Introduction Chronic autoimmune disorder of unknown origin leads to accumulation of autoimmune complexes in joints, skin, kidneys, lungs, heart, blood vessels, and nervous system Genetic & epidemiology common in 15-25 year old African-American women multiple genes involved multiple HLAs involved HLA class II, HLA class III, HLA-DR, HLA-DQ Systemic manifestations joint involvement is most common feature (75%) pancytopenia pericarditis kidney disease is most common cause of mortality hip osteonecrosis is common in patients taking glucocorticoids Raynaud's phenomenon Presentation Symptoms fever pain in multiple joints hip pain (osteonecrosis) Physical exam butterfly malar rash large joint swelling and synovitis hand and wrist manifestation are common (90%) swelling and synovitis of PIPs, MCPs, and carpus ligamentous laxity Raynaud's phenomenon dorsal subluxation of ulna at DRUJ Imaging Studies Radiographs usually no evidence of joint destruction osteonecrosis of hips is common Labs Usually positive for ANA (95%) anti-DNA antibodies HLA-DR3 few are RF positive Treatment Nonoperative NSAIDS, methotrexate, sulfasalazine, cyclosporine, antimalarials, DMARDs treatment is similar to RA Operative digit fusion vs resection arthroplasty for hand indicated in advanced joint disease soft tissue procedures have high failure rates arthrodesis is treatment of choice for PIP or DIP deformities