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You are here: Home / Articles / The History of ACE Inhibitors in Scleroderma Renal Crisis

The History of ACE Inhibitors in Scleroderma Renal Crisis

February 16, 2021 • By Ruth Jessen Hickman, MD

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Scleroderma renal crisis is a true medical emergency in rheumatology, one that requires prompt diagnosis and treatment. Here, we review the historic introduction of the angiotensin-converting enzyme inhibitors in this context, and highlight management and key questions moving forward.

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Background

Awareness of renal disease in scleroderma dates back many years. The revered physician William Osler noted in 1892 that patients with scleroderma were “apt to succumb to pulmonary complaints or to nephritis.”1 Awareness of the condition increased in 1952 with a case report of three patients appearing in The Lancet.2 More case reports and limited reviews of the topic were published, but the disease’s causes, incidence and disease course were not well characterized. 

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Once such case report from 1960 noted the aggressive, fulminant presentation of scleroderma renal crisis: “The hypertension is rapidly followed by retinopathy, cardiac failure, convulsions, profound oliguria and uraemia. The progression of the disease from the appearance of hypertension to death is usually so rapid that there is little time for clinical evidence of renal disease to become fully apparent.”1 The disease was almost always fatal, until the first reported successful nephrectomy followed by renal transplant in 1970.3

In 1974, a seminal paper on scleroderma and renal disease appeared, a clinicopathological review of 210 patients with scleroderma seen over the previous 20 years. The detailed 46-page report included thorough case studies, clinical evaluations, renal blood flow measures, such lab analyses as renin, treatment outcomes and information about renal histopathology. The writers also hypothesized about the pathogenic role of renin release in scleroderma renal crisis.4

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The research team, headed by E. Carwile LeRoy, MD, found evidence of some type of renal involvement in 45% of scleroderma patients, lumping together such markers as proteinuria, hypertension and azotemia. Some of these patients had only low-level, asymptomatic renal disease. Critically, the team reported that the syndrome of malignant hypertension (scleroderma renal crisis), characterized by rapidly progressive renal damage, occurred in 7% of their patients and was fatal in 12 out of these 15 cases. For all patients, scleroderma renal crisis was the leading cause of death.4

Dr. Whitman

Dr. Whitman

Hendricks H. Whitman III, MD, FACP, an assistant professor of medicine at Weill Cornell Medical College, New York City, notes, “In those days, if scleroderma patients became hypertensive and their kidneys began to be damaged, that’s what actually killed them. Scleroderma patients had about a 50% mortality in 10 years. And almost all of them died from hypertension or renal failure.”

A few scattered reports appeared of successful aggressive medical management of scleroderma renal crisis with such agents as minoxidil, but the prognosis was grim.5,6 Virginia D. Steen, MD, a professor and chief of the Division of Rheumatology of the Department of Medicine at Georgetown University in Washington, D.C., recounts, “Even when I was a fellow, we kept trying all these kinds of medications, and nothing did anything. I remember vividly as a student, being in a conference where they decided to do a bilateral nephrectomy on a young woman as the only way to save her life.” 

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Dr. Whitman recalls how patients with scleroderma and baseline hypertension were managed then. “We didn’t know any better in those days, so we’d give them a diuretic, which would actually make things worse rather than better, or we’d give them a beta blocker, and that would be really bad for their Raynaud’s phenomenon.” Some rheumatologists observed that scleroderma renal crisis was sometimes triggered by diuretic therapy initiated to control a baseline high blood pressure.6

Pages: 1 2 3 4 5 | Single Page

Filed Under: Conditions, Scleroderma Tagged With: ACE inhibitors, Lost & Found, Renal diseaseIssue: February 2021

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About Ruth Jessen Hickman, MD

Ruth Jessen Hickman, MD, was born and raised in eastern Kentucky, where she first cultivated her love of literature, writing and personal narratives. She attended Kenyon college, where she received a Bachelor of Arts in philosophy, summa cum laude. She worked with individuals with psychiatric conditions and later in a neuroscience lab at the University of Illinois, Chicago, before graduating from Indiana University Medical School in 2011. Instead of pursuing clinical medicine, Ruth opted to build on her strength of clearly explaining medical topics though a career as a freelance medical writer, writing both for lay people and for health professionals. She writes across the biomedical sciences, but holds strong interests in rheumatology, neurology, autoimmune diseases, genetics, and the intersection of broader social, cultural and emotional contexts with biomedical topics. Ruth now lives in Bloomington, Ind., with her husband, son and cat. She can be contacted via her website at ruthjessenhickman.com.

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