Eosinophilic granulomatosis with polyangiitis: diagnosis and treatment-related issues
نویسندگان
چکیده
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic necrotizing vasculitis of small to medium-sized vessels associated antineutrophil cytoplasmic antibodies (ANCA). EGPA usually diagnosed in patients bronchial asthma (BA) and characterized by predominant lesion the lungs, skin, peripheral nervous system. ANCAs myeloperoxidase are detected 1/3 EGPA. Presence or absence differentiates 2 disease phenotypes different clinical characteristics prognosis. New opportunities for treatment appeared after identification key role eosinophils development targeted drugs eosinophilic BA that being studied now. Aim review highlight modern approaches diagnosis EGPA, primarily through use biological therapy. Conclusion. multisystem ambivalent manifestations ANCA-mediated vessel injuries. Currently, there no “gold standard” although efficacy pharmacological therapy directly related early detection timely initiation treatment. Monoclonal targeting interleukin-5 (IL-5) an effective alternative conventional corticosteroids used alone combination immunosuppressants (cyclophosphamide induction azathioprine maintenance therapy) severe/refractory unfavorable The benefits anti-IL-5 drug mepolizumab were confirmed randomized controlled trial, this was approved new drugs, including ones, tested Pulmonologists rheumatologists should coordinate patient management improve results prognosis disease.
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Clinical and Experimental Rheumatology 2017 1Department of Internal Medicine, National Referral Centre for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance PubliqueHôpitaux de Paris (AP-HP), Université Paris Descartes, Paris; 2Department of Occupational and Environmental Diseases, Cochin Hospital, AP-HP, Université Paris Descartes, Paris, France. Lucas Benarous, MD* Benjamin T...
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ژورنال
عنوان ژورنال: Pul?monologiâ
سال: 2022
ISSN: ['0869-0189', '2541-9617']
DOI: https://doi.org/10.18093/0869-0189-2022-4118