Vyas Ajit, Kadikoy Huseyin, Haque Waqar, Abdellatif Abdul
Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA.
JOP. 2009 Sep 4;10(5):566-9.
Antiphospholipid syndrome is often associated with systemic lupus erythematosus. Both syndromes have different clinical manifestations based on organ involvement. Antiphospholipid syndrome commonly causes spontaneous abortions, cerebral vascular occlusion, and deep venous thrombosis. Catastrophic antiphospholipid syndrome occurs when three or more organ systems are affected by thromboses in less than a week.
We report a unique case of a young woman with a history of systemic lupus erythematosus and antiphospholipid syndrome who presented with recurrent ischemic pancreatitis. Pancreatitis was refractory to anticoagulation and low dose steroids. Secondary to recurrence of pancreatitis and other organ involvement, she was treated as a presumed case of catastrophic antiphospholipid syndrome. Aggressive treatment with plasmapheresis, corticosteroids, cyclophosphamide, and anticoagulation eventually led to her recovery.
Awareness of this rare, rapidly fatal medical condition prompts vital, early intervention to improve patients' survival. This case report aims to add to the limited therapeutic data available as well as suggest a possible approach to treating this rare syndrome with very high morbidity and mortality.
抗磷脂综合征常与系统性红斑狼疮相关。这两种综合征根据器官受累情况有不同的临床表现。抗磷脂综合征通常会导致自然流产、脑血管闭塞和深静脉血栓形成。当三个或更多器官系统在不到一周的时间内受到血栓影响时，就会发生灾难性抗磷脂综合征。
我们报告了一例独特的年轻女性病例，她有系统性红斑狼疮和抗磷脂综合征病史，出现复发性缺血性胰腺炎。胰腺炎对抗凝治疗和低剂量类固醇治疗无效。由于胰腺炎复发和其他器官受累，她被当作疑似灾难性抗磷脂综合征病例进行治疗。积极采用血浆置换、皮质类固醇、环磷酰胺和抗凝治疗最终使她康复。
认识到这种罕见的、迅速致命的疾病状况促使进行至关重要的早期干预，以提高患者的生存率。本病例报告旨在补充有限的治疗数据，并提出一种可能的方法来治疗这种发病率和死亡率极高的罕见综合征。
