80 - A Scleroderma Crisis
Nov 8, 2023
This podcast explores the effectiveness of ACE inhibitors in treating scleroderma renal crisis, highlighting the reduction in mortality rates. It delves into the mechanisms of bilateral nephrectomy and ACE inhibitors in the disease. The physiology of scleroderma crisis and potential treatment options are discussed, including the discovery of Capripl through ACE inhibition. The use of ACE inhibitors in managing vasoconstriction associated with elevated renin levels is also explored.
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ACE Inhibitors' Paradoxical Use
- ACE inhibitors treat scleroderma renal crisis, which is counterintuitive to their typical use in acute kidney injury.
- This sparked Tony Brew's interest in the topic.
Scleroderma Renal Crisis Features
- Key features of scleroderma renal crisis include having scleroderma, abrupt hypertension, and oliguric acute kidney injury.
- A typically normal urinalysis with severe AKI and potential microangiopathic hemolytic anemia are also characteristic.
Poor Outcomes Pre-1970s
- Before the 1970s, scleroderma with kidney involvement was often fatal, especially renal crisis.
- A 1973 study showed that all 17 male veterans with kidney involvement died within 10 months.
