Causes of death in ADPKD

Case: 58 years old male with a history of End-Stage Renal Disease (ESRD) due to Autosomal Dominant Polycystic Kidney Disease (ADPKD) was initially admitted for community-acquired pneumonia on a medical ward. Patient had a "code" and transferred to ICU. The most common cause of death in patients with ADPKD is? (select one)

A) cardiac disease 

B) infection/sepsis 

C) ruptured intracranial aneurysm 

D) hypertensive intracerebral hemorrhage 

E) renal cancer

Answer: A

In contrast to the conventional belief that most ADPKD patients die due to a neurologic event, the most common cause of death in these patients is cardiac. The majority of these patients develop hypertrophy and coronary disease. Only 12 percent of patients die due to hypertensive intracerebral hemorrhage (choice C) or ruptured intracranial aneurysm (choice D), a commonly associated finding in ADPKD. Infection (choice B) can be a cause too but renal cancer (choice E) has no relevance in ADPKD patients.

#nephrology

References:

1. Perrone RD, Ruthazer R, Terrin NC. Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality. Am J Kidney Dis 2001; 38:777. 

2. Fick GM, Johnson AM, Hammond WS, Gabow PA. Causes of death in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1995; 5:2048.