Este protocolo aborda a glomerulonefrite rapidamente progressiva (GNRP) ANCA-positiva — uma apresentação para a qual existe evidência relativamente robusta. A GNRP pauci-imune ANCA-negativa é tratada de forma idêntica e se enquadra no mesmo referencial de diretrizes.
A diretriz confere ênfase especial às apresentações complicadas por disfunção renal avançada ou hemorragia alveolar difusa. Essas características de alto risco determinam se uma intervenção adicional é acrescentada à base imunossupressora.
These guidelines focus on ANCA-positive RPGN, which appears frequently and for which there is relatively strong evidence, and on addressing the severe primary diseases, namely lupus nephritis and anti-GBM antibody RPGN.
For ANCA-negative pauci-immune RPGN, we recommend that the treatment be similar to that of ANCA-positive disease.
In patients with ANCA-positive RPGN complicated with advanced renal dysfunction or pulmonary hemorrhage, the addition of plasmapheresis to immunosuppressive therapy as the initial therapy may improve renal function and survival. We recommend the addition of plasmapheresis in such patients.
The addition of plasmapheresis to the initial therapy with corticosteroids and cyclophosphamide is indicated for patients presenting with advanced kidney failure (serum creatinine, [5.8 mg/dL) or those with diffuse alveolar hemorrhage.
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