Sarcoidosis Presenting as a Case of Pulmonary Thrombotic Disease- An Unusual Presentation

Rasool Muhammad Haseeb ul, Khan Sara Z, Iqbal Pervaiz
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Abstract

Sarcoidosis is a chronic inflammatory disease involving multiple organ systems for which the inciting stimulus is unknown. It is characterized by elevated markers of inflammation and disseminated granuloma in the affected organs. Factor V Leiden deficiency has been long associated with an elevated risk of thrombosis, however, it has been observed in the patients having sarcoidosis, a secondary inciting stimulus is required to trigger the thromboembolic phenomenon, as to date only a few cases of Primary thrombosis have been reported in Factor V Leiden deficient patients culminating in an increased incidence of thromboembolic disease. We are presenting a case of a patient presenting with cerebrovascular events and pulmonary embolism, who was diagnosed later diagnosed to have sarcoidosis with an associated Protein S deficiency and factor V Leiden mutation making her predisposed to thromboembolic disease. It has been speculated that steroids as a treatment elevate the risk of thrombosis in such patients instead of the inflammatory process per se. however; this patient experienced a thrombotic episode before the start of medications. Additionally, an anatomical relationship has been reported in the literature between the site of sarcoidosis and the development of thrombosis, but the patient had repeated episodes of cerebrovascular events, despite no evidence of involvement of cranial vasculature or any heart defect, that could cause paradoxical embolization. This case report can help to elicit the relation of sarcoidosis with the increased tendency towards thromboembolic disease. CaSe RePoRt
结节病表现为肺血栓性疾病——一种不寻常的表现
结节病是一种涉及多器官系统的慢性炎症性疾病,其刺激因素未知。它的特点是炎症标志物升高和受累器官的弥散性肉芽肿。长期以来,人们一直认为赖登因子缺乏与血栓形成风险升高有关,然而,在结节病患者中观察到,需要继发性刺激才能触发血栓栓塞现象,迄今为止,只有少数赖登因子缺乏患者发生原发性血栓形成的病例报道,最终导致血栓栓塞性疾病的发生率增加。我们报告一例以脑血管事件和肺栓塞为表现的患者,她后来被诊断为结节病,伴有相关的蛋白S缺乏和因子V Leiden突变,使她易患血栓栓塞性疾病。据推测,类固醇作为一种治疗方法会增加这些患者血栓形成的风险,而不是炎症过程本身。然而;该患者在开始用药前曾发生血栓形成。此外,文献报道了结节病部位与血栓形成之间的解剖学关系,但患者反复发作脑血管事件,尽管没有证据表明颅脑血管受损伤或任何心脏缺陷,这可能导致矛盾栓塞。本病例报告可以帮助引出结节病与血栓栓塞性疾病增加趋势的关系。病例报告
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