A Rare Presentation of Systemic Lupus Erythematosus with Bilateral Avascular Necrosis of the Femoral Heads

Abstract This case report presents a rare manifestation of systemic lupus erythematosus (SLE) in a female patient in her late 20s. The patient initially presented with bilateral hip pain and was diagnosed with Grade II bilateral avascular necrosis (AVN) of the femoral heads. Concurrent severe autoimmune cytopenias were discovered, including autoimmune hemolytic anemia and immune thrombocytopenia. Laboratory findings revealed positive antinuclear antibody, anti-dsDNA, and other SLE-specific antibodies, along with hypocomplementemia and proteinuria. The patient met both the 2019 European Alliance of Associations for Rheumatology and the American College of Rheumatology and the 2012 Systemic Lupus International Collaborating Clinics classification criteria for SLE. Notably, this presentation occurred without prior corticosteroid use or antiphospholipid syndrome, which are typically associated with AVN in SLE patients. Treatment included high-dose intravenous methylprednisolone, oral prednisone, mycophenolate mofetil, packed red blood cell transfusions, and intravenous immunoglobulin. While cytopenias and proteinuria improved with treatment, the advanced stage of AVN necessitated bilateral total hip arthroplasty. This case highlights the potential for SLE to cause AVN through mechanisms such as vasculitis or hypercoagulable states, independent of steroid use. It also demonstrates the importance of considering SLE in patients presenting with concurrent AVN and severe autoimmune cytopenias, even in the absence of typical risk factors.