[Catastrophic antiphospholipid syndrome: diagnosis and treatment]

To analyse data on patients who developed catastrophic antiphospholipid syndrome (CAPS) in primary and secondary APS, to assess outcomes of CAPS.

We analysed retrospectively the data on 164 patients with systemic lupus erythematosus (SLE) and APS, on 76 patients with primary APS (PAPS) treated in the Institute of Rheumatology from 1989. Verification of vascular complications was made using ultrasonic dopplerography (UDG) of peripheral vessels, echocardiography of the heart, CT of the brain, abdominal organs. Anticardiolipin antibodies (ACLab) and lupus anticoagulant (LA) served as serological markers of APS.

In the observation period of 9.4 +/- 4.2 years, 33 patients (23 females and 10 males) out of 164 patients with SLE+APS developed CAPS, 8 of them survived while 25 died. CAPS patients had no differences by age, duration of the disease, its activity and symptoms from patients who had no CAPS. Ten out of 76 patients with PAPS developed CAPS, 7 of them died. The analysis of the concomitant factors which may initiate PAPS showed that in SLE and APS these factors consisted of initial menopause (n = 2), infection (n = 12), including pneumonia (n = 7), acute respiratory disease (n = 3), food poisoning (n = 1), abscess (n = 1). Cancer was in one patient, trauma after road accident in one patient. Trigger factor was not determined in 13 patients. In PAPS provoking factors were pneumonia (n = 2) and abscess (n = 1), in 7 patients these factors were not detected.

Any infection in SLE patients should be adequately treated with antibiotics; APS patients treated surgically should receive parenteral anticoagulants instead of oral ones; puerperas with APS must receive adequate parenteral anticoagulant therapy for at least 6 weeks; in exacerbation of SLE, APS patients should receive parenteral anticoagulants with following hypocoagulation with oral anticoagulants.