Ascending Giant Cell Aortitis without Systemic Symptoms Is Associated with an Increased Frequency of Subsequent Distal Aortic Events
H Wang, RN Smith, JR Stone. Massachusetts General Hospital and Harvard Medical School, Boston, MA
Background: In patients undergoing resection of the ascending aorta for aneurysm or dissection, giant cell aortitis (GCA) is occasionally identified upon pathologic examination. Frequently, ascending GCA is present without systemic systems of vasculitis (GCA-WSS). The clinical significance of GCA-WSS has been unclear. Specifically, it has not been established if patients with ascending GCA-WSS are at increased risk for more distal aortic events on long-term follow-up.
Design: Ascending aortic segments resected for aneurysm or dissection between 1980 and 2004 were reviewed. GCA-WSS was identified by the presence of granulomatous inflammation with or without giant cells. Exclusion criteria included prior or concurrent evidence of systemic rheumatologic disease, age less than 50, mycotic / infectious aortitis, lymphoplasmacytic aortitis, and follow-up of less than fours years duration. Twelve GCA-WSS cases with adequate follow-up were identified. For each GCA-WSS case, 2 non-aortitis control ascending aorta cases were identified. Aortitis cases and controls were matched for age, gender, indication for surgery, date of surgery, type of surgery, and follow-up duration. In both groups, follow-up included imaging with CT and/or MR for the majority of the patients. Aortic events were defined as operations, new aneurysms greater than 5 cm, ruptured aneurysms, or dissections involving the descending thoracic or abdominal aorta.
Results: 5 of the 12 GCA-WSS patients (42%) experienced distal aortic events during follow-up. There were new large thoracoabdominal or descending thoracic aortic aneurysms in three patients, and ruptured thoracoabdominal aneurysms in two additional patients. Three of these 5 patients underwent subsequent aortic surgery. In contrast, only 1 out of 24 patients (4%) in the non-aortitis group had an event, a new thoracoabdominal aneurysm. The difference in the frequency of events is statistically significant, P=0.01.
Conclusions: In long-term follow-up, patients with GCA-WSS are at significantly increased risk for distal aortic events compared with non-aortitis patients. In GCA-WSS patients, subsequent aortic events occur most often in the descending thoracic aorta and/or suprarenal abdominal aorta rather than the infrarenal abdominal aorta.
Monday, March 22, 2010 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 44, Monday Morning