Lalwani Ayushi, Amin Shefali, Yousaf Zohaib, Riaz Gondal Muhammad Umer, Al Ampnti Sami, Pajak Andrew, Reggio Christopher
Department of Internal Medicine, Reading Hospital, West Reading USA.
Department of Pulmonary and Critical Care Medicine, Reading Hospital, West Reading, USA.
Eur J Case Rep Intern Med. 2024 Jul 30;11(8):004706. doi: 10.12890/2024_004706. eCollection 2024.
We present the case of a 63-year-old female diagnosed with atypical SSc in the setting of acute SRC. She was undergoing work-up for progressive dyspnoea in the outpatient setting when she was found to have newly diagnosed restrictive lung pathology and worsening renal function, thus prompting acute hospital admission. Given multisystem involvement of the pulmonary and renal systems, the differential diagnosis included autoimmune and connective tissue disorders. Although serologies were non-specific, renal biopsy confirmed scleroderma renal disease, and she was started on treatment with captopril. This case highlights the importance of clinical judgment and timely diagnosis, even when laboratory data might indicate otherwise.
Scleroderma renal crisis (SRC) remains an important cause of morbidity and mortality in systemic sclerosis (SSc), and clinicians should have a high index of suspicion to diagnose it.The absence of specific serologic markers makes SSc diagnosis challenging and necessitates reliance on clinical findings and additional diagnostic tools such as imaging studies and tissue sampling.
我们报告一例63岁女性病例，该患者在急性硬皮病肾危象（SRC）背景下被诊断为非典型系统性硬化症（SSc）。她在门诊因进行性呼吸困难接受检查时，被发现新诊断出限制性肺病变且肾功能恶化，因此被紧急收治入院。鉴于肺和肾系统存在多系统受累情况，鉴别诊断包括自身免疫性和结缔组织疾病。尽管血清学检查结果不具特异性，但肾活检确诊为硬皮病肾病，随后她开始接受卡托普利治疗。该病例强调了临床判断和及时诊断的重要性，即使实验室数据可能给出不同提示。
硬皮病肾危象（SRC）仍然是系统性硬化症（SSc）发病和死亡的重要原因，临床医生应高度怀疑以进行诊断。缺乏特异性血清学标志物使得SSc的诊断具有挑战性，需要依靠临床发现以及诸如影像学检查和组织采样等额外诊断工具。
