Este protocolo aborda una presentación grave y urgente de la nefritis lúpica: glomerulonefritis rápidamente progresiva (GNRP) caracterizada por un deterioro veloz de la función renal junto con formación extensa de semilunas confirmada por biopsia que afecta a más del 50% de los glomérulos.
Enfoque Terapéutico resumen parcial
El enfoque para esta presentación incluye terapia inicial de pulsos de corticosteroides intravenosos en dosis altas seguida de inmunosupresión intensiva — con ciclofosfamida intravenosa como la opción más ampliamente estudiada en este subgrupo grave. La inmunosupresión de mantenimiento sigue una vez establecida la respuesta, y se incorporan medidas protectoras específicas para los grupos de pacientes en riesgo. El régimen completo, incluida la selección de agentes, la secuencia y la estrategia de mantenimiento completa, se encuentra en el protocolo estructurado a continuación.
Objetivos del Tratamiento
Los objetivos clave incluyen la preservación o mejora de la función renal al menos al 80% del valor basal en 3 meses, reducción de la proteinuria de al menos el 25% a los 3 meses y el 50% a los 6 meses, con un objetivo de proteinuria a los 12 meses y un umbral definido para la respuesta renal completa.
References
DOI: 10.1016/j.ard.2025.09.007
This recommendation refers to the specific subset of patients who present with rapidly progressive glomerulonephritis, ie, a rapid decline in kidney function accompanied by histologic evidence of extensive crescent formation (typically affecting >50% of the glomeruli).
In patients with rapidly progressive glomerulonephritis, a short course (6–7 monthly pulses) of high-dose intravenous cyclophosphamide can also be considered.
Although such patients can still be treated with the regimens mentioned in recommendation #4, a short course of high-dose intravenous CYC (modified traditional NIH regimen: 0.5–0.75 g/m² monthly for 6 months, total 6–7 pulses) is an additional option, since it remains the therapeutic regimen most studied in severe LN.
Combination with monthly pulse methylprednisolone has been shown to improve long-term renal outcome without adding toxicity; therefore, the addition of monthly intravenous pulses of methylprednisolone (typically 1 pulse prior to CYC administration) is left to the physician’s discretion.
Administration of monthly gonadotropin-releasing hormone analogs is recommended in premenopausal women who receive high-dose intravenous CYC to maximise the possibility of ovarian preservation.
Mycophenolate or azathioprine should replace cyclophosphamide for those initially treated with cyclophosphamide, alone or in combination with belimumab.
Treatment should aim for optimisation (preservation or improvement) of kidney function within 3 months, accompanied by a reduction in proteinuria of at least 25% by 3 months, 50% by 6 months, and a UPCR target <700 mg/g by 12 months, and as low as possible afterwards.
Together with proteinuria, stabilisation (if not improvement) of GFR to ≥80% of baseline value is desirable within the first 3 months to ensure that the patient is not deteriorating and in need of reevaluation of the treatment regimen.
Complete renal response should be defined as UPCR <500 mg/g at any time point.
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