Ce protocole traite la glomérulonéphrite rapidement progressive (GNRP) ANCA-positive — une présentation pour laquelle les preuves sont relativement solides. La GNRP pauci-immune ANCA-négative est traitée de manière identique et relève du même cadre de recommandations.
La recommandation met particulièrement l'accent sur les présentations compliquées d'une insuffisance rénale avancée ou d'une hémorragie alvéolaire diffuse. Ces caractéristiques à haut risque déterminent si une intervention supplémentaire est adjointe à la base immunosuppressive.
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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