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Catastrophic antiphospholipid syndrome (CAPS) is a rare and potentially life-threatening variant of the antiphospholipid syndrome (APS) which is characterized by multiple small vessel thrombosis which can lead to multiorgan failure. CAPS develops in about 1% of all APS cases. Pregnancy is one of the significant triggers of this disease. CAPS is characterized by an extremely unfavorable prognosis and only early diagnosis and treatment may improve maternal and fetal outcome.
The study included 14 women between 19 to 39 years old (27,92±5,83 years) with obstetric CAPS. Prior to the development of acute obstetric situation, all 14 patients (pts) were healthy. Considering the presence of thrombotic microangiopathy (TMA) syndrome in all patients, plasma therapy in transfusion and/or plasma exchange modes was used as first-line therapy, 10 out of 14 (71.4%) pts received LMWH therapy. 2 patients were initially mistakenly diagnosed with aHUS and received complement-blocking treatment with eculizumab; one of them subsequently underwent a genetic study of the complement system, which did not reveal pathogenic mutations associated with aHUS.
In 5 of 14 (35.7%) patients this was the first pregnancy. 9 (64.3%) pts had a history of reproductive losses and other obstetric complications (4 (28.6%) - miscarriage, 3 (21%) - antenatal fetal death, 2 (7%) – PE). In 9 (64.3%) pts the disease manifested itself during pregnancy (3 in the 2nd trimester, 6 in the 3rd trimester), in 5 pts (36.7%) after childbirth. In all cases, after childbirth there was a deterioration in the condition and progression of TMA. The triggers for CAPS were various complications of pregnancy and childbirth (Figure 1).
All pts had anemia (Hb 75.50 [59.0; 85.0] g/l), signs of hemolysis (increased LDH 661.50 [380.0; 1634.0] U/g, decreased haptoglobin, schizocytosis), thrombocytopenia (58.50 [34.0; 142.0]x109/l). The majority (64.3%) had hypertension. Kidney damage in 11 of 14 (78.6%) pts was represented by AKI (anuria, hypercreatininemia 230.0 [105.0; 329.0] mmol/l) with the need for dialysis in 6 (54.5%) of them. In the remaining 3 cases, creatinine values were normal, but proteinuria more than 0.5 g/l and hypertension were observed. Multiple organ failure was diagnosed in all patients. (Figure 2).Mortality was 28.6% (4 out of 14 pts). Among the surviving 10 (71.4%) pts, two were prescribed eculizumab with a positive effect in both cases, regardless of the time of initiation of therapy (on the 7th and 39th day from the onset of the disease). In all surviving pts renal function was completely restored.
1. A complicated obstetric history in pts with TMA syndrome requires, first of all, the exclusion of CAPS
2. In obstetric CAPS, kidney damage may be minimal, without AKI
3. Multiple triggers can activate complement in CAPS, which allows discussion of the effectiveness of eculizumab.