Logee Kristin M, Lakshminarayanan Santhanam
Division of Rheumatology, University of Connecticut Health Center, Farmington, Connecticut, USA.
Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S171-4. Epub 2015 Mar 6.
Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) that is characterised by new-onset malignant hypertension and progressive acute renal failure, often with associated microangiopathic haemolytic anaemia and thrombocytopenia. SRC was at one time almost uniformly fatal, with death often occurring within a few weeks. With the development of angiotensin-converting-enzyme inhibitors (ACE-I), survival has improved dramatically, but death rates still remain unacceptably high. About 20% of SRC cases occur prior to making a diagnosis of SSc and, in some cases, there is no evidence of skin sclerosis at the time that SRC develops. In this report, we present a case in which a patient developed SRC prior to being diagnosed with scleroderma. Additionally, we review the pathogenesis, presenting signs and symptoms, management and prognosis of SRC.
硬皮病肾危象（SRC）是系统性硬化症（SSc）的一种危及生命的并发症，其特征为新发恶性高血压和进行性急性肾衰竭，常伴有微血管病性溶血性贫血和血小板减少。SRC曾一度几乎无一例外是致命的，死亡通常在数周内发生。随着血管紧张素转换酶抑制剂（ACE-I）的发展，生存率有了显著提高，但死亡率仍然高得令人难以接受。约20%的SRC病例在SSc诊断之前出现，并且在某些情况下，SRC发生时没有皮肤硬化的证据。在本报告中，我们介绍了一例在被诊断为硬皮病之前发生SRC的患者。此外，我们还回顾了SRC的发病机制、表现出的体征和症状、管理及预后。
