A 24-year-old woman suffered from blurred vision and periorbital edema with remittent fever. She was diagnosed as having systemic lupus erythematosus (SLE), complicated with myopia and retinopathy and severe chemosis. Antiphospholipid syndrome (APS), hemophagocytic syndrome, and liver involvement were also proven. We considered that APS might cause chemosis as a result of thrombosis-induced perfusion failure in the conjunctiva. In such cases, APS should be considered and anticoagulation therapy associated with steroid therapy should be initiated. In systemic lupus erythematosus (SLE), chemosis, severe hepatitis, and hemophagocytic syndrome (HPS) are rare complications. It is well known that many cases of SLE are complicated with antiphospholipid syndrome (APS), which causes arteriovenous thrombosis. We report a case of SLE with transient myopia and severe chemosis complicated with severe hepatitis and HPS. As this patient had antiphospholipid antibodies, these ocular complications were considered to be related to APS.

A 56-year-old man presented with hyperproteinemia and renal dysfunction associated with antineutrophil cytoplasmic antibodies (ANCA). He had had a ventriculoperitoneal shunt tube made of silicone implanted 4 years earlier. In his renal biopsy, necrotizing crescentic glomerulonephritis was identified: tests for both myeloperoxidase ANCA and proteinase 3 ANCA were initially weakly positive. Antinuclear and other autoantibodies were also present. We diagnosed ANCA-associated vasculitis, probably induced by the silicone tube.