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A Rare Association of Rheumatoid Arthritis with Ascending Aortic Aneurysm

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RAVI PRAKASH PANDEY, KD SINGH, M INDURKAR, DHARMENDRA JAIN    24 October 2017

Keywords

Aortic aneurysm, cardiovascular diseases, inflammatory disorder, rheumatoid arthritis

Rheumatoid arthritis (RA) is the commonest inflammatory polyarthritis seen in clinical practice. The prevalence of RA is about 0.5-1% in industrialized nations with 5-50 cases per 1,00,000 populations annually. It predominantly occurs in females and elderly people. In India, prevalence of RA was found to be 0.5-0.75%.1,2 RA primarily affects joints; however, it also affects other organ in 15-25% of individuals.3 Much less appreciated is the fact that what hurts in RA is the joint, but what kills is the ‘heart’. Over the years, cardiovascular disease (CVD) has emerged as a major cause of morbidity and mortality in RA. It can cause a spectrum of CVDs viz. myocardial infarction, valvular dysfunctions and possibly aortic aneurysm.4,5 Here we report one such case of ascending aortic aneurysm in association with RA.

CASE REPORT

A 72-year-old male, nonsmoker, presented to the outpatient department of our tertiary care hospital with complaints of difficulty in swallowing, blood tinged sputum, palpitation and breathlessness since 1½ months. Patient was a known case of RA since last 12 years. He had a negative history for hypertension, diabetes mellitus, ischemic heart disease and chronic obstructive pulmonary disease. On general examination, patient was average built with radial deviation at the wrist and bony deformity in digits of hands and feet (Fig. 1). No sign of pallor, cyanosis, clubbing, icterus, edema and lymphadenopathy was present. Patient was conscious, well-oriented to time, place and person. Vital parameters were as follows: Patient was afebrile, respiratory rate (14/min), pulse rate (90/min), irregular, high volume, collapsing with apex pulse deficit of 12. Blood pressure in right and left upper limb supine position was 128/50 mmHg and 120/40 mmHg and right and left lower limb was 170/60 mmHg and 156/50 mmHg, respectively.

Cardiovascular (CVS): On inspection, apex impulse was seen over left 6th intercostal space 1.5 cm lateral to the mid-clavicular line. On palpation, apex beat was felt in the same space with hyperdynamic character. On auscultation, S1 was variable in intensity in aortic, pulmonary, tricuspid and mitral area, respectively with soft S2. A decrescendo diastolic murmur extending up to late diastole was heard to the right of sternum which was soft-high-pitched and blowing, best appreciated during expiration and leaning forward. Other systemic examinations were unremarkable.

Routine investigations were within normal limits. RA factor was strongly positive (296 IU/mL). Chest X-ray PA view was suggestive of cardiomegaly with left ventricular (LV) type of apex (Fig. 2). ECG showed LV hypertrophy (LVH) with LV strain pattern with underlying atrial fibrillation (Fig. 3). 2D-echocardiography (TTE) of the patient revealed grossly dilated ascending aorta (diameter ~7.11 cm) with nonrheumatic severe AR with mild AS with moderate left ventricle systolic dysfunction (left ventricular ejection fraction [LVEF] = 35.24%) (Figs. 4 and 5). Tricuspid aortic valve (unequal cusp) with annuloaortic ectasia with effacement of sinotubular junction was present. Mild-to-moderate mitral regurgitation was also present.

DISCUSSION

Aneurysm of aorta is an abnormal dilatation of aorta in all the three layers of aortic wall. The basic defect is destruction of the elastic fibers in the tunica media, which are replaced by fibrosis. The association between RA and loss of elasticity of aorta is principally due to inflammatory changes and structural alteration such as smooth muscle hypertrophy and changes in intracellular matrix characterized by an increase in collagen and decrease in elastin.

Smooth muscle cells can produce bioapatite (they express osteoblastic markers under inflammatory conditions), which causes calcification of the aortic tunica media. Perivascular inflammation and cellular infiltration around the vasa vasorum have been, observed to result in ischemia.6

Moreover, in RA, a type of peripheral neuropathy caused by necrotizing vasculitis of the vasa vasorum has been reported. Elderly onset RA has been associated with poorer prognosis, as manifested by persistent disease activity, more frequent systemic association and more rapid functional decline. Patients having high titers of rheumatoid factor have more aggressive disease activity as compared to seronegative patients.

Hollan et al7 studied changes in the aortic wall (i.e., inflammatory changes) in specimens obtained following coronary artery revascularization surgery and compared findings in patients with and without RA. They excluded patients with infiltration close to the intima, which can occur in normal individuals and focussed on observations in the tunica media and tunica adventitia. They concluded that the observed changes were greater in patients with RA and that they could represent an inflammatory state that promotes the development of arteriosclerosis and the formation of aneurysms by decreasing the resistance of the arterial wall. Aortic aneurysm can also be the result of medial dysfunction induced by chronic inflammation and accelerated by prolonged corticosteroid therapy.8

Vasulitis occurs in less than 1% of patients with RA and usually only in those with severe deforming RA and high titers of rheumatoid factor. Large vessel involvement with aortitis occurs in 5% of patients with rheumatoid vasculitis. Aneurysm formation secondary to rheumatoid vasulitis is extremely rare and then usually affects the aortic root and ascending aorta.9

Although initially, the vasculitis was attributed to occlusive vascular disease associated with giant cell arteritis (which corresponds to the current understanding of RA, principally with regard to coronary arteries), it was subsequently clear that inflammatory changes in the aorta associated with high blood pressure can also lead to the formation of an aneurysm and dissection.10,11 In the past aortic aneurysm has been reported with several connective tissue disorders like Marfan’s syndrome, Ehler-Danlos syndrome and systemic lupus erythematosus.

CONCLUSION

Involvement of ascending aorta in RA is very rare. Such massive aneurysm along with valvular dysfunction has been rarely reported in literature. Thus, the above case report highlights the possibility of a massive aneurysm being attributed to the long-standing inflammatory changes and structural alteration brought to the great vessel by RA.

REFERENCES

  1. Chopra A, Patil J, Billempelly V, Relwani J, Tandle HS; WHO-ILAR COPCORD Study. WHO International League of Associations from Rheumatology Community Oriented Program from Control of Rheumatic Diseases. Prevalence of rheumatic diseases in a rural population in western India: a WHO-ILAR COPCORD Study. J Assoc Physicians India. 2001;49:240-6.
  2. Malaviya AN, Kapoor SK, Singh RR, Kumar A, Pande I. Prevalence of rheumatoid arthritis in the adult Indian population. Rheumatol Int. 1993;13(4):131-4.
  3. Turesson C, O’Fallon WM, Crowson CS, Gabriel SE, Matteson EL. Extra-articular disease manifestations in rheumatoid arthritis: incidence trends and risk factors over 46 years. Ann Rheum Dis. 2003;62(8):722-7.
  4. Hadda V, Handa R, Aggarawal P, Lakshmy R, Kumar U, Pandey RM. Disease activity and lipids in rheumatoid arthritis: a prospective. Indian J Rheumatol. 2007;2(4): 137-40.
  5. Handa R, Chaurasia A, Hari S, Kumar U, Gupta R. Endothelial cell dysfunction in rheumatoid arthritis. Ann Rheum Dis. 2007;66(Suppl II):67.
  6. Yildiz M. Arterial distensibility in chronic inflammatory rheumatic disorders. Open Cardiovasc Med J. 2010;4:83-8.
  7. Hollan I, Scott H, Saatvedt K, Prayson R, Mikkelsen K, Nossent HC, et al. Inflammatory rheumatic disease and smoking are predictors of aortic inflammation: a controlled study of biopsy specimens obtained at coronary artery surgery. Arthritis Rheum. 2007;56(6):2072-9.
  8. Ohara N, Miyata T, Sato O, Oshiro H, Shigematsu H. Aortic aneurysm in patients with autoimmune diseases treated with corticosteroids. Int Angiol. 2000;19(3):270-5.
  9. Modi P, Suvarna K, Cooper GJ. Images in cardiology. Descending thoracic aortic aneurysm in rheumatoid arthritis. Heart. 2000;84(2):122.
  10. He R, Guo DC, Estrera AL, Safi HJ, Huynh TT, Yin Z, et al. Characterization of the inflammatory and apoptotic cells in the aortas of patients with ascending thoracic aortic aneurysms and dissections. J Thorac Cardiovasc Surg. 2006;131(3):671-8.
  11. Gonzalez-Gay MA, Garcia-Porrua C, Piñeiro A, Pego-Reigosa R, Llorca J, Hunder GG. Aortic aneurysm and dissection in patients with biopsy-proven giant cell arteritis from northwestern Spain: a population-based study. Medicine (Baltimore). 2004;83(6):335-41.

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