[Catastrophic antiphospholipid syndrome during pregnancy]

Area of Science:

• Obstetrics and Gynecology
• Rheumatology
• Hematology

Background:

• Antiphospholipid syndrome (APS) is an autoimmune disorder increasing clotting risk.
• Pregnant women with APS often receive low-molecular weight heparin for thromboembolic prevention.
• Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of APS.

Observation:

• Three pregnant women with prior APS diagnoses presented with severe complications.
• Two cases involved fetal growth retardation, leading to suspected HELLP syndrome or APS exacerbation.
• One patient developed pyelonephritis followed by HELLP syndrome symptoms and hepatic infarcts.

Findings:

• Two patients were diagnosed with catastrophic antiphospholipid syndrome (CAPS), one resulting in maternal death from pulmonary embolism.
• The second CAPS patient experienced improvement with glucocorticoids but had a poor fetal prognosis.
• The third patient also developed CAPS with hepatic infarcts, but recovered after combined immunosuppressive therapy and delivery.

Implications:

• CAPS in pregnancy presents similarly to HELLP syndrome, necessitating accurate diagnosis due to differing treatments.
• High maternal and perinatal mortality rates underscore the need for specialized management of pregnant APS patients.
• Centralized care in academic centers and multidisciplinary collaboration are vital for managing pregnant women with APS and CAPS.