Rheumatoid aortitis: a rarely recognized but clinically significant entity

Medicine (Baltimore). 1989 Mar;68(2):95-106.

Abstract

Aortitis as a feature of rheumatoid arthritis is considered rare. We have, however, identified 10 patients with aortitis from among 188 consecutive autopsy cases of rheumatoid arthritis. There were 5 men and 5 women with a mean duration of rheumatoid arthritis of 9.6 years. Nine were rheumatoid factor positive and had associated nodules. In addition to standard treatment regimens, 9 patients received corticosteroids. Although involvement of the thoracic aorta was most common, involvement of both the thoracic and abdominal aorta was present in 4 cases. Two patients had aneurysmal dilatation of the thoracic aorta and 1 of the abdominal aorta. Microscopic features of aortitis included necrosis of medial smooth muscle and elastica, with an inflammatory infiltrate comprising primarily lymphocytes and plasma cells. A panmural aortitis was seen in 3 cases. Rheumatoid granulomas were noted in the aortic wall in 5. The diagnosis of aortitis was not made until autopsy in any case. Aortitis was hemodynamically significant in 3 patients. Two had congestive heart failure secondary to thoracic aortitis and aortic valvulitis, and 1 had rupture of an abdominal aortic aneurysm at a site involved by aortitis. Seven patients had rheumatoid vasculitis with a mean of 10 organs involved. Six of these died of complications directly related to vasculitis, including 4 patients with coronary arteritis and associated myocardial infarction. Aortitis can be a feature of severe rheumatoid arthritis and is often associated with rheumatoid vasculitis. Hemodynamic compromise does occur and may be fatal.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Aorta, Abdominal / pathology
  • Aorta, Thoracic / pathology
  • Aortitis / etiology*
  • Aortitis / pathology
  • Arthritis, Rheumatoid / complications*
  • Arthritis, Rheumatoid / pathology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Rheumatoid Nodule / pathology
  • Vasculitis / etiology
  • Vasculitis / pathology