| Summary: | Diffuse alveolar hemorrhage and acute glomerulonephritis characterize pulmonary-renal syndrome, in which anti-glomerular basement membrane antibodies (anti-GBM) and anti-neutrophil cytoplasmic antibodies (ANCA) are often assessed. The treatment is complex, requiring a multidisciplinary approach, based on immunosuppressant therapies, mechanical ventilation, plasma exchange (PLEX), and kidney replacement therapy. This clinical case describes a 7-year-old female hospitalized for edema, hypertension, and acute kidney failure. Laboratory tests showed the coexistence of ANCA and anti-GBM autoantibodies. A kidney biopsy revealed necrotizing crescentic glomerulonephritis with linear deposits of IgG along the GBM. Corticosteroids and cyclophosphamide, followed by rituximab and PLEX represented the therapeutical strategies. Hemodialysis was needed for the fluid overload and acute kidney injury (AKI) management. Posterior reversible leukoencephalopathy syndrome (PRES), hemorrhagic alveolitis, and cutaneous necrotizing skin lesions requiring the amputation of the limb complicated the clinical course, until the death of the patient. Early diagnosis and multidisciplinary and personalized therapies represent the management dilemmas for this disease. The evaluation of new treatments, such as avacopan and imlifidase, is much needed in pediatric-onset disease.
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