Miesbach W, Asherson R A, Cervera R, Shoenfeld Y, Puerta J Gomez, Espinosa G, Bucciarelli S
Department of Internal Medicine III / Institute of Transfusion Medicine, Johann Wolfgang Goethe University Hospital, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany.
Division of Immunology, School of Pathology, University of the Witwatersrand and the Rosebank Clinic, Johannesburg, South Africa.
Clin Rheumatol. 2007 Dec;26(12):2109-2114. doi: 10.1007/s10067-007-0634-x. Epub 2007 May 24.
The catastrophic anti-phospholipid syndrome (CAPS) differs from the anti-phospholipid syndrome in its accelerated systemic involvement leading to multi-organic failure. In this study, the occurrence of malignancies in patients with CAPS was evaluated and the clinical findings of CAPS patients with and without malignancies were compared. We investigated the web site-based international registry of patients with CAPS for all cases in which both CAPS and underlying malignancies were present. The clinical characteristics of these cases were subsequently evaluated to establish common characteristics. The CAPS registry included information on a total of 262 cases. Twenty-three (9%) patients suffered from malignancies. In 78% of these patients, the malignancy itself or the treatment modalities instituted for the carcinoma was the precipitating factor of CAPS. Only 39% of CAPS patients with malignancies recovered in comparison to 58% of patients without malignancies (p = 0.07). Treatment modalities, however, did not differ significantly between these patients. Infections were not evident as precipitating factors for any of the malignancy patients. The mean age of patients with malignancies was 9 years older than the average age of other patients with CAPS and the prevalence of SLE was significantly less common than in patients without malignancy. Malignancy may play a pathogenic role in patients with CAPS, whereas infections are more important as triggering factors in patients without malignancies. CAPS patients with malignancies are generally older than CAPS patients without malignancies; they generally have the worst prognosis of the entire CAPS cohort.
灾难性抗磷脂综合征（CAPS）与抗磷脂综合征不同，前者会加速全身性病变，导致多器官功能衰竭。在本研究中，我们评估了CAPS患者中恶性肿瘤的发生情况，并比较了伴有和不伴有恶性肿瘤的CAPS患者的临床特征。我们在基于网站的CAPS患者国际登记处调查了所有同时存在CAPS和潜在恶性肿瘤的病例。随后对这些病例的临床特征进行评估，以确定共同特征。CAPS登记处共纳入了262例患者的信息。其中23例（9%）患者患有恶性肿瘤。在这些患者中，78%的患者其恶性肿瘤本身或针对癌症采取的治疗方式是CAPS的诱发因素。与58%无恶性肿瘤的患者相比，只有39%患有恶性肿瘤的CAPS患者康复（p = 0.07）。然而，这些患者之间的治疗方式并无显著差异。对于任何恶性肿瘤患者，感染均未表现为诱发因素。患有恶性肿瘤的患者的平均年龄比其他CAPS患者的平均年龄大9岁，且系统性红斑狼疮（SLE）的患病率明显低于无恶性肿瘤的患者。恶性肿瘤可能在CAPS患者中发挥致病作用，而感染在无恶性肿瘤的患者中作为触发因素更为重要。患有恶性肿瘤的CAPS患者通常比无恶性肿瘤的CAPS患者年龄更大；在整个CAPS队列中，他们的预后通常最差。
