A Case of Refractory Systemic Capillary Leak Syndrome (Clarkson's Disease) during Pregnancy

A 32-year-old woman, pregnant with twins, presented with a chief complaint of general fatigue. Her general condition had rapidly deteriorated since her last visit to the primary obstetrician; the patient was then referred to our hospital because of suspected fetal death. She underwent emergency cesarean section because fetal death had indeed occurred, and she was then admitted to the intensive care unit (ICU). On ICU admission, she was found to be in shock. Laboratory analysis revealed extreme hemoconcentration and a low albumin level, and initially, septic shock with obstetric complications was suspected. However, because she did not respond to conventional therapy but instead, rapidly developed severe generalized edema, systemic capillary leak syndrome (SCLS) was diagnosed. The patient remained in shock for several days until undergoing plasma exchange (PE), despite some earlier empirical treatments. She eventually recovered from profound shock status and was discharged from the ICU without sequelae. Among potentially effective treatments, PE seemed to be the most reasonable choice for the treatment of her SCLS.