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Goodpasture Syndrome Definition — ESENeph MCQ

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HardRenal VasculitisGoodpasture Syndrome DefinitionESENeph

A dialysis-dependent patient has biopsy-proven anti-GBM disease and active pulmonary haemorrhage. Does dialysis dependence remove the indication for immunosuppression and plasma exchange?

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Correct answer: ATreat pulmonary anti-GBM disease despite dialysis dependence

The best answer is “Treat pulmonary anti-GBM disease despite dialysis dependence”. Even when kidney recovery is unlikely, pulmonary haemorrhage requires urgent disease-specific therapy. The exception to aggressive treatment in dialysis-dependent patients applies only when there is no pulmonary haemorrhage and biopsy shows extensive irreversible injury. “Yes; dialysis dependence makes pulmonary haemorrhage self-limiting” is less appropriate because lung bleeding remains life-threatening and treatable “Use dialysis alone because it removes anti-GBM antibody efficiently” is less appropriate because standard dialysis does not provide therapeutic antibody exchange “Give antibiotics as isolated treatment” is less appropriate because anti-GBM pulmonary-kidney syndrome is autoimmune “Wait for renal recovery before treating the lungs” is less appropriate because treatment delay risks fatal haemorrhage

Reference: KDIGO 2021 glomerular diseases guideline: https://kdigo.org/wp-content/uploads/2021/10/KDIGO-2021-Guideline-for-the-Management-of-Glomerular-Diseases.pdf