Background/Purpose: Idiopathic aortitis is a rare diagnosis that may occur in the context of a primary systemic vasculitis, as part of a systemic autoimmune disease, or in isolation. In patients with focal isolated aortitis (FIA), surgery alone may be curative; however, new vascular lesions have been reported to develop in between 5-47% of cases. The risk of progression to systemic disease and optimal management strategy for FIA patients is uncertain.
Methods: Patients with biopsy-proven aortitis, diagnosed following thoracic aortic surgery at the Cleveland Clinic between 1996 and 2012, were retrospectively reviewed. Patients were classified into clinical subgroups [Giant cell arteritis (GCA), Takayasu’s arteritis (TAK), Focal Isolated Aortitis (FIA) or Other] at the time of surgery using pre-defined criteria. Symptoms, pathology, laboratory and imaging results were recorded at surgery and over time using a standardized database. Patients with FIA at surgery were followed for progression to systemic disease and outcomes of clinical subgroups were compared.
Results: Of 7,551 patients who underwent thoracic aortic surgery between 1996-2012, 196 patients with biopsy-proven aortitis were identified for review. Median age at surgery was 69 years (range 15-88) and 67% were female. At the time of surgery, 129 (65.8%) patients met criteria for FIA, 42 (21.4%) for GCA, 14 (7.1%) for TAK, and 11 (5.6%) for Other. A minimum of 6 months of clinical follow-up was available for 73 FIA patients. During follow-up (median 45 months, range 6-201 months), 14/73 (19.2%) FIA patients developed symptoms of systemic disease, 17/40 (42.5%) developed elevated inflammatory markers, 29/65 (44.6%) developed new vascular lesions on imaging, 30/73 (41.1%) required a second vascular surgery, 7(9.6%) dissected and 9 died (12.3%.) Ultimately 23 of 73 (31.5%) with FIA progressed to have features of a systemic disease: 21 GCA, 1 TAK, and 1 Other. When compared to patients with known systemic disease at surgery, patients with FIA were less likely to develop symptoms (p=0.01) but no different with respect to development of elevated inflammatory markers (p=0.19), new vascular lesions by imaging (p=0.92), need for further vascular surgery (p=0.84), dissection (p=0.40) or death (p=0.76) over time. Only 12 patients with FIA at surgery received immunosuppressive therapy post-operatively. Over time, 0/11 treated FIA patients with follow-up imaging developed aneurysms, but 2 (18.2%) developed new stenoses. Among the 54 untreated FIA patients with imaging available, 27 (50%) developed new lesions (23 aneurysms and 5 stenoses.) Additional tissue obtained after subsequent surgery in 2 untreated FIA patients revealed persistent inflammation in the distal aorta.
Conclusion: Over time, nearly one third of patients classified as FIA at the time of surgery progressed to have features of a systemic autoimmune disease. Patients with FIA are less likely to develop overt symptoms, but equally likely to develop elevated inflammatory markers or new vascular lesions on imaging when compared to GCA, TAK and Others. These patients require regular clinical follow-up and serial imaging to assess for progression.
Disclosure:
A. Clifford,
None;
A. Arafat,
None;
J. Idrees,
None;
E. Roselli,
None;
C. D. Tan,
None;
E. R. Rodriguez,
None;
L. Svensson,
None;
E. Blackstone,
None;
G. S. Hoffman,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/aortitis-outcomes-from-a-cohort-of-196-patients/