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1例可能为灾难性抗磷脂综合征伴多器官功能衰竭,表现为抗磷脂抗体水平短暂升高

[A case of probable catastrophic antiphospholipid syndrome with multi-organ failure presenting as a transient increase of antiphospholipid antibody levels].

作者信息

Sato Rubuna, Sato Hiroshi, Nishiwaki Atsuma, Yokoe Isamu, Tsuruta Shinji, Haraoka Hitomi

机构信息

Department of General internal medicine, Itabashi Chuo Medical Center.

出版信息

Nihon Rinsho Meneki Gakkai Kaishi. 2014;37(3):183-8. doi: 10.2177/jsci.37.183.

DOI:10.2177/jsci.37.183
PMID:24974932
Abstract

A 44-year-old woman was admitted to our hospital with shock, massive pneumonia and respiratory failure, liver and renal dysfunction, and cerebral infarction. Based on these symptoms, we suspected the presence of disseminated intravascular coagulation and multiple organ dysfunctions due to massive pneumonia or catastrophic antiphospholipid syndrome (CAPS). Therefore, the patient was placed on a respirator and was administered ciprofloxacin, doripenem hydrate, thrombomodulin, antithrombin III, and methylprednisolone pulse therapy. Because the patient's antiphospholipid antibody titer was low on the day of admission (day 1), we did not include CAPS in the differential diagnosis and discontinued prednisolone treatment on day 6. However, the anticardiolipin immunoglobulin M antibody titer was found to be elevated on day 7; in addition, a transient increase in the anticardiolipin anti-β2 glycoprotein antibody titer was noted on re-examination. Moreover, on day 8, the thrombopenia and alveolar hemorrhage suddenly exacerbated. We finally diagnosed the patient with CAPS, and therefore resumed methylprednisolone therapy. Subsequently, the inflammation, respiratory failure, and thrombopenia rapidly improved, and the patient was extubated on day 12.

摘要

一名44岁女性因休克、重症肺炎及呼吸衰竭、肝肾功能不全和脑梗死入住我院。基于这些症状,我们怀疑存在因重症肺炎或灾难性抗磷脂综合征(CAPS)导致的弥散性血管内凝血和多器官功能障碍。因此,患者接受了呼吸机支持,并给予环丙沙星、多尼培南水合物、血栓调节蛋白、抗凝血酶III和甲泼尼龙冲击疗法。由于患者入院当天(第1天)抗磷脂抗体滴度较低,我们在鉴别诊断中未考虑CAPS,并在第6天停用了泼尼松龙治疗。然而,在第7天发现抗心磷脂免疫球蛋白M抗体滴度升高;此外,复查时抗心磷脂抗β2糖蛋白抗体滴度出现短暂升高。而且,在第8天,血小板减少症和肺泡出血突然加重。我们最终诊断该患者为CAPS,因此恢复了甲泼尼龙治疗。随后,炎症、呼吸衰竭和血小板减少症迅速改善,患者于第12天拔管。

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