Small vessel thrombosis without major thrombotic events in systemic lupus erythematosus patients with antiphospholipid syndrome.
Antiphospholipid syndrome has been defined by the presence of antiphospholipid antibodies or lupus anticoagulant in association with certain clinical events, including recurrent arterial or venous thromboses and recurrent fetal loss. It comprises two separate clinical entities: simple, characterized by large vessel occlusions, and catastrophic, with multiple occlusive events predominantly affecting small vessels. Three patients with systemic lupus erythematosus and permanently increased IgG anticardiolipin antibody levels are being described. Only postmortem histopathological examination revealed microangiopathic thrombotic changes in different organs, which were clinically silent in early stages of the disease and misinterpreted later in its course because of a peculiar clinical picture. All patients presented features of catastrophic antiphospholipid syndrome in the final stage of the disease.
Sipek-Dolnicar
A
A
Department of Rheumatology, University Medical Center, Ljubljana, Slovenia.
Hojnik
M
M
Rozman
B
B
Vizjak
A
A
Ferluga
D
D
D000293:Adolescent; D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000097:blood*; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000175:diagnosis; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis; D013927:Thrombosis / Q000276:immunology*
D017153:Antibodies, Anticardiolipin; D007074:Immunoglobulin G