Echoes of a Cytokine Storm: HLH-Associated Bi-Ventricular Failure in a Young Woman with Lupus – A Case Report.
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Keywords
Haemophagocytic lymphohistiocytosis, cardiomyopathy, systemic lupus erythematosus, cytokine storm, echocardiography, acute heart failure, tricuspid regurgitation
Abstract
Haemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome that can lead to rapid multiorgan failure. Cardiovascular involvement is under-recognised but may manifest as acute, reversible cardiomyopathy, particularly in patients with autoimmune disease. Early identification of cardiac complications is essential to guide timely immunosuppression and supportive therapy.
A 33-year-old woman with systemic lupus erythematosus (SLE) was transferred for specialist management of HLH. After initial stabilisation, she developed chest pain and haemodynamic compromise. Transthoracic echocardiography revealed severe bi-ventricular failure (EF 18%) and torrential tricuspid regurgitation. She was readmitted to ICU and managed with levosimendan, glyceryl trinitrate, and milrinone. Immunosuppression was escalated with IV methylprednisolone, IVIG, anakinra, etoposide, and rituximab.
Cardiac function improved rapidly, guided by serial echocardiography and biomarker monitoring. She was discharged with normalised EF and good functional recovery on tapering steroids and mycophenolate.
This case demonstrates that HLH can cause severe but reversible cardiomyopathy, particularly in patients with autoimmune disease. Early echocardiographic assessment and prompt initiation of immunosuppression alongside cardiovascular support enabled full recovery of cardiac function. Timely, multidisciplinary management is essential to prevent irreversible multiorgan dysfunction in HLH.
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