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Ask the Experts - Patient With Malignant Nephrosclerosis?
A 19-year-old female patient presents with acute renal failure due to malignant nephrosclerosis. Peritoneal dialysis was started, and the patient was sent home with instructions on how to perform continuous ambulatory peritoneal dialysis; hemodialysis is not an option, and the possibility of a donor organ is slim. What is the long-term prognosis for this patient, considering that she has no other organ dysfunction and her hypertension is well controlled?
Although the clinical details presented concerning this case are limited, a few general comments can be made in response to this question. First, the patient's prognosis will likely be heavily influenced by the underlying etiology of her malignant hypertension. Possible etiologies include renal artery stenosis; scleroderma, vasculitis (systemic lupus erythematosus, antineutrophil cytoplasmic autoantibody [ANCA]-associated disease, so-called microscopic polyarteritis); thrombotic microangiopathies, such as thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, underlying parenchymal renal disease, drug-related (especially cocaine, methamphetamines); and essential hypertension. Essential hypertension would be an unusual cause of malignant hypertension in a young female unless she is black. Even with that racial background, I would look thoroughly for an underlying etiology other than essential hypertension. It is conceivable that specific treatment for one of these underlying etiologies might lead to a significant improvement in renal function and allow cessation of dialysis. For example, there are documented cases in which correction of renal artery stenosis has restored enough renal function to allow an individual to stop dialysis, even after a significant period of dialysis therapy. Another consideration is that the nature of the underlying process may have a bearing on her course and management after renal transplantation, if she does eventually have the possibility of getting a kidney.
Regardless of the etiology of her malignant hypertension, there is a significant chance that with optimal control of her hypertension (by means of medication and ultrafiltration on dialysis), she could regain enough renal function to come off dialysis. The arterial and arteriolar lesions of malignant hypertension can heal, restoring enough renal blood flow and glomerular filtration to permit cessation of renal replacement therapy. Her urine volume and laboratory data should be monitored for evidence of improved function. It would also be wise to defer consideration of renal transplantation for several months because of the possibility of return of significant native kidney function. Even if there is short-term improvement in renal function, most patients will eventually have deterioration of renal function in the long term; degree of blood pressure control influences the likelihood of progressive renal dysfunction.
Even in the event that this patient must remain on dialysis, her prognosis is not necessarily much different from that of other renal failure patients of the same age. Prognosis will be heavily influenced by the underlying etiology of the hypertension. In the era before the availability of effective, potent antihypertensive agents or renal replacement therapy, the prognosis of malignant hypertension was grim, with most patients succumbing to end-organ damage (ie, heart attack, stroke, renal failure) within a few years at most. However, even if this patient's renal failure is permanent, if she currently has little or no end-organ damage in other target organs, her prognosis (with good blood pressure control) will be similar to that of other dialysis patients of the same age. Recent data suggest that her chances of long-term survival would be further increased by kidney transplantation, should that option be at all possible.
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