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SHR Neuro Cancer Cardio Lipid Metab Microb

Papo, M; Sinico, RA; Teixeira, V; Venhoff, N; Urban, ML; Iudici, M; Mahrhold, J; Locatelli, F; Cassone, G; Schiavon, F; Seeliger, B; Neumann, T; Kroegel, C; Groh, M; Marvisi, C; Samson, M; Barba, T; Jayne, D; Troilo, A; Thiel, J; Hellmich, B; Monti, S; Montecucco, C; Salvarani, C; Kahn, JE; Bonnotte, B; Durel, CA; Puéchal, X; Mouthon, L; Guillevin, L; Emmi, G; Vaglio, A; Terrier, B, , French, Vasculitis, Study, Group, and, the, EGPA, European, Study, Group.
Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
Rheumatology (Oxford). 2021; 60(9): 4355-4360. Doi: 10.1093/rheumatology/keaa805
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Co-authors Med Uni Graz: Thiel Jens
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Abstract:: OBJECTIVES: Only a third of patients with eosinophilic granulomatosis with polyangiitis (EGPA) are ANCA-positive, mainly directed against MPO. ANCA directed against PR3 are rarely found in EGPA. We aimed to examine the significance of PR3-ANCA in EGPA. METHODS: We set up a retrospective European multicentre cohort including 845 patients. Baseline characteristics and outcomes were analysed and compared according to ANCA status. RESULTS: ANCA status was available for 734 patients: 508 (69.2%) ANCA-negative, 210 (28.6%) MPO-ANCA and 16 (2.2%) PR3-ANCA. At baseline, PR3-ANCA patients, compared with those with MPO-ANCA and ANCA-negative, less frequently had active asthma (69% vs 91% and 93%, P = 0.003, respectively) and peripheral neuropathy (31% vs 71% and 47%, P < 0.0001), more frequently had cutaneous manifestations (63% vs 38% and 34%, P = 0.03) and pulmonary nodules (25% vs 10% and 8%, P = 0.046), and lower median eosinophil count (1450 vs 5400 and 3224/mm3, P < 0.0001). Vasculitis relapse-free survival was shorter for PR3-ANCA (hazard ratio 6.05, P = 0.005) and MPO-ANCA patients (hazard ratio 1.88, P = 0.0002) compared with ANCA-negative patients. CONCLUSION: PR3-ANCA EGPA patients differ from those with MPO-ANCA and negative ANCA, and share clinical features with granulomatosis with polyangiitis. This suggests that PR3-ANCA EGPA could be a particular form of PR3-ANCA-associated vasculitis.
Find related publications in this database (using NLM MeSH Indexing): Adult - administration & dosage; Aged - administration & dosage; Antibodies, Antineutrophil Cytoplasmic - immunology; Churg-Strauss Syndrome - immunology; Female - administration & dosage; Granulomatosis with Polyangiitis - immunology; Humans - administration & dosage; Male - administration & dosage; Middle Aged - administration & dosage; Retrospective Studies - administration & dosage
Find related publications in this database (Keywords): ANCA; vasculitis; Churg-Strauss