Session Type: Abstract Submissions (ACR)
Background/Purpose: To analyze the clinical and laboratory features as well as the precipitating factors, treatment and outcome of patients with catastrophic antiphospholipid syndrome (CAPS).
Methods: We analyzed the clinical and serologic features of the patients included on May 31, 2013, in the “CAPS Registry”, a web-site based international registry of patients with this condition. Categorical variables are presented as frequencies and continuous variables are presented as mean ± standard deviation. Comparative analysis was performed with chi-square test for categorical variables and t-test for quantitative variables using non-parametric tests when parametric tests were not applicable.
The entire series includes 441 patients (456 episodes). Thirteen (2.9%) patients recurred twice and 2 recurred three times. Three hundred (68%) patients were female and 136 (30.8%) were male. They had a mean age of 38.4 ± 17.0 years (range, 0-85). Male patients were older than female (43.4 years vs. 36.2 years; p<0.001). The main underlying conditions were primary APS (59.4%), systemic lupus erythematosus (SLE) (30.4%), and lupus-like disease (3.4%). The catastrophic episode was the first manifestation of the APS in 223 (50.6 %) patients. A precipitating factor was reported in 286 (62.7%) episodes, including infections (30.3%), malignancy (10.7%), surgery (10.5), oral contraceptive pills (7.2%), drugs (4.4%), cesarean section (4.2%), lupus flare (2.0%) and trauma (1.1%).
A variety of thrombotic manifestations involving the majority of organs were recorded, including renal (73.6%), lung (58.4%), cerebral (56.3%), heart (49.3%), hepatic (36.3%), gastrointestinal (24%), splenic (16.3%), adrenal (10.4%), and pancreatic (7.1%) manifestations.
Patients with APS associated with SLE had more episodes of livedo reticularis (29.1% vs. 15% p=0.001), heart valve lesions (22.1% vs. 10.4%; p=0.01), seizures (12.8% vs. 6.9%; p=0.042), pancreatitis (13.4% vs. 4.4%; p=0.001) and more frequently exhibited thrombocytopenia (72.4% vs. 62.5%; p=0.05). No differences in clinical manifestations were found between male and female patients.
Regarding immunologic features, IgG anticardiolipin antibodies (aCL) were positive in 81.6% of the patients, lupus anticoagulant in 81.3%, IgM aCL in 48.6%, IgM anti-β2GPI in 3.2% and IgG anti-β2GPI in 11.1%. Female patients had a higher frequency of hemolysis and schistocytes. One hundred and seventy-five (38.6%) patients died at the time of the CAPS event.
Conclusion: The CAPS is an uncommon but potentially life-threatening condition that needs a high clinical awareness. CAPS may affect any organ of the body and display a broad spectrum of manifestations.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/catastrophic-antiphospholipid-syndrome-caps-clinical-immunologic-features-and-outcome-of-441-patients-from-the-caps-registry/