Department of Pediatrics, College of Medicine and Philippine General Hospital, University of the Philippines, Manila, Philippines.
Division of Pediatric Rheumatology, Department of Pediatrics, College of Medicine and Philippine, General Hospital, University of the Philippines, Manila, Philippines.
BMC Pediatr. 2022 Dec 28;22(1):737. doi: 10.1186/s12887-022-03761-w.
Juvenile Scleroderma is a rare autoimmune disease of the connective tissue. Its concurrence with COVID-19 can lead to limb ischemia as both disease entities are pro-inflammatory and pro-thrombotic. To date, there is no case report describing the symptomatology and course of disease in patients with juvenile Scleroderma and COVID-19.
An adolescent with acute limb ischemia presented with a history of generalized hypo-and-hyperpigmented skin lesions and mild, non-productive cough. She tested positive for SARS-CoV-2 on nasopharyngeal swab RT-PCR. Further work-up revealed elevated anti-phospholipid antibodies, anti-nuclear antibody, and D-dimer; low Protein S activity; and evidence of peripheral arterial disease on imaging studies. She was started on peripheral vasodilators, Methotrexate, and anticoagulation. Close monitoring of the affected limbs and other organs involved was done. Control of limb ischemia was achieved after 4 months of regular Cyclophosphamide infusion. Continued multi-disciplinary care was ensured for this patient.
There is evolving knowledge about the interplay of COVID-19 hyperinflammatory state and rheumatologic disorders. COVID-19 is thought to exacerbate cutaneous manifestations of autoimmune disorders via antigen protein mimicry and cytokine imbalance. Moreover, COVID-19 is characterized by complex hematopathologic processes that put a patient in a hypercoagulable state. Elevated D-dimer can be seen in both COVID-19 and systemic sclerosis owing to their pro-thrombotic sequela. There is scarcity of data on the association of Protein S activity with COVID-19 and systemic sclerosis. More studies need to be carried out to ultimately arrive at a consensus on thrombosis prophylaxis for patients with Scleroderma and COVID-19.
幼年型硬皮病是一种罕见的结缔组织自身免疫性疾病。它与 COVID-19 的同时发生可导致肢体缺血，因为这两种疾病实体都是炎症和血栓形成前的。迄今为止，尚无病例报告描述患有幼年型硬皮病和 COVID-19 的患者的症状和疾病过程。
一名患有急性肢体缺血的青少年有全身色素减退和色素沉着皮肤病变以及轻度、非渗出性咳嗽的病史。她的鼻咽拭子 RT-PCR 检测 SARS-CoV-2 呈阳性。进一步检查显示抗磷脂抗体、抗核抗体和 D-二聚体升高；蛋白 S 活性降低；影像学检查显示周围动脉疾病。她开始接受外周血管扩张剂、甲氨蝶呤和抗凝治疗。密切监测受影响的四肢和其他受累器官。经过 4 个月的定期环磷酰胺输注，实现了对肢体缺血的控制。为该患者提供持续的多学科护理。
关于 COVID-19 过度炎症状态和风湿性疾病之间相互作用的知识不断发展。COVID-19 被认为通过抗原蛋白模拟和细胞因子失衡加剧自身免疫性疾病的皮肤表现。此外，COVID-19 的特征是复杂的血液病理过程，使患者处于高凝状态。COVID-19 和系统性硬化症中均可看到升高的 D-二聚体，这是由于它们的血栓形成后遗症。关于 COVID-19 和系统性硬化症与蛋白 S 活性的关联，数据稀缺。需要进行更多的研究，最终就硬皮病和 COVID-19 患者的血栓预防达成共识。
