Sustained remission with eculizumab in refractory lupus nephritis with atypical haemolytic uraemic syndrome

Lupus nephritis (LN) is a severe manifestation of systemic lupus erythematosus with approximately 50% of patients having renal involvement. Patients who fail to respond to appropriate induction immunosuppression are considered to have refractory disease. Refractory LN is associated with increased risk of progression to end-stage chronic kidney disease and mortality. Despite the development of modern therapeutics, refractory LN remains challenging in clinical practice and continues to be associated with treatment failure. Complement has a well-described role in the pathogenesis of LN; however, complement inhibition is not routinely used for refractory disease. We present the case of a woman in her 30s with refractory LN who presented with atypical haemolytic uraemic syndrome, macrophage activation syndrome and low-severity COVID-19 infection. She was treated with eculizumab with rapid clinical and biochemical improvement. The patient achieved complete remission of her refractory LN after eculizumab and remains in remission 2 years post eculizumab.