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

Abstract

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.

Keywords: Townes model; antiphospholipid antibody; antiphospholipid syndrome; autoimmunity; sickle cell disease; systemic lupus erythematosus.

FIGURE 1

Renal biopsy. (A–D) Light microscopic images (original magnification 40×). (A–C) H&E, PAS and Jones' silver stained sections, respectively, show glomeruli with a slight increase in the urinary space and segmental wrinkling of the capillary loops. (D) H&E‐stained section shows tubular luminal bile casts and bile pigment in the tubular epithelial cell cytoplasm (D, black asterisk). (E) Fibrinogen stain (original magnification 400×) shows glomerular and numerous peritubular capillary fibrin thrombi. (F, G). Electron microscopic images show segmental subendothelial electron lucent widening (F, red asterisk) and early basement membrane reduplication (G, red asterisk).