Rapid response to high-dose Anakinra in refractory SLE-associated Haemophagocytic Lymphohistiocytosis: a case report

Haemophagocytic Lymphohistiocytosis (HLH) causes life-threatening hyperinflammation and has conventionally been treated with glucocorticoids plus additional immunosuppression such as ciclosporin and anakinra. We describe the case of a 47-year-old female who presented to a DGH with HLH secondary to both Systemic Lupus Erythematosus (SLE) and Covid infection. Her disease relapsed despite an initial response to first-line treatment including glucocorticoids, ciclosporin, intravenous immunoglobulins (IVIG) and conventional doses of anakinra. She subsequently responded to a higher dose of anakinra, resulting in sustained remission. This case illustrates the potentially life-saving difference between conventional and higher anakinra doses in severe HLH- an observation that is consistent with other reported cases of HLH with different triggers. We argue, therefore, for high-dose anakinra to be used first-line in refractory HLH, given the high mortality of the disease, while also carefully monitoring for infections post-interleukin-1-blockade. This adds weight to the shifting national treatment paradigm in this direction.