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Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology which is characterised pathologically by the presence of non-caseating granulomas. Typical presenting symptoms include cough and dyspnoea in addition to skin and/or eye lesions. We report a case of sarcoidosis presenting as upper extremity venous thrombosis.
A 39 year old woman presented with sudden swelling of the right upper limb and right side of the neck and face. She reported no cough, dyspnoea, chest pain, or systemic symptoms of fever or weight loss. She had no previous medical history, smoked 20 cigarettes daily, and was on an oral contraceptive pill (levonorgestrel, Schering Health Care Ltd). On examination her body mass index was 31. She had extensive swelling of the right upper limb and right side of the neck and face. Her upper limb pulses were normal and she had no palpable lymphadenopathy. Full blood count, coagulation screen, electrolytes, renal function indices, and serum angiotensin converting enzyme levels were normal. Liver function tests and fasting cholesterol were mildly raised. Serum calcium and 24 hour urinary calcium levels were normal. Pulmonary function testing revealed normal spirometric parameters (FEV1 2.32 litres (85% predicted), FVC 2.97 litres (93% predicted), reduced carbon monoxide transfer factor at 15.7 mmol/min/mm Hg/l (62% predicted)). A plain chest radiograph showed slight widening of the superior mediastinum, and a computed tomographic (CT) scan showed thrombosis of the right brachiocephalic vein with enlargement of the axillary and mediastinal lymph nodes (fig 1). She underwent mediastinoscopy with biopsy of lymph nodes and histopathological examination revealed fibrosis and hyalinised granulomas consistent with sarcoidosis. She was treated with low molecular weight heparin and intravenous steroids which resulted in resolution of her symptoms and clinical signs over the subsequent week. Oral anticoagulants and corticosteroids were continued as an outpatient. A thrombophilia screen performed 16 weeks after the thrombosis revealed normal protein C and S levels, normal antithrombin III levels, and testing for the lupus anticoagulant was negative.
This is the first report to our knowledge of sarcoidosis presenting as upper extremity deep vein thrombosis, although a recent case reported in Thorax described a patient with tuberculous mediastinal lymphadenopathy associated with pulmonary artery occlusion.1 In addition to venous compression by enlarged lymph nodes, possible contributing factors in our patient include the use of the oral contraceptive pill, cigarette smoking and obesity, all of which have been associated with an increased risk of venous thromboembolic disease related to venous stasis and/or altered coagulation. Upper extremity thrombosis due to venous compression from enlarged mediastinal lymph nodes is usually associated with malignant lymphadenopathy. However, the present report shows that benign mediastinal lymphadenopathy, such as that associated with sarcoidosis, also has the potential to cause venous thrombosis.
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