Acta Scientific Clinical Case Reports

Case ReportVolume 3 Issue 5

Unexpected Evolution of Multiple Aneurysms as a Complication of p-ANCA Exchanged Vasculitis with Prednisolone Therapy: A Case Report and Review of the Literature

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

Abstract

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

Bibliography

  1. O'Sullivan KM and Holdsworth SR. “Neutrophil Extracellular Traps: A Potential Therapeutic Target in MPO-ANCA Associated Vasculitis?” Frontiers in Immunology 12 (2021): 635188.
  2. Geetha D and Jefferson JA. “ANCA-Associated Vasculitis: Core Curriculum 2020”. American Journal of Kidney Diseases 75 (2020): 124-137.
  3. Kawasaki A and Tsuchiya N. “Advances in the genomics of ANCA-associated vasculitis-a view from East Asia”. Genes Immunity 22 (2021): 1-11.
  4. Kronbichler A., et al. “Immunopathogenesis of ANCA-Associated Vasculitis”. International Journal of Molecular Sciences 21 (2020).
  5. Ayoub I and Nachman PH. “Advances in ANCA-associated vasculitis and lupus nephritis”. Nature Reviews Nephrology 17 (2021): 89-90.
  6. Singhal M., et al. “Imaging in small and medium vessel vasculitis”. International Journal of Rheumatic Diseases 22 (2019): 78-85.
  7. Moiseev S., et al. “2020 international consensus on ANCA testing beyond systemic vasculitis”. Autoimmune Review 19 (2020): 102618.
  8. Zheng Y., et al. “Central Nervous System Involvement in ANCA-Associated Vasculitis: What Neurologists Need to Know”. Frontier in Neurology 9 (2018): 1166.
  9. Arzoun H., et al. “Recent Advancements in the Management of Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review”. Cureus 14 (2022): e21814.
  10. Arman F., et al. “Antineutrophil cytoplasmic antibody-associated vasculitis, update on molecular pathogenesis, diagnosis, and treatment”. International Journal of Nephrology and Renovascular Disease 11 (2018): 313-319.
  11. Li SS., et al. “The pathogenesis and treatment in antineutrophil cytoplasmic antibody associated vasculitis”. American Journal of Translational Research 12 (2020): 4094-4107.
  12. Gibson KM., et al. “Autoantibodies Against Lysosome Associated Membrane Protein-2 (LAMP-2) in Pediatric Chronic Primary Systemic Vasculitis”. Frontiers in Immunology 11 (2020): 624758.
  13. Li N., et al. “Serum lysosomal-associated membrane protein-2 levels are increased in small and medium-vessel vasculitis, especially in polyarteritis nodosa”. Clinical and Experimental Rheumatology 117 (2019): 79-85.
  14. Chen Z., et al. “Study on the association of serum pentraxin-3 and lysosomal-associated membrane protein-2 levels with disease activity in Chinese Takayasu's arteritis patients”. Clinical and Experimental Rheumatology 117 (2019): 109-115.
  15. Kain R and Rees AJ. “What is the evidence for antibodies to LAMP-2 in the pathogenesis of ANCA associated small vessel vasculitis?” Current Opinion on Rheumatology 25 (2013): 26-34.
  16. Peschel A., et al. “Autoantibodies to hLAMP-2 in ANCA-negative pauci-immune focal necrotizing GN”. Journal of the American Society of Nephrology 25 (2014): 455-463.
  17. Danlos FX., et al. “Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome”. Autoimmune Review 16 (2017): 1036-1043.
  18. Erden A., et al. “Do ANCA-associated vasculitides and IgG4-related disease really overlap or not?” International Journal of Rheumatic Diseases 22 (2019): 1926-1932.

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.