Article Text

PDF
AB0449 Isolated aortitis as an atypical presentation of giant cell arteritis
  1. F. Martinez-Valle1,
  2. M. Ramentol-Sintes1,
  3. C. Perez-Bocanegra1,
  4. S. Bujan-Rivas1,
  5. M. Vilardell-Tarrés1,
  6. R. Solans-Laqué1
  1. 1Internal Medicine, Vall d’Hebron Hospital, Barcelona, Spain

Abstract

Background The most common causes of aortitis are the large-vessel vasculitis, although it also is associated with several other rheumatologic diseases. Giant cell arteritis (GCA) is the most common form of large vessel vasculitis (1). Because aortic inflammation has no symptoms until complications arise, aorta has been believed to be uncommon target of the disease. However, aortic involvement has been estimated to occur in up to 18% with GCA, but some studies marks than in early histological proven GCA any degree of thickening of the abdominal aorta may be found in up to 27% of patients (2).

Objectives To describe the presence of isolated aortitis as a presentation of GCA without typical clinical symptoms.

Methods We included all the patients diagnosed of CGA in our hospital from 1991 to 2011, according to the American College of Rheumatology (ACR) classification criteria. Statistical analysis was performed using the SSPS vs. 15.0.

Results Among the 174 patients with GCA, 124 were women (70.9%) and 50 men (29.1%). Mean age at diagnosis was 76 years (51-92). Typical symptoms at presentation were headache in 150 (86.2%), intermittent claudication of the muscles of mastication in 93 patients (53.4%), constitutional syndrome in 84 (48.3%), polymyalgia rheumatic syndrome in 74 (42.5%), fever in 38 (21.8%), permanent visual loss in 51 (29.3%), and amaurosis fugax in 25 (14.4%). Temporal artery biopsy was performed in 162 patients (93%), with intimal hyperplasia in 94 (54%), inflammatory infiltrate in 91 (52.3%), presence of giant cells in 80 (46%), and internal elastic lamina disruption in 62 (35.6%). Artery biopsy was normal in 9 patients (5.2%).

Most frequently, the combination of typical clinical symptoms in the setting of an aged patient triggered the suspicion of GCA. However, in three patients the only clinical manifestations were weight loss and asthenia. In this clinical setting a thoracic-abdominal scan was performed with the suspicion of disseminated neoplasm and a diagnosis of isolated aortitis was made. One of the patients had floating thrombi attached to the wall of the aorta, probably secondary to aortitis. In front of the presence of an elevated acute reactants and an aortitis, a temporal artery biopsy was carried out, demonstrating the presence of a GCA. In this subset of patients, mean age was 74.3 years old (70-77), and raised erythrocyte sedimentation rate (mean 97) and anemia (mean 83 gr/l) was present in the three. Good evolution of the aortitis was seen in the three patients.

Conclusions Aortic involvement in the acute phase of GCA is probably also more frequent than estimated because it is symptomatic in only a minority of patients. The presence of isolated aortitis should rule out a diagnosis of GCA, even though the absence of typical symptoms.

  1. Martínez-Valle F, Solans-Laqué R., Bosch-Gil J, Vilardell-Tarrés M. Aortic involvement in giant cell arteritis. Autoimm Rev 2010 May;9(7):521-4.

  2. Agard C., Barrier J, Dupas B, Ponge T, Mahr A, et al. Aortic involvement in recent-onset giant cell (temporal) arteritis: a case-control prospective study using helical aortic computed tomodensitometric scan. Arthritis and Rheum 2008. 59(5): 670-6.

Disclosure of Interest None Declared

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.