The European League Against Rheumatism (EULAR) has developed recommendations for the management of primary small and medium vessel vasculitis. These recommendations aim to guide the evaluation, investigation, treatment, and monitoring of patients with conditions such as Wegener granulomatosis (WG), microscopic polyangiitis (MPA), Churg–Strauss syndrome (CSS), essential cryoglobulinemic vasculitis, and polyarteritis nodosa (PAN). The recommendations are based on a systematic review of the literature and expert consensus, with a focus on evidence-based practices. Key recommendations include managing patients with primary small and medium vessel vasculitis in collaboration with or at centres of expertise. Anti-neutrophil cytoplasmic antibody (ANCA) testing is recommended in appropriate clinical contexts, with a focus on detecting cytoplasmic or perinuclear patterns. A positive biopsy is strongly supportive of vasculitis and is recommended for diagnosis and further evaluation. A structured clinical assessment, urine analysis, and basic laboratory tests are recommended at each clinical visit. Patients with ANCA-associated vasculitis should be categorized by severity to assist treatment decisions. For remission induction, a combination of cyclophosphamide and glucocorticoids is recommended, with pulsed intravenous cyclophosphamide showing better remission rates than continuous oral therapy. Methotrexate is recommended as a less toxic alternative for non-organ threatening or non-life threatening ANCA-associated vasculitis. High-dose glucocorticoids are an important part of remission induction therapy. Plasma exchange is recommended for selected patients with rapidly progressive severe renal disease to improve renal survival. Remission maintenance therapy should involve low-dose glucocorticoids and either azathioprine, leflunomide, or methotrexate. Alternative immunomodulatory therapy should be considered for patients who do not achieve remission or relapse on standard therapy, with referral to an expert centre and enrollment in clinical trials. For hepatitis C-associated cryoglobulinaemic vasculitis, antiviral therapy is recommended. For hepatitis B-associated PAN, a combination of antiviral therapy, plasma exchange, and glucocorticoids is recommended. Patients with mixed essential cryoglobulinemic vasculitis should be treated similarly to other small vessel diseases, with rituximab as a potential option. The recommendations emphasize the importance of long-term follow-up, monitoring for adverse effects, and the need for periodic assessments. They also highlight the importance of research agendas to improve diagnostic criteria, identify biomarkers, and conduct adequately powered randomized controlled trials for alternative treatments. The recommendations are intended for healthcare professionals, medical students, specialist trainees, and pharmaceutical industries.The European League Against Rheumatism (EULAR) has developed recommendations for the management of primary small and medium vessel vasculitis. These recommendations aim to guide the evaluation, investigation, treatment, and monitoring of patients with conditions such as Wegener granulomatosis (WG), microscopic polyangiitis (MPA), Churg–Strauss syndrome (CSS), essential cryoglobulinemic vasculitis, and polyarteritis nodosa (PAN). The recommendations are based on a systematic review of the literature and expert consensus, with a focus on evidence-based practices. Key recommendations include managing patients with primary small and medium vessel vasculitis in collaboration with or at centres of expertise. Anti-neutrophil cytoplasmic antibody (ANCA) testing is recommended in appropriate clinical contexts, with a focus on detecting cytoplasmic or perinuclear patterns. A positive biopsy is strongly supportive of vasculitis and is recommended for diagnosis and further evaluation. A structured clinical assessment, urine analysis, and basic laboratory tests are recommended at each clinical visit. Patients with ANCA-associated vasculitis should be categorized by severity to assist treatment decisions. For remission induction, a combination of cyclophosphamide and glucocorticoids is recommended, with pulsed intravenous cyclophosphamide showing better remission rates than continuous oral therapy. Methotrexate is recommended as a less toxic alternative for non-organ threatening or non-life threatening ANCA-associated vasculitis. High-dose glucocorticoids are an important part of remission induction therapy. Plasma exchange is recommended for selected patients with rapidly progressive severe renal disease to improve renal survival. Remission maintenance therapy should involve low-dose glucocorticoids and either azathioprine, leflunomide, or methotrexate. Alternative immunomodulatory therapy should be considered for patients who do not achieve remission or relapse on standard therapy, with referral to an expert centre and enrollment in clinical trials. For hepatitis C-associated cryoglobulinaemic vasculitis, antiviral therapy is recommended. For hepatitis B-associated PAN, a combination of antiviral therapy, plasma exchange, and glucocorticoids is recommended. Patients with mixed essential cryoglobulinemic vasculitis should be treated similarly to other small vessel diseases, with rituximab as a potential option. The recommendations emphasize the importance of long-term follow-up, monitoring for adverse effects, and the need for periodic assessments. They also highlight the importance of research agendas to improve diagnostic criteria, identify biomarkers, and conduct adequately powered randomized controlled trials for alternative treatments. The recommendations are intended for healthcare professionals, medical students, specialist trainees, and pharmaceutical industries.