Este protocolo aborda uma apresentação grave e urgente da nefrite lúpica: glomerulonefrite rapidamente progressiva (GNRP), caracterizada por deterioração rápida da função renal associada a formação extensa de crescentes confirmada por biópsia, afetando mais de 50% dos glomérulos.
Abordagem Terapêutica visão parcial
A abordagem para esta apresentação inclui terapia inicial com pulso intravenoso de corticosteroide em alta dose, seguida de imunossupressão intensiva — com ciclofosfamida intravenosa representando a opção mais amplamente estudada neste subgrupo grave. A imunossupressão de manutenção é iniciada após o estabelecimento de resposta, e medidas protetoras específicas são incorporadas para grupos de pacientes de risco. O regime completo, incluindo seleção de agentes, sequenciamento e a estratégia completa de manutenção, está no protocolo estruturado abaixo.
Objetivos do Tratamento
As metas principais incluem preservação ou melhora da função renal para pelo menos 80% do valor basal em 3 meses, redução da proteinúria de pelo menos 25% em 3 meses e 50% em 6 meses, com uma meta de proteinúria em 12 meses e um limiar definido para resposta 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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