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Objectives We describe a case of systemic lupus erythematosus (SLE) presenting with immune thrombotic thrombocytopaenic purpura, Kikuchi lymphadenitis, proliferative lupus nephritis and HLH, complicated by sepsis, refractory haemolysis, severe renal thrombotic microangiopathy and hyperinflammatory cardiomyopathy. We report the effective combination of anakinra, rituximab, caplicazumab and eculizumab, with IV cyclophosphamide, high dose glucocorticoids and plasma exchange, resulting in full recovery of critical end organ dysfunction.Methods A 24-year-old from West Africa was admitted with high grade pyrexia, lymphadenopathy, thrombocytopaenia, active urinary sediment with nephrotic range proteinuria. There was a prodromal illness of arthralgia, anaemia and blood transfusion in the country of origin. Investigations support diagnosis of TTP and HLH (ferritin >52 000; H-Score 161). Serology was compatible with SLE: ANA, anti-dsDNA and chromatin antibody positive with deep hypocomplementaemia. Anti-phospholipid profile was negative. There was a concurrent MRSA bacteraemia.Results Initial management comprised broad spectrum antibiotics, therapeutic plasma exchange, IV caplacizumab, IVIG, IV anakinra and introduction of IV methylprednisolone after lymph node biopsy. Fever remits rapidly on anakinra and ferritin falls. ADAMTS13 activity normalises and despite TTP remission fulminant intravascular haemolysis on a background G6PD and sickle trait emerges on day 9, with gross haemoglobinuria, jaundice, stage 3 AKI, metabolic acidosis and methaemaglobinaemia. Renal biopsy demonstrates severe thrombotic microangiopathy with grossly oedematous glomeruli and surrounding class IV lupus nephritis. Further gram-negative bacteraemia precipitates cardiogenic shock. MRI confirms severe global LV systolic dysfunction with grossly oedematous myocardium. High dose vasopressors and hemodiafiltration are initiated.Addition of eculizumumab following further plasma exchange rapidly attenuates the haemolytic process. IV cyclophosphamide is added to rituximab, broad spectrum antimicrobials, ongoing IV anakinra and reducing doses of methylprednisolone. Care is stepped down from ICU on Day 27 with progressive recovery of renal function and LV systolic function, falling ferritin and stabilized full blood count.Conclusions This case demonstrates that in fulminant multisystem SLE complicated by HLH, personalized immunomodulation with combination targeted biologics—including eculizumab—may achieve full end-organ recovery despite life-threatening immune dysregulation.