, palpable purpura in 5) of definite vasculitis. The majority of patients in both groups were females of similar age at disease onset. The occurrence of constitutional symptoms, except myalgia, nasal involvement, cardiovascular and pulmonary manifestations did not differ between patients with genuine polyangiitis and HASM. However, patients with EGPA usually required more intensive immunosuppressive treatment, including cyclophosphamide, while monotherapy with moderate to high dose corticosteroids was adequate for the majority of patients with HASM.Table 1 Clinical and demographic characteristics of patients with genuine EGPA and HASMConclusionsAt least a quarter of patients with EGPA classified according to the ACR criteria do not have genuine polyangiitis. The revised criteria of EGPA and HASM may have a significant value for choosing treatment options.Disclosure of InterestNone declared
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.