Aterini Lorenzo, Gallo Marco, Vadalà Barbara, Aterini Stefano
Nephrology, AOU (Azienda Ospedaliera Universitaria) Meyer Children Hospital, School of Human Health Sciences at University of Firenze, Firenze, ITA.
Hemodialysis Centre, Istituto Fiorentino di Cura e Assistenza (IFCA), Firenze, ITA.
Cureus. 2023 Aug 29;15(8):e44322. doi: 10.7759/cureus.44322. eCollection 2023 Aug.
This case report can be considered a rare occurrence of scleroderma renal crisis (SRC) presenting with a severe clinical course and multiple organ failure. A patient diagnosed with systemic sclerosis four years earlier was admitted to the hospital because of severe malignant systolic-diastolic arterial hypertension and acute kidney injury (AKI). Exacerbating disease suggested thrombotic microangiopathy (TMA) and the PLASMIC (Platelet count; combined hemoLysis variable; absence of Active cancer; absence of Stem-cell or solid-organ transplant; MCV; INR; Creatinine) score was used in the differential diagnosis. Despite the timely initiation of therapy with ACE inhibitors (ACE-I), the progressive renal failure required hemodialysis treatment, but renal function never recovered. Disease duration, skin involvement, and previous specific pharmacological therapy represented multiple risk factors that determined a clinical course complicated by pericardial tamponade with acute heart failure, acute pancreatitis, and ischemic stroke, with fatal evolution. These complications presented a challenging clinical sequence of events requiring an interdisciplinary course of action. Timely ascertainment of the SRC is important given the possible severe organ involvement as well as mortality. A diagnosis of scleroderma renal crisis should be considered in cases of acute kidney injury associated with known risk factors. Early treatment and collaboration between rheumatology and renal physicians can improve patient outcomes.
本病例报告可视为硬皮病肾危象（SRC）的罕见病例，其临床过程严重且伴有多器官功能衰竭。一名4年前被诊断为系统性硬化症的患者因严重的恶性收缩期 - 舒张期动脉高血压和急性肾损伤（AKI）入院。病情加重提示血栓性微血管病（TMA），并使用PLASMIC（血小板计数；合并溶血变量；无活动性癌症；无干细胞或实体器官移植；平均红细胞体积；国际标准化比值；肌酐）评分进行鉴别诊断。尽管及时开始使用血管紧张素转换酶抑制剂（ACE-I）治疗，但进行性肾衰竭仍需要血液透析治疗，且肾功能从未恢复。疾病持续时间、皮肤受累情况以及先前的特定药物治疗是导致临床过程复杂的多个危险因素，包括心包填塞伴急性心力衰竭、急性胰腺炎和缺血性中风，并最终导致死亡。这些并发症呈现出具有挑战性的临床事件序列，需要跨学科的行动方案。鉴于可能出现的严重器官受累及死亡率，及时确诊SRC很重要。对于伴有已知危险因素的急性肾损伤病例，应考虑硬皮病肾危象的诊断。早期治疗以及风湿病科和肾内科医生之间的合作可以改善患者的预后。
