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.
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Nihtyanova SI, Tang EC, Coghlan JG, Wells AU, Black CM, Denton CPL. Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 2010;103:109–15. https://doi.org/10.1093/qjmed/hcp174.
Steen VD, Costantino JP, Shapiro AP, Medsger TA Jr. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med. 1990;113(5):352–7. https://doi.org/10.7326/0003-4819-113-5-352.
Batal I, Domsic RT, Medsger TA, Bastacky S. Scleroderma renal crisis: a pathology perspective. Int J Rheumatol. 2010. https://doi.org/10.1155/2010/543704.
Bose N, Chiesa-Vottero A, Chatterjee S. Scleroderma renal crisis. Semin Arthritis Rheum. 2015;44:687–94. https://doi.org/10.1016/j.semarthrit.2014.12.001.
Hudson M, Baron M, Tatibouet S, Furst DE, Khanna D. International Scleroderma Renal Crisis Study I. Exposure to ACE inhibitors prior to the onset of scleroderma renal crisis-results from the International Scleroderma Renal Crisis Survey. Semin Arthritis Rheum. 2014;43(5):666–72. https://doi.org/10.1016/j.semarthrit.2013.09.008.
Mouthon L, Bussone G, Berezne A, Noël LH. Scleroderma renal crisis. J Rheumatol. 2014;41(6):1040–8. https://doi.org/10.3899/jrheum.131210.
Van Den Born BH, Lip GYH, Brguljan-Hitij J, Cremer A, Segura J, Morales E, Mahfoud F, Amraoui J, Persu A, Kahan T, AgabitiRosei E, de Simone G, Gose P, Williams B. ESC Council on hypertension position document on the management of hypertensive emergencies [published correction appears in Eur Heart J CardiovascPharmacother. 2019 Jan 1;5(1):46]. Eur Heart J CardiovascPharmacother. 2019;5(1):37–46. https://doi.org/10.1093/ehjcvp/pvy032.
Stern E, Steen V, Denton C. Diagnosis, management and prevention of renal involvement in scleroderma. Curr Treat Opt Rheumatol. 2015;1(1):106–18. https://doi.org/10.1097/BOR.0b013e3283108df7.
Montrief T, Koyfman A, Long B. Scleroderma renal crisis: a review for emergency physicians. Intern Emerg Med. 2019;14(4):561–70. https://doi.org/10.1007/s11739-019-02096-2.
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Sauza-Sosa, J.C., Zenteno-Langle, R. & Zamora-Medina, M.C. Hypertensive Emergency in a Woman with Systemic Sclerosis. High Blood Press Cardiovasc Prev 27, 597–599 (2020). https://doi.org/10.1007/s40292-020-00411-z
- Hypertensive emergency
- Hypertensive encephalopathy
- Scleroderma renal crisis
- Systemic sclerosis