Concurrent MSSA infective endocarditis in a patient with probable catastrophic antiphospholipid syndrome resulted in rapid clinical deterioration and death despite aggressive immunosuppressive therapy.
Case Report (n=1)
This case highlights the high mortality of catastrophic antiphospholipid syndrome when complicated by concurrent infective endocarditis, emphasizing the need for early recognition and multidisciplinary management.
Abstract Catastrophic antiphospholipid syndrome (CAPS) is a rare but severe thrombotic disorder with a high mortality rate that occurs infrequently in individuals with antiphospholipid syndrome (APS). A definite diagnosis requires involvement of three or more organ systems, development of symptoms within one week, biopsy confirmation of vessel occlusion, and positive APS antibodies. Probable CAPS is diagnosed when three of these criteria are met. Infections are the most common triggers, and treatment typically includes anticoagulation, corticosteroids, plasma exchange, and/or IVIG. We present an unusual case of probable CAPS presenting concurrently with infective endocarditis (IE), likely triggered by methicillin-susceptible Staphylococcus aureus (MSSA) bacteremia, in a 33-year-old female with a history of APS, cerebrovascular accident, rheumatoid arthritis, and mechanical mitral valve replacement. The patient presented with encephalopathy and vomiting following a cruise. On admission to an outside hospital, she was febrile and tachycardic with leukocytosis, thrombocytopenia, acute kidney injury, transaminitis, and coagulopathy. Initial CT imaging revealed chronic left occipital infarcts and a stable vestibular schwannoma. Broad-spectrum antibiotics were started without improvement. Further imaging showed multiple renal, splenic, and basal ganglia infarcts. Given concern for CAPS, she was started on IV methylprednisolone, IVIG, and full-dose enoxaparin, with transient improvement. Echocardiography revealed vegetations on her mechanical mitral valve, and blood cultures grew MSSA, confirming infective endocarditis. She was transferred to our facility for surgical evaluation. Soon after, she developed new left-sided weakness, and repeat brain imaging showed hemorrhagic conversion of lacunar infarcts. Enoxaparin was reversed, methylprednisolone was continued, and rituximab with plasmapheresis was initiated following multidisciplinary discussion. Despite aggressive treatment, her condition deteriorated, and MRI demonstrated cerebellar tonsillar herniation. Comfort measures were pursued, and the patient passed away. This case met criteria for probable CAPS as defined by the 10th International Congress on aPL, with involvement of three organ systems, rapid progression, and confirmed APS antibodies (lupus anticoagulant, anticardiolipin, and anti-β2-glycoprotein I). Although definite CAPS requires histopathologic confirmation, biopsy was not performed. Infection is the most frequent trigger of CAPS, and in this case, MSSA endocarditis was the likely precipitant. While Libman-Sacks endocarditis has been described in CAPS, coexistent IE is rare and complicates diagnosis and management. The overlap of thrombotic and infectious pathology created diagnostic uncertainty regarding the etiology of cerebral infarcts. This case underscores the high mortality of CAPS despite aggressive therapy and highlights the need for early recognition, prompt multidisciplinary management, and consideration of advanced therapies such as rituximab. This abstract is funded by: None
Moussa et al. (Fri,) conducted a case report in Catastrophic antiphospholipid syndrome (CAPS) with infective endocarditis (n=1). IV methylprednisolone, IVIG, enoxaparin, rituximab, and plasmapheresis was evaluated. Concurrent MSSA infective endocarditis in a patient with probable catastrophic antiphospholipid syndrome resulted in rapid clinical deterioration and death despite aggressive immunosuppressive therapy.
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