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We hope that readers have found this interactive case report interesting and educational. The rapid responses—from many countries, specialties, and grades—have been informative. We would like to thank BMJ readers for taking such an interest in the case and taking time to post their responses.
This patient presented with scleroderma renal crisis and features typical of accelerated hypertension—acute renal failure, pulmonary oedema, microangiopathic haemolysis, and hypertensive encephalopathy.
We agree with many responders that investigation for recurrent miscarriage was not necessary on the basis of the reproductive history alone, but that other features of her illness certainly warranted investigation. Investigation of accelerated hypertension may include endocrine testing, renovascular studies, serology, and renal biopsy. In our experience, white patients with this presentation often have an underlying cause.
As regards her management, basic resuscitation, adequate monitoring, and safe and timely transfer to a specialist unit were crucial. We used nitrates for pressure control in view of her volume overload and pulmonary oedema, followed by ultrafiltration and oral therapy. Some of the blood pressure agents suggested by responders would be equally efficacious, as would prostacycline. Most guidelines recommend initial lowering of diastolic pressure to 100-105 mm Hg over two to six hours, with an initial drop of no more than 25%. This can then be lowered to 85-90 mm Hg over several weeks. Acute dialysis (or haemofiltration) was clearly needed, and we felt early plasma exchange was justified given the possibility of primary TTP.
Our patient had an overwhelming illness, which evolved extremely rapidly—apparently “out of the blue.” She had a fortnight of intensive treatment and investigation and then had to adjust rapidly to the prospect of long term dialysis. She coped with these demands remarkably well.
In spite of advances in technology, mortality from acute renal disease remains high. Patients with such disease often present to non-specialists. Early recognition and treatment, with early involvement of nephrology and critical care services, is essential for a good outcome.
Competing interests: None declared.