Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) manifest with involvement of the ear, nose, throat, skin, neurological system, and constitutional symptoms, as well as potentially life-threatening cardiac, renal, and pulmonary organ involvement leading to organ dysfunction. Early diagnosis is critical, necessitating increased awareness among clinicians. Advances in technology have facilitated the use of various laboratory, imaging, and interventional methods for diagnosis, differential diagnosis, and disease monitoring, with composite disease activity indices employed to assess disease activity and organ damage. In patients presenting with systemic features suggestive of AAV, such as cutaneous vasculitis, chronic upper and lower respiratory tract diseases, rapidly progressive renal impairment, or peripheral neuropathy, high-quality antigen-specific assays for proteinase-3 ANCA and myeloperoxidase ANCA should be performed as primary diagnostic tests. Management by multidisciplinary teams experienced in vasculitis is recommended. AAV treatment is divided into remission induction and maintenance phases; induction therapy for organ- or life-threatening disease typically includes glucocorticoids combined with rituximab- or cyclophosphamide-based regimens. Maintenance therapy, often with rituximab, follows remission to prevent relapse. While glucocorticoids remain a cornerstone of induction therapy, studies demonstrate that reduced-dose steroid regimens offer comparable efficacy to standard doses with lower infection risk. The introduction of biologics such as rituximab and mepolizumab has significantly decreased treatment-related damage associated with glucocorticoids and other immunosuppressants. Throughout follow-up, patients should be regularly monitored for treatment-related adverse effects and comorbidities (including hypertension, osteoporosis, and cardiovascular disease) with appropriate lifestyle modifications recommended to optimize long-term outcomes.