The European League Against Rheumatism (EULAR) has developed recommendations for the management of small and medium vessel vasculitis, a group of rare and challenging conditions affecting blood vessels. The recommendations are based on a systematic literature search and expert consensus, with 15 statements addressing evaluation, investigation, treatment, and monitoring. Key points include the importance of collaboration with centers of expertise, ANCA testing, structured clinical assessments, and the use of immunosuppressive therapies such as cyclophosphamide, methotrexate, and glucocorticoids. The recommendations also highlight the need for long-term follow-up and monitoring, especially for patients with refractory disease or those at risk of relapse. Additionally, the document proposes a research agenda to address unexplained issues, such as persistent hematuria in patients exposed to cyclophosphamide. These guidelines aim to provide a practical framework for healthcare professionals managing these complex conditions.The European League Against Rheumatism (EULAR) has developed recommendations for the management of small and medium vessel vasculitis, a group of rare and challenging conditions affecting blood vessels. The recommendations are based on a systematic literature search and expert consensus, with 15 statements addressing evaluation, investigation, treatment, and monitoring. Key points include the importance of collaboration with centers of expertise, ANCA testing, structured clinical assessments, and the use of immunosuppressive therapies such as cyclophosphamide, methotrexate, and glucocorticoids. The recommendations also highlight the need for long-term follow-up and monitoring, especially for patients with refractory disease or those at risk of relapse. Additionally, the document proposes a research agenda to address unexplained issues, such as persistent hematuria in patients exposed to cyclophosphamide. These guidelines aim to provide a practical framework for healthcare professionals managing these complex conditions.