T. Fehr et al., Foetal loss, liver necrosis and acute lupus erythematosus in a patient with antiphospholipid antibody syndrome, LUPUS, 10(8), 2001, pp. 576-579
The antiphospholipid antibody syndrome (APS) is characterized by arterial o
r venous thromboses and recurrent foetal loss. It occurs as primary disease
, but also in the context of systemic lupus erythematosus (SLE). Whereas pr
imary APS induces a thrombotic microangiopathy without significant inflamma
tory reaction, secondary APS in SLE is usually associated with vasculitis.
Here we report a patient with APS who presented with acute diarrhoea and th
en developed a HELLP-like syndrome characterized by a spontaneous abortion,
multifocal hepatic necroses and thrombocytopenia. Thereafter an acute flar
e of SLE with arthralgias, pleuritis, skin rash and glomerulitis occurred.
Clinical amelioration was only achieved by combining curettage, anticoagula
tion and immunosuppression, a treatment taking into account the pathogenesi
s of HELLP-like disease, APS and SLE. To our knowledge this is the first re
ported case of APS associated with combined acute manifestations of these t
hree syndromes triggered by a presumable intestinal infection.