Microthrombotic renal involvement in an SLE patient with concomitant catastrophic antiphospholipid syndrome: the beneficial effect of rituximab treatment
Microthrombotic renal involvement in an SLE patient with concomitant catastrophic antiphospholipid syndrome: the beneficial effect of rituximab treatment
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伴有灾难性抗磷脂综合征的 SLE 患者的微血栓性肾脏受累：利妥昔单抗治疗的有益效果
DOI：
10.1177/0961203318768890
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发表时间：
2018
期刊：
影响因子：
2.6
通讯作者：
P. Soltész
中科院分区：
文献类型：
--
作者：
Á. Diószegi;T. Tarr;M. Nagy;M. Nanasy;R. Veisz;L. Bidiga;Balázs Dezső;József Balla;Peter Szodoray;Z. Szekanecz;P. Soltész
抗磷脂综合征的特征是多种动脉和/或静脉血栓形成事件，抗磷脂抗体（aPL）存在时反复发生胎儿丢失。灾难性抗磷脂综合征是一种危及生命的罕见的抗磷脂综合征亚型，当血栓事件影响至少三个器官，临床表现同时发生或在一周内发生。诊断上，小血管闭塞可通过组织病理学检测存在aPL。我们的病例报告描述了一名18岁的男性，自2011年以来一直治疗与系统性红斑狼疮（SLE）相关的抗磷脂综合征。临床表现以复发性深静脉血栓形成、狼疮性肾炎所致严重蛋白尿为主，伴轻度血清学及实验室检查。患者因严重血小板减少症和感染性腹泻于2014年3月住院。此时肾功能迅速恶化。同时观察左上肢轻瘫；计算机断层扫描显示大脑中动脉的局部缺血性病变。出现腹部不适和疼痛。计算机断层扫描在脾脏、右肾和腹腔干可见缺血性病变。实验室和血清学结果证实存在aPL和抗dna抗体，贫血和血小板减少症。根据上述临床和实验室结果，建立了灾难性抗磷脂综合征的诊断。开始使用抗凝、皮质类固醇和血浆交换治疗以及血液扩张性滤过。虽然在这些干预措施后血栓级联减慢，但我们没有看到肾功能的改善。开始利妥昔单抗治疗，导致肾功能显著改善。治疗5周后，患者出院。
Antiphospholipid syndrome is characterized by multiple arterial and/or venous thrombotic events, recurrent fetal losses in the presence of antiphospholipid antibodies (aPL). Catastrophic antiphospholipid syndrome is a life-threatening, rare subset of antiphospholipid syndrome when the thrombotic events affect at least three organs, and clinical manifestations develop simultaneously or within a week. Diagnostically, small vessel occlusions can be detected by histopathology in the presence of aPL. Our case report describes an 18-year-old man who has been treated for antiphospholipid syndrome associated with systemic lupus erythematosus (SLE) since 2011. The clinical findings were dominated by recurrent deep vein thrombosis, and severe proteinuria caused by lupus nephritis, accompanied by mild serological and laboratory findings. The patient was hospitalized in March 2014 because of severe thrombocytopenia and infective diarrhoea. At this time the renal functions deteriorated rapidly. Simultaneously, left upper extremity paresis was observed; computed tomography showed ischaemic lesions in the territory of the middle cerebral artery. Abdominal discomfort and pain occurred. On computed tomography scan ischaemic lesions were seen in the spleen, the right kidney and the coeliac trunk. Laboratory and serological findings verified the presence of aPL and anti-DNA antibodies, anaemia and thrombocytopenia. Based on the above-mentioned clinical and laboratory findings, the diagnosis of catastrophic antiphospholipid syndrome was established. Anticoagulation, corticosteroids and plasma exchange treatment, as well as haemodiafiltration were initiated. Although the thrombotic cascade decelerated following these interventions, we could not see an improvement in the renal function. Rituximab treatment was started, leading to a significant improvement in renal function. After 5 weeks of treatment the patient was discharged from hospital.