Hughes syndrome with cerebral, skeletal infarction & retinal vein thrombosis

Isolated osteonecrosis revealing primary antiphospholipid syndrome

“…14 These bone infarctions are significantly more frequent during secondary APS associated with systemic lupus erythematosus; Systemic corticosteroid therapy, often prescribed at high doses during lupus, plays a role in the pathogenesis of these infarcts. [15][16][17] Bone infarction in primary APS can be isolated, but most often is associated with other thromboembolic manifestations of the disease (cutaneous, cerebral, intestinal, hepatic, ocular, cardiac, renal, etc.). 16,18 In the most severe forms of the disease, osteonecrosis fits into a clinical picture of multiorgan damage defining the catastrophic syndrome of antiphospholipids.…”

“…[15][16][17] Bone infarction in primary APS can be isolated, but most often is associated with other thromboembolic manifestations of the disease (cutaneous, cerebral, intestinal, hepatic, ocular, cardiac, renal, etc.). 16,18 In the most severe forms of the disease, osteonecrosis fits into a clinical picture of multiorgan damage defining the catastrophic syndrome of antiphospholipids. 18 Bone infarction associated with primary APS can be uni-, bilateral, or multiple (involving three or more bone anatomic sites).…”

“…18 Bone infarction associated with primary APS can be uni-, bilateral, or multiple (involving three or more bone anatomic sites). 4,7,8,12,17,19 Several sites of avascular osteonecrosis have been reported as sporadic cases during this syndrome: 4 vertebrae, 16 flat bones of the feet and wrists, 15 femurs, 7,8,15,17 tibia, 8,17 humerus, 7 and ribs. 20 The revealing forms of the disease remain exceptional.…”