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Polycystic Kidney Disease (ADPKD) — Written Clinical Reasoning
DCE written preparation: structured reasoning for ADPKD scenarios — the new diagnosis in a young man with preserved GFR, and the tolvaptan decision in a woman with rapid progression.
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SAQ — ADPKD: new diagnosis and the tolvaptan decision (20 marks, 30 minutes)
Prompt: Answer both parts, addressing diagnosis and staging, management with the tolvaptan decision, extrarenal disease, and family counselling. Justify each decision with reference to evidence. [7]
Case vignette Part A: A 32-year-old man with an affected father (ESKD in his 50s) is found incidentally to have bilaterally enlarged cystic kidneys with preserved eGFR. [1]
Case vignette Part B: A 44-year-old long-haul flight attendant with ADPKD, Mayo class 1D, eGFR slope 4 to 5 mL/min/1.73 m² per year, whose sister died of subarachnoid haemorrhage. [3]
References9ShowHide
- [1]Pei Y, Obaji J, Dupuis A, et al. Unified criteria for ultrasonographic diagnosis of ADPKD J Am Soc Nephrol, 2009.PMID 18945943
- [2]Iliuta IA, Kalatharan V, Wang K, et al. Polycystic Kidney Disease without an Apparent Family History J Am Soc Nephrol, 2017.PMID 28522688
- [3]Irazabal MV, Rangel LJ, Bergstralh EJ, et al. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials J Am Soc Nephrol, 2015.PMID 24904092
- [4]Schrier RW, Abebe KZ, Perrone RD, et al. Blood pressure in early autosomal dominant polycystic kidney disease N Engl J Med, 2014.PMID 25399733
- [5]Torres VE, Chapman AB, Devuyst O, et al. Tolvaptan in patients with autosomal dominant polycystic kidney disease N Engl J Med, 2012.PMID 23121377
- [6]Torres VE, Chapman AB, Devuyst O, et al. Tolvaptan in Later-Stage Autosomal Dominant Polycystic Kidney Disease N Engl J Med, 2017.PMID 29105594
- [7]Kidney Disease: Improving Global Outcomes (KDIGO) ADPKD Work Group KDIGO 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) Kidney Int, 2025.PMID 39848759
- [8]Irazabal MV, Huston J 3rd, Kubly V, et al. Extended follow-up of unruptured intracranial aneurysms detected by presymptomatic screening in patients with autosomal dominant polycystic kidney disease Clin J Am Soc Nephrol, 2011.PMID 21551026
- [9]Wang CJ, Creed C, Winklhofer FT, et al. Water prescription in autosomal dominant polycystic kidney disease: a pilot study Clin J Am Soc Nephrol, 2011.PMID 20876670