Hypertensive Emergency in a Woman with Systemic Sclerosis
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CASE REPORT
Hypertensive Emergency in a Woman with Systemic Sclerosis Julio C. Sauza‑Sosa1 · Raul Zenteno‑Langle2 · Maria del C. Zamora‑Medina3 Received: 12 July 2020 / Accepted: 12 September 2020 © Italian Society of Hypertension 2020
Abstract Systemic sclerosis (SSc) is a rare autoimmune disease that causes fibrosis in the skin and subcutaneous tissue, involving other organs such as the heart, lungs, kidneys, and gastrointestinal tract. Additionally, it can cause pulmonary arterial hypertension. Scleroderma renal crisis (SRC) is one of the most dreadful complications of SSc. SRC is a medical emergency that can present as a clinical picture of hypertensive encephalopathy. The pathophysiology involves an abrupt onset of moderate to severe hypertension that ranges from days to weeks; it is associated with an increase in plasma renin activity and acute kidney injury. It is known that by introducing angiotensin-converting enzyme inhibitors, the mortality decreases significantly in SRC. The renal biopsy plays an important role on the diagnosis and opportune treatment. We present a clinical case of SRC with a typical presentation of hypertensive emergency and acute kidney injury. Keywords Hypertensive emergency · Hypertensive encephalopathy · Scleroderma renal crisis · Systemic sclerosis
1 Introduction Systemic sclerosis (SSc) is a rare autoimmune disease that causes fibrosis in the skin and subcutaneous tissue involving other organs such as the heart, lungs, kidneys, and gastrointestinal tract. Additionally, it can cause pulmonary arterial hypertension. Scleroderma renal crisis (SRC) is one of the most dreadful complications of SSc. The incidence of SRC in patients with SSc ranges between 10 and 19% [1]. The pathophysiology involves an abrupt onset of moderate to severe hypertension ranging from days to weeks and its associated with an increase in plasma renin activity and acute kidney injury. It is known that by introducing Electronic supplementary material The online version of this article (https://doi.org/10.1007/s40292-020-00411-z) contains supplementary material, which is available to authorized users. * Julio C. Sauza‑Sosa [email protected] 1
Cardiology and Echocardiography Department, The American British Cowdray Medical Center, Av. Carlos Graef Fernández 154‑410, Tlaxcala, 05300 Mexico City, Mexico
2
Nephrology Department, Hospital Sedna, Mexico City, Mexico
3
Rheumatology and Immunology Department, National Institute of Medical Science and Nutrition “Salvador Zubiran”, Mexico City, Mexico
angiotensin-converting enzyme inhibitors (ACEi), the mortality decreases significantly in SRC [2]. The renal biopsy plays an important role on the diagnosis and opportune treatment. We present a clinical case of SRC with a typical presentation of hypertensive emergency and acute kidney injury.
2 Clinical Case A 74-year-old woman with history of hypothyroidism, SSc with interstitial lung disease, and pulmonary arterial hypertension arrived at emergency room because of sudden onset of headache and
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