Systemic lupus erythematosus-associated autoantibodies in sickle cell disease: Spontaneous emergence in a patient and in transgenic sickle mice

We describe a patient with sickle cell disease (SCD) and elevated antiphospholipid antibodies (aPL) who developed multi-organ failure resembling catastrophic antiphospholipid syndrome. Autoimmune screening revealed several autoantibodies characteristic of systemic lupus erythematosus (SLE). Notably, routinely housed and unmanipulated transgenic sickle mice displayed significantly elevated titres of aPL- and SLE-associated autoantibodies. We hypothesize that SCD may be a risk factor not only for the development of aPL but also more widespread autoimmunity.