Ren Yan, Yang Ya, Sun Fei, Wei Yufei and Guo Weihua*
Unit 4 of Cardiovascular Center, Dongzhimen Hospital, Beijing University of Chinese Medicine, Beijing, China
*Corresponding Author: Guo Weihua, Unit 4 of Cardiovascular Center, Dongzhimen Hospital, Beijing University of Chinese Medicine, Beijing, China.
Received: March 30, 2022; Published: April 08, 2022
Background: ANCA-associated vasculitis (AAV) is a rare and potentially fatal systemic autoimmune disease characterized by inflammation and fibrinoid necrosis of the vascular wall, primarily triggering small-vessel pauci-immune vasculitis. Coronary artery aneurysms (CAAs) are an extremely uncommon finding though medium-size vessels can sometimes be involved, here we describe a case of multiple medium-size vessel impairment of AAV. To our knowledge, this is the first documented case of unexpected evolution of multiple vasculature involvement as a complication in a patient of plasma p-ANCA exchanged from positive to negative after tapering of Prednisolone (PSL) therapy. Case Report: A 59-year-old Chinese male whose previous chief complain was chest pain and dizzy with history of MPO-ANCA-associated glomerulonephritis was referred to our hospital because of angina. On admission, laboratory data showed sedimentation rate of red cells was 18mm/H and immunoglobulin G was 20.79 g/L, while other specific antibodies and blood tests were negative. His angiography demonstrated vasculitis with multiple vessel wall irregularities, abrupt terminations, and giant aneurysms typically found in carotid arteries, cerebral arteries as well as coronary arteries, which indicating medium-size vessels impairment. More importantly, we have noted that his vascular impairment relapsed insidiously after tapering of Prednisolone (PSL). The diagnosis of ANCA-associated vasculitis was confirmed on the basis of the clinical presentation in the end, however, we missed the therapeutic opportunity to reverse his vascular impairment with initial misdiagnosis. Discussion: The pathogenesis of AAV is multifaceted and numerous factors may be involved except ANCA-induced activation of cytokine-primed neutrophils and the formation of neutrophils extracellular traps. Once kidney and lung are involved, the fatality rate is high and the prognosis is poor. Therefore, healthcare providers should be aware of vascular lesions complications either in clinical or imageological examination and mechanism of these diseases including plasma ANCA exchanged AAV, of which mechanistic insights may have potential to open new therapeutic strategies for them. Keywords: p-ANCA Exchanged Vasculitis; Angina; Multiple Aneurysms; Prednisolone
Citation: Guo Weihua., et al. “Unexpected Evolution of Multiple Aneurysms as a Complication of p-ANCA Exchanged Vasculitis with Prednisolone Therapy: A Case Report and Review of the Literature". Acta Scientific Clinical Case Reports 3.5 (2022): 26-30.
Copyright: © 2022 Guo Weihua., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.