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Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Source :
- Rheumatology, Vol. 60, No 9 (2021) pp. 4355-4360
- Publication Year :
- 2020
-
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 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.
- Subjects :
- Adult
Male
Vasculitis
medicine.medical_specialty
030204 cardiovascular system & hematology
Churg-Strauss Syndrome
urologic and male genital diseases
Gastroenterology
Antibodies, Antineutrophil Cytoplasmic
03 medical and health sciences
0302 clinical medicine
Rheumatology
immune system diseases
Internal medicine
Eosinophilic
Humans
Medicine
Pharmacology (medical)
cardiovascular diseases
ANCA
Churg-Strauss
vasculitis
skin and connective tissue diseases
Churg strauss
Aged
Retrospective Studies
Asthma
030203 arthritis & rheumatology
ddc:616
business.industry
Hazard ratio
Granulomatosis with Polyangiitis
Middle Aged
Eosinophil
medicine.disease
Dermatology
respiratory tract diseases
Peripheral neuropathy
medicine.anatomical_structure
Churg-Strau
vasculiti
Cohort
Female
business
Granulomatosis with polyangiitis
Immunosuppressive Agents
Subjects
Details
- Language :
- English
- ISSN :
- 14620324
- Database :
- OpenAIRE
- Journal :
- Rheumatology, Vol. 60, No 9 (2021) pp. 4355-4360
- Accession number :
- edsair.doi.dedup.....5691fa9d0497c5d0a01ec11b26b85623