Background Aortitis syndrome (Takayasu’s arteritis) associated with various autoimmune diseases have been reported.
Objectives We report the rare association of aortitis syndrome with SLE in two patients.
Methods Case report.
Results Case 1. A 49-year-old woman admitted with Raynaud’s phenomenon, right upper extremity claudication, malar rash, and oral ulcerations. Examination showed that her right radial pulse was not palpable. A systolic bruit was heard over her right carotid artery. Laboratory results were as follows: erythrocyte sedimentation rate 89 mm/h, leukopenia, thrombocytopenia, positive ANA, positive anti-ds-DNA. An artogram demonstrated a complete obstruction of the right subclavian artery near its origin. The patient was diagnosed as having SLE and TA based on ACR criteries for both diseases. She was treated with prednisolone and azathioprine. 6 months later, her right radial pulse was weakly present.
Case 2. A 30-year-old man was diagnosed in 1985 as having SLE based on malar rash, arthritis, positive ANA, positive anti-ds-DNA, hematologic abnormalities. He was treated with prednisolone (P) and azathioprine. He was readmitted in 1992 with progressive difficulty walking and symptoms of lower extremity claudication. Aortogram revealed occlusion of the abdominal aorta below the renal arteries. In 1994, he developed renal and cerebral involvement and superior vena cava syndrome (SVCS). He was again treated with P and cyclophosphamide (C). In 2000, A computed tomogram of the thorax confirmed SVCS due to mediastinal fibrosis. He has been using P and tamoxifen for 1 month.
Conclusion Our experience with these cases suggests that all patients with SLE be followed carefully for the development of occlusive arterial involvement.