Takayasu’s arteritis associated with antiphospholipid antibodies

Abstract

The finding of elevated level of antiphospholipid antibodies (APLAs) in a proportion of Takayasu’s arteritis (TA) patients suggests a contribution of APLAs in vascular injury at least in some of TA patients [1–3]. However, these findings have not been confirmed in other series of TA [4]. The controversy regarding the APLAs in TA is in part due to the small number of reported TA patients with APLAs assayed, and therefore we present a TA patient with increased levels of APLAs and lupus anticoagulant (LAC). In 2001 a 27-year-old woman developed Raynaud’s phenomenon, claudication of both upper extremities and visual disturbances. The complains started during the last 3 months of her the only pregnancy and she delivered a healthy child. She presented at hospital in January 2003 with absence of radial and brachial pulses in both upper extremities and vascular bruits over the right carotid and both subclavian arteries (SCA). Femoral and dorsum pedis pulses were palpable and blood pressure was 160/95 mm Hg on both legs. Color Doppler sonography: occlusion of the left common carotid artery (ACC), internal carotid artery (ACI) and external carotid artery (ACE), concentric thickening of the right ACC, ACI and ACE, stenosis of both SCA. The flow through both verterbral arteries (VA) was normal with a poststenotic spectrum type. Descending aorta and renal arteries were normal. Transcranial Doppler sonography: poststenotic spectra in both middle and posterior cerebral arteries. Angiography (Fig. 1): occlusion of ACC and left SCA, circular narrowing of the right ACC and narrowing of the right SCA to the origin of VA. Collateral circulation with retrograde flow through the left VA to the left SCA. Funduscopy: left, optic disc atrophy; right, diffuse small hemorrhages and extravasations. Erythrocyte sedimentation rate (ESR) was 85 mm/h and C-reactive protein (CRP) level was 19.1 mg/l (normal <5 mg/l). Platlets count was normal. Antinuclear antibody, antiSmith antibody, and anti dsDNA were negative. C3 and C4 levels were normal. Circulating immunocomplex level was 7.3 lg/ml (normal <5). The patient possessed B8 and DR3 HLA alleles Lupus anticoagulant and anticardiolipin antibodies (aCLa) were positive in two subsequent measurements ( IgM aCL 157 and 172 IU/ml, normal <100; IgG aCL 95 and 93 IU/ml; normal <100). Therapy included methylprednisolone (pulses 1 g i.v./ 3 days, 1 mg/kg body weight/day/4 weeks followed by 6 mg/day orally), cyclophosphamide (1 g i.v. every 4 weeks), and methotrexate 10 mg weekly. Ethylbuscomate was given for positive LAC and aCL antibodies although there was no manifest thrombosis. In the course of therapy, a moderate improvement of carotid flow and laboratory test normalization (ESR 6 mm/h, CRP 4 mg/l) occurred. One month latter, at home, the patient suddenly lost consciousness with epileptic attack. She died few hours later, most likely due to ischemic brain insult; autopsy was not performed. In our patient, the diagnosis of TA (type III according to Ishisawa) relied on her clinical data meeting all American College of Rheumatology 1990 criteria [5], J. Morovic-Vergles Department of Clinical Immunology and Rheumatology, University Department of Medicine, School of Medicine, University of Zagreb, Zagreb, Croatia