DOI: 10.36347/sjmcr.2024.v12i06.033

Pages: 1102-1104

Wegener's granulomatosis, a rare disease, remains poorly understood. Its pathogenesis involves suspected immune, infectious, and environmental factors, but no definitive relationships have been established. Diagnosis is often delayed and relies on clinical, biological, and histological findings, necessitating an association with an ENT (ear, nose, and throat), pulmonary, and renal triad. ENT symptoms, particularly naso-sinus issues, are the most prominent indicators of the disease. To support the diagnosis, biological and immunological tests are essential. Biopsies, especially from the ENT region, are recommended when possible and safe. Histological confirmation is crucial, and renal biopsies provide valuable prognostic information, particularly in cases with renal manifestations. Treatment primarily involves corticosteroids and immunosuppressants. New biotherapies, such as rituximab, anti-TNF-α, and abatacept, are currently under investigation and show promise for significantly improving patient management in the future. Despite early treatment being crucial due to the disease's severe prognosis and potential complications, over 85% of cases achieve remission. Nonetheless, the high relapse rate remains a significant. highlights the need for ongoing research and development of more effective long-term treatment strategies to manage and mitigate relapses in this complex and challenging disease.