Clinical Report of Probable Catastrophic Antiphospholipid Syndrome in Pregnancy

Abstract

Background 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 occlusions in at least one organ or tissue. The development of CAPS in pregnancy poses many diagnostic challenges as a result of its broad range of clinical presentations and its overlap with other obstetric complications and microangiopathic diseases. Because of the high associated mortality rate, prompt recognition and treatment are paramount. Case A twenty-five-year-old G3P0111 with a history of multiple thromboembolisms presented at 21 weeks and 3 days of gestation with complaints of right upper quadrant pain, visual disturbances, headache, and syncopal episodes. Laboratory evaluation demonstrated microangiopathic disease with hemolysis (confirmed on peripheral smear), elevated liver enzymes, and abnormal 24-hour urine protein with vital signs within the normal range. Presence of significantly elevated antiphospholipid antibodies was noted, facilitating the diagnosis of probable CAPS. Proper workup was achieved based on clinical suspicion, allowing immediate and appropriate management. Conclusion CAPS is a life-threatening condition rarely seen in pregnancy making early recognition difficult. A low threshold to initiate urgent and aggressive treatment should be maintained to minimize the risk of adverse outcomes.

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“…The case presented in the study described the rapid development of liver, central nervous system, and renal lesions within a week in a patient with catastrophic anti-phospholipid syndrome (CAPS). It is worth noting that unlike HELLP syndrome, a condition that shares some similarities with CAPS, the patient's clinical condition did not show improvement following the pregnancy termination, which is consistent with observations from other cases [9]. The current approach to managing patients with CAPS involves a combination of therapies, including the administration of anticoagulants, corticosteroids, and either plasma exchange or intravenous immunoglobulin therapy [6,12].…”

“…The findings of the conducted study highlight the importance of timely identification and management of catastrophic antiphospholipid syndrome (CAPS) to effectively mitigate the risk of fatal outcomes in patients. The study provides evidence supporting the notion that prompt intervention and treatment can significantly improve patient outcomes and reduce mortality rates associated with CAPS [9,10]. Early recognition of CAPS is crucial to initiate appropriate therapeutic interventions promptly.…”

Treatment of catastrophic antiphospholipid syndrome to pregnant woman

“…Early recognition and treatment of CAPS may prevent fatal outcomes (Gоmez-Puerta et al, 2007;Hakman & Mikhael, 2018). In our patient manifestation of the disease fit into the picture of definite CAPS (Asherson's syndrome) (Asherson et al, 2003;Widana et al, 2021), because our patient met all four of the four criteria for the classification of СAPS: multifocal thrombosis with a multiple organ failure: lesions of the central nervous system, liver, kidneys, and placenta.…”

Catastrophic Antiphospholipid Syndrome: A Review

“…Fetal morbidities, such as premature birth, growth restriction, or mortality, are associated with placental insufficiency [51,52]. CAPS symptoms and laboratory findings can overlap with other obstetric disorders including acute fatty liver of pregnancy (AFLP), thrombotic thrombocytopenic purpura (TTP), or HELLP syndrome, delaying diagnosis and treatment [53][54][55]. Catastrophic antiphospholipid syndrome remains a diagnostic challenge due to its low frequency and non-specific clinical presentation.…”

“…Catastrophic antiphospholipid syndrome remains a diagnostic challenge due to its low frequency and non-specific clinical presentation. Triple therapy, which consists of anticoagulants, corticosteroids, plasmapheresis and intravenous immunoglobulins, is typically administered to pregnant patients with CAPS [52][53][54]. Pathological antibodies and other pro-inflammatory and prothrombotic mediators can be eliminated with plasmapheresis.…”