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一名患有系统性红斑狼疮和抗磷脂综合征的患者,尽管接受了适当的抗凝治疗,但仍出现多灶性缺血性骨坏死。

Multifocal avascular osteonecrosis despite appropriate anticoagulation therapy in a patient with systemic lupus erythematosus and antiphospholipid syndrome.

作者信息

Cecchi Irene, Pérez Sánchez Laura, Sciascia Savino, Roccatello Dario

机构信息

Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, University of Turin and S. Giovanni Bosco Hospital, Turin, Italy.

School of Specialization of Clinical Pathology, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.

出版信息

BMJ Case Rep. 2018 Nov 1;2018:bcr-2018-225532. doi: 10.1136/bcr-2018-225532.

Abstract

Multifocal avascular osteonecrosis (AON) is a serious manifestation of systemic lupus erythematosus (SLE). Prothrombotic factors, especially antiphospholipid antibodies (aPL), have been associated with the development of AON; therefore, attenuating the procoagulant state while balancing the haemorrhagic risks might have a rationale when managing this condition. We report a case of a 37-year-old patient with SLE, treated with low doses of corticosteroids and immunosuppressive therapy, who was started on vitamin K antagonist following an episode of deep vein thrombosis while having persistent positivity for aPL. After 2 years, he presented with multifocal AON, involving both femurs and shoulders. The patient underwent a bilateral hip replacement, but despite appropriate anticoagulation therapy after 2 years, he developed another episode of AON at both distal epiphyses of the femurs and proximal epiphyses of the tibias. Multifocal AON should be suspected, especially in the presence of aPL positivity. Its aetiology is still unknown and is most likely multifactorial. Its management is challenging and requires combined approaches.

摘要

多灶性无菌性骨坏死(AON)是系统性红斑狼疮(SLE)的一种严重表现。血栓形成前因素,尤其是抗磷脂抗体(aPL),与AON的发生有关;因此,在治疗这种疾病时,在平衡出血风险的同时减轻促凝状态可能有其合理性。我们报告一例37岁的SLE患者,接受低剂量皮质类固醇和免疫抑制治疗,在发生深静脉血栓形成且aPL持续阳性后开始使用维生素K拮抗剂治疗。2年后,他出现了多灶性AON,累及双侧股骨和肩部。该患者接受了双侧髋关节置换术,但尽管在2年后进行了适当的抗凝治疗,他在双侧股骨远端骨骺和胫骨近端骨骺又出现了一次AON发作。应怀疑多灶性AON,尤其是在aPL阳性的情况下。其病因仍不清楚,很可能是多因素的。其治疗具有挑战性,需要综合方法。

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Current concepts on osteonecrosis of the femoral head.当前关于股骨头坏死的概念
World J Orthop. 2015 Sep 18;6(8):590-601. doi: 10.5312/wjo.v6.i8.590.
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GAPSS: the Global Anti-Phospholipid Syndrome Score.GAPSS:全球抗磷脂综合征评分。
Rheumatology (Oxford). 2013 Aug;52(8):1397-403. doi: 10.1093/rheumatology/kes388. Epub 2013 Jan 12.
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Pathogenesis of antiphospholipid syndrome: understanding the antibodies.抗磷脂综合征的发病机制:认识抗体。
Nat Rev Rheumatol. 2011 Jun;7(6):330-9. doi: 10.1038/nrrheum.2011.52. Epub 2011 May 10.
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Osteonecrosis in patients with SLE.系统性红斑狼疮患者的骨坏死
Clin Rev Allergy Immunol. 2003 Aug;25(1):13-24. doi: 10.1385/CRIAI:25:1:13.

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