Goodpasture Syndrome: current review on pathogenesis, diagnosis, and therapeutic approaches

Abstract

Goodpasture Syndrome is a rare autoimmune disease characterized by the presence of antibodies against the glomerular basement membrane, leading to rapidly progressive glomerulonephritis and pulmonary hemorrhage. This syndrome predominantly affects young males and is associated with genetic factors, such as the HLA-DRB1*1501 allele, in addition to environmental factors like tobacco exposure and respiratory infections. Clinical presentation can vary significantly, ranging from mild symptoms like fatigue and dyspnea to severe manifestations such as acute renal failure and massive alveolar hemorrhage, often resulting in delayed diagnosis and worsened prognosis. Diagnosis is confirmed by the detection of anti-glomerular basement membrane antibodies, with renal biopsy being necessary in atypical cases. Treatment involves aggressive immunosuppression with corticosteroids and cyclophosphamide, along with plasmapheresis to remove pathogenic antibodies. Recently, the use of biological agents, such as rituximab, has shown promising results, especially in refractory cases. However, mortality remains high, particularly in patients with severe renal involvement, and many survivors develop chronic complications such as hypertension and chronic kidney disease. Early identification and appropriate management are crucial for improving prognosis, and ongoing research is needed to enhance therapeutic options and reduce the morbidity and mortality associated with this devastating syndrome.