Reversible Segmental Stenotic Lesion of Subclavian Artery in an 8-year-old Girl: An Atypical Takayasu's Arteritis? H. Tanaka1 and S. Waga2. 1Hirosaki ...
Letter Reversible Segmental Stenotic Lesion of Subclavian Artery in an 8-year-old Girl: An Atypical Takayasu’s Arteritis? H. Tanaka1 and S. Waga2 1
Hirosaki University School of Medicine, Hirosaki; 2National Sanatorium Iwaki Hospital, Namioka, Japan
Takayasu’s arteritis (TA) is a segmental inflammatory arteritis leading to stenosis and aneurysms of large- and medium-sized arteries [1–3]. Its clinical course is usually chronic and progressive. We report a Japanese girl aged 8 years who presented with a 3month history of a non-specific inflammatory condition. Although she had not complained of pulselessness, magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) revealed a segmental stenotic lesion of the left subclavian artery. Follow-up DSA performed after 6-months of prednisolone treatment revealed complete resolution of the lesion. Her clinical course was not that of typical TA [2–5]. Since June 2000, the patient, a Japanese girl aged 8, had developed a remitting fever. She had been sent to a regional hospital with a diagnosis of fever of unknown origin. On admission, physical examination revealed a well developed, normotensive girl. Laboratory tests showed non-specific inflammatory reactions (i.e. serum C-reactive protein (CRP) 14.1 mg/dl, and erythrocyte sedimentation rate (ESR) 85 mm/h). Serum antinuclear antibody (ANA) was absent and hypocomplementaemia was not seen. No abnormal findings were observed in the bone marrow aspiration. Blood cultures were negative. A routine gallium-67 citrate scintigram of the whole body showed no abnormal accumulation. Although her remitting fever and serum CRP elevation had seemed to subside after intravenous antibiotics, the fever recurred. Because she was suspected of having a systemic rheumatic disease, several non-steroidal anti-inflammatory drugs were started but did not prove effective. On 26 August, she suddenly developed a generalised tonic seizure with high fever. A CT scan of her head, lumbar puncture and electroencephalography did not show any abnormalities. Prednisolone, 1.5 mg/kg daily, was started from 2 September, and proved effective for the remittent fever. On 5 September, she was referred to us for further examination. On admission, she was not in a serious condition. Her blood pressure was 112/54 mmHg. Peripheral pulse was normal. No abnormal cardiac and vascular murmurs were found. No skin or mucosal lesions were observed. There was no evidence of arthritis or Raynaud’s phenomenon. No abnormal findings were observed in the chest radiograph. Laboratory studies revealed the following: ESR 48 mm/h; white blood cells 7200/ml with a normal haemogram; haemoglobin 11.4 g/dl; haematocrit 35.3%; platelets 406 000/ml;
serum total protein 7.8 g/dl; albumin 3.8 g/dl; aspartate aminotransferase 32 U/l; alanine aminotransferase 31 U/l; urea nitrogen 12 mg/dl; creatinine 0.3 mg/dl; sodium 142 mEq/l; potassium 4.5 mEq/ l; chloride 104 mEq/l; and CRP 1.1 mg/dl (normal range
