Sauza-Sosa Julio C, Zenteno-Langle Raul, Zamora-Medina Maria Del C
Cardiology and Echocardiography Department, The American British Cowdray Medical Center, Av. Carlos Graef Fernández 154-410, Tlaxcala, 05300, Mexico City, Mexico.
Nephrology Department, Hospital Sedna, Mexico City, Mexico.
High Blood Press Cardiovasc Prev. 2020 Dec;27(6):597-599. doi: 10.1007/s40292-020-00411-z. Epub 2020 Oct 1.
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.
系统性硬化症（SSc）是一种罕见的自身免疫性疾病，可导致皮肤和皮下组织纤维化，并累及心脏、肺、肾和胃肠道等其他器官。此外，它还可引起肺动脉高压。硬皮病肾危象（SRC）是SSc最可怕的并发症之一。SRC是一种医疗急症，可表现为高血压脑病的临床症状。其病理生理学涉及在数天至数周内突然出现中度至重度高血压；它与血浆肾素活性增加和急性肾损伤有关。已知通过使用血管紧张素转换酶抑制剂，SRC的死亡率会显著降低。肾活检在诊断和及时治疗中起着重要作用。我们报告一例具有高血压急症和急性肾损伤典型表现的SRC临床病例。
