Bilateral avascular necrosis of both hips and both shoulders in a case with systemic lupus erythematosus: What should we blame,steroid or secondary antiphospholipid syndrome?

A 38‐year‐old female patient was diagnosed as a case of systemic lupus erythematosus (SLE) in 1994. Her initial presentation was nephritis which remitted on combination of steroid, azathioprine and pulse cyclophosphamide therapy. One year later the patient developed bilateral avascular necrosis (AVN) both hips and underwent bilateral hip replacement. In 2003 the patient developed bilateral AVN of both shoulders. In view of this uncommon presentation the patient screened for hidden secondary antiphospholipid syndrome and surprisingly investigations revealed negative anticardiolipin antibodies, weakly positive lupus anticoagulant test and positive reactivity against β2 glycoprotein 1. Although steroid is well know for its major role in AVN in patients with SLE, the presence of hidden secondary antiphospholipid syndrome augments the deleterious effects of steroid on bone and leads to AVN in uncommon sites. It is suggested that in SLE patients with positive lupus anticoagulant and negative antiphospholipid antibodies, testing for reactivity against β2 glycoprotein 1 is mandatory.