Subclavian artery thrombosis mimicking the aortic arch syndrome in systemic lupus erythematosus

Abstract

Lesions of the aorta and its main branches have rarely been described in systemic lupus erythematosus (SLE) (1,2). Arteriolar and postcapillary venular lesions predominate. The vasculitis of both large and small caliber vessels is thought to be caused by endothelial cell injury secondary to immune complex deposition ( 3 ) . The following patient with undiagnosed active SLE had left hand digital ischemia, and her left arm arterial pulses were absent due to thrombosis of the subclavian and brachial arteries.Case report. A 1Pyear-old black woman was admitted to Emory University Hospital. She complained of bilateral angioedema of the hands and digital cyanosis on the left. Three days before admission she developed facial edema and was given Benadryl by her physician, without relief. On the evening of admission she developed numbness and cyanosis of her left hand. She had a history of alopecia, anemia, and a documented weight loss of 20 pounds over 3 months.Physical examination revealed a lethargic young woman with stable vital signs, severe left hand pain, bilateral periorbital edema, chemotic conjuctivae, and malar hyperpigmentation. Multiple white plaques (0.5-1 .O cm in diameter) covered the tongue, oral mucous membranes, and mucosa of the external nares. Enlarged, bilateral, movable, nontender lymph nodes were present in the supraclavicular, axillary, and posterior cervical areas. Pulmonary auscultation indicated the presence of bilateral pleural effusions.Findings from a cardiovascular examination were normal, except for absent left brachial, radial, and ulnar pulses. All other arterial pulses were strongly palpable. The abdomen was distended, with hypoactive bowel sounds and shifting dullness, but there was no evidence of hepatosplenomegaly. The results of pelvic, rectal, and neurologic examinations were normal. Her admission white blood cell (WBC) count was 17,500/mm3 with a monocytosis (12%). She had a Westergren erythrocyte sedimentation rate (ESR) of 87 mm/hour, and an albumin of 2.4 gm/dl. The chest roentgenogram demonstrated bilateral pleural effusions and bibasilar linear atelectasis with interstitial infiltrates present in both lower lobes. A kidneyureter-bladder roentgenogram of the abdomen revealed ascites. The results of the electrocardiogram, urinalysis, thyroid function studies, and all chemical, hematologic, and clotting studies (including prothrombin and partial thromboplastin times) were normal.Thoracentesis and abdominal paracentesis were nondiagnostic. Cultures and cytologic examinations of both body fluids, as well as the pleural biopsy, were negative. All cultures were sterile except those from the oral and nasal plaques, which grew Candida albicans. The patient was treated with oral and topical ately distal to the juxtaposition of the subclavian artery over the first rib (Figure 1). There was no evidence of occlusion or narrowing proximally. Oblique views of the aortic arch including the left upper arm showed abrupt occlusion of the brachial artery in its distal third with arterial collater...

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