A 12-year-old girl was referred to the hospital for a 4-month's history of a dry cough, night sweats, persistent fever, marked weakness and weight loss of 3 kg. All these symptoms failed to respond to conventional antibiotics and inhaled corticosteroids. The patient was free from underlying diseases and had a record of Bacillus Calmette-Guerin vaccination at birth. There was no history of contact with Tb patient or family history of the vascular or rheumatic disorder.
On admission, she was afebrile and pale. Physical examination revealed impalpable peripheral pulses and inaudible blood pressure (BP) on upper limbs; BPs in right and left lower limbs were 160/90 mmHg and 140/87 mmHg, respectively. Auscultation showed heart rate at 108 beats/min with no added sound but revealed a systolic bruit along the path of the right external carotid artery. Peripheral pulses in lower limbs were full, and no bruit was detected over the abdomen or elsewhere. All other systemic examinations including pulmonary auscultation were normal.
Laboratory findings were: White blood cells at 8600/mm3, hemoglobin at 8.6 g/dl, platelet counts at 522,000/mm3, reticulocytes at 105,900 /mm3, serum ferritin at 417 mug/l. Coombs test was negative. Erythrocyte sedimentation rate (ESR) was at 140 mm/h, C-reactive protein (CRP) was at 125 mg/L; fibrinogen was 7.12 g/L; albuminemia was at 21.1 g/l and alpha2- and gamma-globulinemia were respectively at 15.2 g/l and 14 g/l. Serum electrolytes, liver and kidney function tests and urinary analysis were normal. Viral markers were negative for hepatitis B and C, human immunodeficiency virus, cytomegalovirus and Epstein-Barr virus. Antinuclear antibody, antineutrophil cytoplasmic antibodies, antiphospholipid and anticardiolipin antibodies were negative.
Doppler ultrasound and computed tomography (CT) angiography scans showed circumferential wall thickening of both common carotid arteries [Figure 1a] leading to stenosis of the right common carotid [Figure 1b] and its branches and severe stenosis of the left subclavian artery [Figure 2a and b] but excluded further vessel involvement of descending aorta, renal, or cerebral arteries. The ophthalmological examination was normal.
Investigations for Tb showed a strongly positive Mantoux test at 20 mm and positive Quantiferon-TB test. However, three specimens of sputum smear and culture for acid-fast bacilli were negative.
Chest X-ray/CT scans revealed numerous parenchymal nodes in both lungs associated to hilar lymphadenopathy suggesting active Tb [Figure 3a and b]. All these data were compatible with the diagnosis of TA according to the final EULAR/PRINTO/PRES TA criteria [Table 1] associated with active pulmonary Tb.
The patient was placed then on antitubercular therapy, with oral prednisone (0.5 mg/kg/day) and low-dose aspirin for its antithromboxane effect. Within 4 months of treatment, the patient showed clinical recovery of fever, dry cough, and night sweats. However, inflammatory activity persisted (ESR: 79 mm/h; CRP: 91mg/dL; hemoglobin: 9.9g/dl). Immediately after stopping antitubercular drugs, the prednisone dose was increased to 2 mg/kg/day for 30 days. Then, prednisone dose was progressively decreased. Acute phase reactants returned to normal values. During 4 years of follow-up, subsequent relapses were observed whenever prednisone dose decreased to under 20 mg/day without new vessel involvement or progression of the previous vascular damage. Oral methotrexate (12.5 mg/m2 /week) was added then leading to relapse spacing and reduction of corticosteroids dose to 10 mg/kg each 2 days.