|Year : 2022 | Volume
| Issue : 5 | Page : 62-64
Aortic aneurysm associated with rheumatoid aortitis
Deepak Moka, Sai Subrahmanyam Pappu, Azigiri Sri Nanditha, Naval Chandra
Department of General Medicine, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
|Date of Submission||10-Dec-2021|
|Date of Decision||04-Mar-2022|
|Date of Acceptance||04-Apr-2022|
|Date of Web Publication||07-Jul-2022|
Additional Professor, Department of General Medicine, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad 500 082, Telangana
Source of Support: None, Conflict of Interest: None
A 59-year-old female, presented with dry cough, burning micturition with urgency and frequency for 2 months. She was known to have hypertension, hypothyroidism and rheumatoid arthritis for the past 30 years; and was receiving oral methotrexate 20 mg per-week. She also had complianed of low backache for 2 months. No phenotypic features of Marfans/Ehler Danlos were evident. Cardiovascular system examination was unremarkable. Chest radiograph showed mediastinal widening with a large fusiform descending thoracic aortic aneurysm. Computed tomography (CT) of the chest showed subpleural fibrosis and fusiform dilatation of the aorta from the origin till the supracoeliac aorta. CT aortogram revealed an irregular non-enhancing wall thickening and calcification of the aorta with fusiform dilatation (4.5 cm × 4.6 cm in the ascending aorta and 4.4 cm × 4.3 cm in the distal descending aorta). The patient did not have any features of giant cell arteritis (GCA)/Takayasu's arteritis; Venereal Disease Research Laboratory (VDRL) test was negative. The patient was diagnosed to have aortic regurgitation due to long standing rheumatoid arthritis and was referred to the department of cardiothoracic and vascular surgery for further management.
Keywords: Aneurysm, aortitis, rheumatoid arthritis
|How to cite this article:|
Moka D, Pappu SS, Nanditha AS, Chandra N. Aortic aneurysm associated with rheumatoid aortitis. J Clin Sci Res 2022;11, Suppl S1:62-4
|How to cite this URL:|
Moka D, Pappu SS, Nanditha AS, Chandra N. Aortic aneurysm associated with rheumatoid aortitis. J Clin Sci Res [serial online] 2022 [cited 2022 Dec 7];11, Suppl S1:62-4. Available from: https://www.jcsr.co.in/text.asp?2022/11/5/62/355076
| Introduction|| |
Rheumatoid arthritis is characterised by symmetric polyarthritis and is the most common cause of chronic inflammatory arthritis. Vasculitis occurs in fewer than 1% of patients with rheumatoid arthritis and usually in those with severe deforming disease and high titres of rheumatoid factor. Rheumatoid vasculitis usually involves small-medium vessels. Large vessel involvement with aortitis occurs in 5% of patients with rheumatoid vasculitis.
Ascending aortitis is underrecognised as a cause of ascending aortic aneurysms. Diagnosis is often incidental as most patients are asymptomatic. Aneurysm formation secondary to rheumatoid vasculitis is extremely rare and then usually affects the aortic root and ascending aorta. We report a case of thoracic aortic aneurysm secondary to rheumatoid arthritis.
| Case Report|| |
A 59-year-old female with a history of hypertension, hypothyroidism, and rheumatoid arthritis for 30 years on methotrexate 20 MG once/week, came with a complaint of dry cough for 2 months and burning micturition with urgency and frequency. There was a complaint of low backache for 2 months. No phenotypic features of Marfans/Ehler Danlos were evident. On cardiovascular system examination, no abnormalities were found.
Chest X-ray showed mediastinal widening with a large fusiform descending thoracic aortic aneurysm [Figure 1]. Computed tomography (CT) of the chest was done for the evaluation of chronic cough, which showed subpleural fibrosis and fusiform dilatation of the aorta from the origin till the supraceliac aorta. On CT aortogram, there was irregular non-enhancing wall thickening and calcification of the aorta with fusiform dilatation (4.5 cm × 4.6 cm in the ascending aorta and 4.4 cm × 4.3 cm in the distal descending aorta) likely to be sequelae of rheumatoid arthritis [Figure 2].
|Figure 1: Chest radiograph (postero-anterior view) showing mediastinal widening with a large fusiform shadow suggestive of descending thoracic aortic aneurysm|
Click here to view
|Figure 2: CT Angiography showing aneurysmal dilatation of root of aorta, ascending aorta, arch of aorta and descending aorta with irregularly thickened root of aorta|
Click here to view
The patient did not have any features of giant cell arteritis (GCA)/Takayasu's arteritis and VDRL test was negative.
The patient had mild aortic regurgitation (AR) and asymptomatic aneurysm with a size of 4.5 cm; she was advised to follow-up under cardiothoracic surgery.
| Discussion|| |
Aortitis is a general term that refers to a broad category of infectious or non-infectious conditions, in which there is an abnormal inflammation of the aortic wall. Aortitis occurs in a number of inflammatory and non-inflammatory disorders, including Kawasaki's syndrome, Behçet's syndrome, rheumatoid arthritis, syphilis and tuberculosis. Most commonly Salmonella and Staphylococcal species, along with Streptococcus pneumoniae, are the infectious causes of aortitis. Syphilitic or luetic aortitis, now exceedingly rare, typically involves the ascending aorta and is associated with thoracic aortic aneurysm.
The overall most common causes of aortitis are the large-vessel vasculitides GCA and Takayasu's arteritis. In addition to GCA and Takayasu's arteritis, other rheumatologic disorders including rheumatoid arthritis, systemic lupus erythematosus, granulomatosis with polyangiitis, Behçet's disease, polyarteritis nodosa and microscopic polyangiitis may lead to aortitis. In the case of rheumatoid-associated aortitis, rheumatoid nodules are in the aortic wall in up to 50% of pathologic specimens.
In an autopsy study of 188 patients with rheumatoid arthritis, a 5.3% prevalence of aortitis and 1.6% prevalence of aneurysm formation were reported. The aortic valve and annulus may also be affected with granulomatous or non-granulomatous inflammation, leaflet thickening and secondary regurgitation.
RA-associated aortitis essentially occurs in patients with severe RA, indicating an association with RV. The following risk factors for RV onset have been reported: (i) high-titre rheumatoid factor; (ii) course of 1 year or more after the onset; (iii) presence of bone erosions; and (iv) presence of rheumatoid nodules.
Characteristic histopathology confirmation of vasculitis is generally necessary for a diagnosis of rheumatoid vasculitis. Other clinical and laboratory features are often supportive and may be used to assist follow-up. Since the introduction of CT, it has become the preferred imaging technique to define aortic anatomy. The sensitivity and specificity of angioscans have increased greatly in the last few years reaching up to 100%. As the patient had multiple risk factors for atherosclerotic cardiovascular disease, and as RA can lead to accelerated atherosclerosis, atherosclerotic aneurysm was considered but the aortogram was suggestive of aneurysm as sequelae of inflammatory aortitis.
If diagnosed early, mild-to-moderate dilated ascending aortas can certainly benefit directly from medications such as beta-blockers and angiotensin converting enzyme inhibitors. In asymptomatic patients, surgical repair is indicated when the size exceeds 5.5 cm or a growth rate of 0.5 cm/year. Aortitis is a rare cause of aneurysm. Aortic aneurysm as a consequence of aortitis due to inflammatory disorders line GCA or Takayasu's arteritis has been reported. In a patient with suggestive history, the possibility of rheumatoid aortitis needs to be considered.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
| References|| |
Modi P, Suvarna K, Cooper GJ. Images in cardiology. Descending thoracic aortic aneurysm in rheumatoid arthritis. Heart 2000;84:122.
Restrepo CS, Ocazionez D, Suri R, Vargas D. Aortitis: Imaging spectrum of the infectious and inflammatory conditions of the aorta. Radiographics 2011;31:435-51.
Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med 2003;349:160-9.
Gornik HL, Creager MA. Aortitis. Circulation 2008;117:3039-51.
Gravallese EM, Corson JM, Coblyn JS, Pinkus GS, Weinblatt ME. Rheumatoid aortitis: A rarely recognized but clinically significant entity. Medicine (Baltimore) 1989;68:95-106.
Levine AJ, Dimitri WR, Bonser RS. Aortic regurgitation in rheumatoid arthritis necessitating aortic valve replacement. Eur J Cardiothorac Surg 1999;15:213-4.
Kaneko S, Yamashita H, Sugimori Y, Takahashi Y, Kaneko H, Kano T, et al.
Rheumatoid arthritis-associated aortitis: A case report and literature review. Springerplus 2014;3:509.
Bartels CM, Bridges AJ. Rheumatoid vasculitis: Vanishing menace or target for new treatments? Curr Rheumatol Rep 2010;12:414-9.
Hartnell GG. Imaging of aortic aneurysms and dissection: CT and MRI. J Thorac Imaging 2001;16:35-46.
Saliba E, Sia Y, Dore A, El Hamamsy I. The ascending aortic aneurysm: When to intervene? IJC Heart Vasc 2015;6:91-100.
[Figure 1], [Figure 2]