Asfaw Yonathan Aliye, Huang Helen, Anand Ayush, Taimur Muhammad, Khan Asad Ali, Shah Sangam, Poudel Sujan, Michel Jack, Michel George
Internal medicine, Addis Hiwot Hospital, Addis Ababa, Ethiopia.
Department of Research & Academic Affairs, Larkin Community Hospital, Miami, USA.
SAGE Open Med Case Rep. 2022 Dec 5;10:2050313X221141796. doi: 10.1177/2050313X221141796. eCollection 2022.
Scleroderma renal crisis with left ventricular diastolic dysfunction can lead to significant mortality. We presented the case of a 32-year-old female with anuria for 2 days. On further inquiry, she had joint pain, difficulty turning her head sidewise, and associated difficulty in finger movement. Also, hyperpigmentation with superimposed hypopigmentation was reported, which reduced during her pregnancy and worsened post-partum. Her family history suggested similar complaints in her mother. In addition, she had a blurring of vision. She had hypertension, microangiopathic hemolytic anemia, deranged renal function, and retinopathy on ophthalmologic examination. Radiological investigations revealed pulmonary edema, pleural effusion, and left ventricular diastolic dysfunction. Hence, a diagnosis of scleroderma renal crisis complicated by left ventricular diastolic dysfunction was made. She was managed conservatively using anti-hypertensive medications and hemodialysis, which resulted in gradual improvement. Our case highlighted the management approach to this rare presentation with anti-hypertensives and hemodialysis in a resource-limited setting.
硬皮病肾危象合并左心室舒张功能障碍可导致显著的死亡率。我们报告了一例32岁女性，无尿2天。进一步询问得知，她有关节疼痛、向一侧转头困难以及手指活动相关困难。此外，有色素沉着伴色素减退，在孕期减轻，产后加重。她的家族史提示其母亲有类似症状。另外，她有视力模糊。她患有高血压、微血管病性溶血性贫血、肾功能紊乱，眼科检查发现有视网膜病变。影像学检查显示肺水肿、胸腔积液和左心室舒张功能障碍。因此，诊断为硬皮病肾危象合并左心室舒张功能障碍。对她采用抗高血压药物和血液透析进行保守治疗，病情逐渐好转。我们的病例突出了在资源有限的情况下，针对这种罕见表现采用抗高血压药物和血液透析的管理方法。
