Libman sacks endocarditis with multivalvular involvement, hemorrhagic stroke, and concurrent mixed plasmodium malaria...

The Libman-Sacks endocarditis (LSE) is a sterile inflammatory condition of the heart valves that occurs in the setting of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Multivalvular affection is a known entity; however, it is not a common occurrence. The association between multivalvular LSE and hemorrhagic stroke is a very rare entity.

The case involves a 29-year-old female with a four-year history of SLE that had been diagnosed on the basis of the classification criteria formulated by EULAR and ACR in 2019. In addition, the patient reported fever that had been persisting over a period of ten days along with a condition of symmetrical polyarthritis that had lasted for three weeks following a pregnancy loss in the first trimester. It was documented that the patient was not compliant with immunosuppressive medications including her discontinuation of hydroxychloroquine, prednisolone, and mycophenolate mofetil. Encephalopathy followed which progressed rapidly enough to result in the development of unresponsiveness (Glasgow Coma Score 7/15). Physical examination showed that there was right hemiplegia along with bilateral extensor plantar response (Babinski sign). In terms of investigations, haemolytic anaemia was found (haemoglobin 6.0 g/dL), thrombocytopenia (platelet nadir 39 × 10³/µL), leukocytosis with neutrophilia (neutrophil count 14.2 × 10³/µL), significantly increased levels of inflammatory markers (CRP 42.2 mg/dL, ESR 120 mm/hr), anti-dsDNA level > 250 IU/mL, complement deficiency (C3 0.38 g/L; C4 0.05 g/L) and high titers of antiphospholipid antibodies (anticardiolipin IgG 72 GPL U/mL; anti-β2-glycoprotein I IgG 58 U/mL; lupus anticoagulant positive). Mixed infection caused by Plasmodium vivax and Plasmodium falciparum was confirmed through peripheral blood smear. The transthoracic echocardiography revealed presence of echogenic masses that were seen oscillating with adhesion to the posterior leaflet of mitral valve and aortic valve, associated with moderate to severe regurgitation from both valves, along with mitral stenosis, hence confirming multivalvular LSE. Cranial computed tomography without contrast revealed a large hemorrhagic infarct lesion (measuring 7 × 5.2 cm) in the left frontoparietal lobe with 15 mm midline shift. Management included administration of methylprednisolone pulses and artesunate for treating malaria, as well as blood products and osmotherapy using intravenous mannitol. Surgery was contraindicated due to presence of resistant thrombocytopenia and coagulopathy. The patient could not be managed despite maximal supportive treatment, and extremely poor prognosis was conveyed to the family.

Multiple valvular Libman-Sacks endocarditis can lead to a hemorrhagic stroke due to the combination of embolic vegetations, vasculopathy related to lupus, and coagulation problems with thrombocytopenia. This is an example of how it is necessary for all SLE patients to be screened through echocardiography and tested for antiphospholipid antibodies, adherence to the drug regimen, and a multidisciplinary team approach.