Supraclavicular pulsatile mass as presenting sign of brachiocephalic trunk aneurysm in the long-term followup of giant cell arteritis
Article first published online: 2 JUN 2005
Copyright © 2005 by the American College of Rheumatology
Arthritis Care & Research
Volume 53, Issue 3, pages 475–476, 15 June 2005
How to Cite
Garcia-Porrua, C., Pego-Reigosa, R., Piñeiro, A., Armesto, V. and González-Gay, M. A. (2005), Supraclavicular pulsatile mass as presenting sign of brachiocephalic trunk aneurysm in the long-term followup of giant cell arteritis. Arthritis & Rheumatism, 53: 475–476. doi: 10.1002/art.21163
- Issue published online: 2 JUN 2005
- Article first published online: 2 JUN 2005
To the Editor:
Aortic aneurysm, aortic dissection, and large-artery stenosis of the arm and leg arteries or cervical arteries have been described in giant cell arteritis (GCA) (1–7). The identification of vascular complications is of main importance, because rupture of aneurysms has been reported to be a cause of death in these patients (4, 7, 8). We report a patient with GCA who had a supraclavicular pulsatile mass as the presenting manifestation of a brachiocephalic trunk aneurysm, observed several years after the diagnosis of GCA.
In 1988, the patient, a 68-year-old woman, was admitted to our hospital because of a 2-month history of a constitutional syndrome (asthenia, anorexia, and a 4-kg weight loss), headache, jaw claudication, and polymyalgia rheumatica (PMR) symptoms involving the neck, shoulder, and hip girdles. Her past clinical history was unremarkable. On examination, tenderness to palpation and decreased pulsation with nodules in her right temporal artery were observed. Pain on movement of the shoulders and hips was also observed. Routine laboratory analyses showed a hemoglobin level of 12.4 gm/dl, a platelet count of 676,000/mm3, and an increased erythrocyte sedimentation rate (ESR) of 115 mm/hour (normal <20 mm/hour). Other laboratory data, including coagulation tests, anticardiolipin antibodies, and hepatic and renal function parameters, were negative or normal. Plain chest radiography did not show abnormalities. A biopsy of the right temporal artery performed 24 hours after admission showed interruption of the internal elastic laminae with infiltration of mononuclear cells and the presence of giant cells into the arterial wall.
Corticosteroid therapy (40 mg of prednisone/day) was started, with rapid improvement of symptoms after 48 hours of treatment. The dosage of prednisone was progressively tapered, up to complete discontinuation 15 months later. However, 5 years later the patient experienced a relapse of the disease, manifested by headache, jaw claudication, PMR symptoms, and elevation of the ESR (80 mm/hour). Steroid therapy was restarted (40 mg/day) for 3 weeks. Following this procedure, rapid improvement of symptoms and normalization of the ESR were again achieved. The prednisone dosage was progressively reduced, to 2.5 mg/day, during the following 18 months. At that time a new relapse characterized by asthenia, PMR symptoms, and an increase in the ESR (45 mm/hour) was observed. Because of this relapse, combined therapy with methotrexate was considered, but the patient refused it. The dosage of prednisone was increased (up to 20 mg/day) because no headache or ischemic manifestations were observed. Afterwards, prednisone therapy was slowly tapered down, with complete discontinuation 15 months later.
Twelve years after the diagnosis of GCA (in 2000), during a routine visit at the outpatient rheumatology clinic, a right supraclavicular pulsatile mass was detected. At that time, the patient was free of symptoms, and both the ESR and the C-reactive protein levels were within normal range. A magnetic resonance angiography disclosed the presence of an aneurysm in the brachiocephalic trunk (Figure 1). Antiaggregation treatment with aspirin (100 mg/day) was commenced. No further complications have occurred since then.
Large-artery complications in the followup of GCA patients have been reported, based on evidence on imaging studies (plain radiography, ultrasonography, arteriography, intravenous angiography, computed tomography scanning, and magnetic resonance angiography), as well as surgical pathology and autopsy (4, 9, 10).
This case has significance because, to the best of our knowledge (using a PubMed search, Key Words: GCA/temporal arteritis/Horton disease and pulsatile mass or brachiocephalic aneurysm), there are no reports in the literature of aneurysms associated with GCA in which this presentation is described.
In our patient, the occurrence of several relapses of the disease may have accounted for the presence of a silent, smoldering, inflammatory vascular disease that may have led to aneurysm formation. In cases such as this, periodic physical examinations may be of some help in detecting asymptomatic vascular complications in the followup of GCA.
- 1Incidence and predictors of large-artery complications (aortic aneurysm, aortic dissection and/or large-artery stenosis) in patients with giant cell arteritis: a population-based study over 50 years. Arthritis Rheum 2003; 48: 3522–31., , , , .
- 8Increased risk of death in patients with giant cell arteritis related to thoracic aortic dissection: a population-based study over 50 years. Arthritis Rheum 2002; 46 Suppl 5 183: 406., , , , .
Carlos Garcia-Porrua MD, PhD*, Robustiano Pego-Reigosa MD, PhD*, Angela Piñeiro MD*, Victor Armesto MD*, Miguel A. González-Gay MD, PhD*, * Hospital Xeral-Calde Lugo, Spain.