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1.
Arthritis Rheum ; 43(4): 901-7, 2000 Apr.
Article in English | MEDLINE | ID: mdl-10765937

ABSTRACT

OBJECTIVE: To determine 1) the frequency of idiopathic aortitis in a large surgical cohort, 2) how often aortitis was associated with a systemic disease, and 3) whether the findings of aortitis in resected specimens predicted future occurrences of clinically apparent vascular injury due to vasculitis. METHODS: Retrospective chart and pathology review of 1,204 aortic surgical specimens that were gathered over a period of 20 years at a tertiary care medical center. A standardized database was used to compare features of aortitis patients with those of controls in whom inflammation was not present. RESULTS: Among 1,204 aortic specimens, 52 (4.3%) were clinically and pathologically classified as idiopathic aortitis. Sixty-seven percent of patients with idiopathic aortitis were women. In 96% of idiopathic aortitis patients with aneurysm formation, aortitis was present only in the thoracic aorta. Among 383 thoracic aortic aneurysms, 12% had idiopathic inflammatory features. In 96% of patients with idiopathic aortitis, symptoms of systemic illness had not been present at the time of surgery. In 31%, aortitis was associated with a remote history of vasculitis and a variety of other systemic disorders. During a mean followup period of 41.2 months, new aneurysms were identified among 6 of 25 patients who were not treated with glucocorticoids. None were identified among 11 patients who were treated with glucocorticoids (mean followup 35.5 months). CONCLUSION: The frequency of idiopathic aortitis in a large surgical cohort was found to be 4.3%. Thoracic aorta aneurysm formation, in the absence of systemic illness, was the most common manifestation. In the setting of a cardiovascular surgery practice, aortitis may first become apparent only after pathologic evaluation of excised specimens. The appropriate medical treatment for patients with incidentally discovered aortitis is not known. Because 17% of our patients subsequently developed new aneurysms, we suggest that it would be prudent for patients with idiopathic aortitis identified at the time of surgery to be periodically evaluated for recurrent or persistent disease.


Subject(s)
Aortitis/etiology , Adult , Aged , Aged, 80 and over , Aorta/pathology , Aorta/surgery , Aortic Aneurysm/surgery , Cohort Studies , Female , Humans , Male , Middle Aged , Retrospective Studies
2.
J Pediatr ; 122(1): 26-31, 1993 Jan.
Article in English | MEDLINE | ID: mdl-8419611

ABSTRACT

We prospectively studied and compared clinical features, treatment, course of illness, and long-term morbidity and mortality rates for Wegener granulomatosis in 23 childhood-onset patients with those of 135 adult-onset patients who were studied concurrently. Treatment was usually provided with glucocorticoids and cyclophosphamide. The mean follow-up period was 8.7 years for childhood-onset and 7.6 years for adult-onset Wegener granulomatosis. Most aspects of Wegener granulomatosis were similar in childhood-onset and adult-onset patients. Permanent morbidity from disease occurred in 86% of both groups. However, some features were significantly different. Wegener granulomatosis in childhood-onset patients was complicated five times more often by subglottic stenosis and twice as often by nasal deformity. Treatment-related permanent morbidity occurred in 22% of childhood-onset patients and 45% of adult-onset patients. After similar periods of cyclophosphamide therapy and follow-up, cyclophosphamide-related malignancies were less likely (0% vs 11%) to have developed in childhood-onset patients. Although 89% of patients treated with glucocorticoids and cyclophosphamide had remission, prolonged delay in achieving remission and relapses led in both patient groups to freedom from active disease for approximately 50% of the total patient-years. As a result, morbidity was substantial and has led to comparative studies of alternative therapies.


Subject(s)
Granulomatosis with Polyangiitis/physiopathology , Adolescent , Adult , Age Factors , Child , Cyclophosphamide/adverse effects , Cyclophosphamide/therapeutic use , Eye Diseases/physiopathology , Female , Follow-Up Studies , Glomerulonephritis/physiopathology , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/drug therapy , Humans , Laryngostenosis/physiopathology , Lung Diseases/physiopathology , Male , Nose Diseases/physiopathology , Opportunistic Infections , Prednisone/adverse effects , Prednisone/therapeutic use , Prospective Studies , Remission Induction , Survival Rate , Treatment Outcome
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