Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are a group of inflammatory disorders in which autoantibodies damage small arteries throughout the body, including in the upper and lower respiratory system, kidneys, as well as the skin. AAV may be precipitated by a variety of causes, including infections. In this report, we examine the case of a patient who developed AAV that was suspected primarily based on mucocutaneous hemorrhagic bullae, elevated ANCA levels, and subsequently confirmed by kidney biopsy, while recovering from coronavirus disease 2019 (COVID-19) infection. AAV and COVID-19 infections may present with similar symptoms, rendering an accurate diagnosis challenging. Additionally, only a few other cases describing a similar onset of AAV post-COVID-19 infection have been described in the literature. Initial presenting features of AAV in such cases have varied considerably, which makes the diagnosis even more challenging. We also engage in a review of such cases to assess key similarities, different treatment options, and outcomes. Lastly, the fact that several mechanisms have been proposed for AAV highlights the need for continued research to help clarify the pathophysiology while also identifying the optimal therapy.
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http://dx.doi.org/10.7759/cureus.31441 | DOI Listing |
Int Immunopharmacol
January 2025
Department of Pulmonary and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu 610041, People's Republic of China; State Key Laboratory of Respiratory Health and Multimorbidity, West China Hospital, Sichuan University, Chengdu 610041, People's Republic of China. Electronic address:
Objectives: Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a rare and potential devastating disease with high mortality, frequently with pulmonary involvement. Our study aimed to explore the pulmonary features of AAV and identify predictors of long-term survival.
Methods: We retrospectively analyzed 538 AAV patients diagnosed between January 2013 and July 2019, with follow-up data extending to August 2020.
J Rheum Dis
January 2025
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Oman Med J
July 2024
Department of Medicine, Faculty of Medicine, Royal College of Medicine, Universiti Kuala Lumpur, Perak, Malaysia.
Polyangiitis overlap syndrome (POS) is a systemic vasculitis characterized by overlapping features of more than one well-defined vasculitic syndrome. We present the case of a 38-year-old Malay man with progressive dyspnea and palpable purpura in his lower limbs. The diagnostic evaluation revealed right-sided segmental pulmonary consolidation with pleural effusion, systolic cardiac dysfunction with the presence of an intracardiac thrombus, and left vocal cord palsy secondary to laryngeal mononeuropathy.
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December 2024
Department of Medicine, Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, 55 Fruit St, Yawkey 4B, Boston, MA, USA.
Objectives: Patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) face excess mortality compared with the general population. Mortality in clinical epidemiology research is often examined using death certificate diagnosis codes; however, the sensitivity of such codes in AAV is unknown.
Methods: We performed a retrospective cohort study using the Mass General Brigham AAV Cohort, including patients with AAV who died between 2002 and 2019.
PeerJ
December 2024
Department of Nephrology, The Second Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, China.
Background: Heredity and epigenetics affect the pathogenesis of microscopic polyangiitis (MPA). Tyrosine kinase 2 (TYK2) polymorphisms (rs2304256C > A, rs280519A > G, and rs12720270G > A) may be potential protective factors against anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Current research suggests that TYK2 is associated with various autoimmune diseases; however, no study has examined the relationship between TYK2 polymorphisms and AAV.
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