Catastrophic antiphospholipid syndrome in pregnancy, a diagnosis that should not be missed

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is an accelerated form of the antiphospholipid antibody syndrome resulting in multi-organ ischemia and failure. It is a rare and life-threatening condition that can be easily mistaken with hemolysis elevated liver enzymes low platelets syndrome, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. In order to make a diagnosis, it is required to have multi-organ thrombosis over 1 week affecting at least three organs or systems, and to have positive antiphospholipid antibody on two occasions (6 weeks apart), and histopathologic confirmation of small vessel occlusion. However, due to similarities in clinical and laboratory findings between CAPS and some other obstetric complications, potential misdiagnosis or delay in diagnosis are common, increasing the risk of adverse maternal and perinatal outcomes. In this review we summarized information presented in previous studies, focusing on CAPS related to pregnancy. We reviewed diagnostic criteria, differential diagnosis, and common presentation ranging from malaise, abdominal pain, dyspnea, hypertension, to altered mental status and seizures. We also discussed management in pregnancy and included a detailed algorithm with steps to take. Of note, the most significant reduction in mortality was seen in patients receiving triple therapy which will be discussed in this review.

Eculizumab in a pregnant patient with laboratory onset of catastrophic antiphospholipid syndrome

“…[ 9 – 11 ] Patients with APS and triple APL positivity with suspicious laboratory findings represent a challenge for the clinician, as deterioration can occur abruptly when CAPS develops. [ 1 , 3 ] Here, we show that eculizumab administered before multiorgan thrombosis was safe and effective in a patient at a high risk of CAPS with features of TMA, allowing safe delivery and uneventful puerperium.…”

“…[ 1 ] Catastrophic antiphospholipid syndrome (CAPS) is a severe acute complication characterized by multiple organ damage and failure due to widespread thrombotic microvascular angiopathy (TMA) associated with high mortality in pregnant patients (up to one-third of patients). [ 2 , 3 ] Initiating and/or precipitating factors in pregnant patients include infection, surgery, bleeding, delivery, and puerperium. Early diagnosis and aggressive therapy are essential.…”

“…Early intervention before microvascular thrombosis and organ failure would be a fundamental goal in pregnant patients, particularly so because the delivery itself represents a further hit, capable of precipitating the clinical situation and associated with adverse outcomes, and the risk of catastrophic episodes is markedly higher during the postpartum period. [ 3 ]…”

Catastrophic antiphospholipid syndrome in pregnancy: a life-threatening condition

“…CAPS is rare and develops in about 1% of cases of APS 4. CAPS is defined as multiorgan thrombosis, affecting a minimum of three different organs, requiring histopathological confirmation of small vessel occlusion in at least one organ or tissue and presence of antiphospholipid antibodies on two separate occasions, 6 weeks apart 4 5…”

Clinical Report of Probable Catastrophic Antiphospholipid Syndrome in Pregnancy

“…APS is associated with the development of arterial and venous thrombi, most commonly manifested as deep venous thrombosis and pulmonary embolism [ 1 ]. Catastrophic APS (CAPS) is a rare but life-threatening form of APS defined as multiorgan thrombosis, affecting a minimum of three organs with confirmation by histopathology of small vessel occlusion in at least one organ or tissue [ 2 ] and presence of antiphospholipid (aPL) antibodies on two separate occasions, six weeks apart [ 2 , 3 ]. CAPS rapidly develops and has a reported mortality rate as high as 50% making its prompt diagnosis critical [ 4 ].…”