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Clinical Infection & Immunity

Case Report

Volume 2, Number 1, March 2017, pages 16-18

Rheumatic Mitral Stenosis With Takayasu’s Arteritis: Coincidence or Association?

Takayasu’s arteritis (TA) is an idiopathic chronic inflammatory disease of unknown etiology, pathologically characterized by granulomatous changes of the aorta and its major branches [1]. TA has been reported worldwide; it is more common in young Asian women. Females in the second or third decade of life are at greatest risk for this disease. Female-to-male ratio varied from 9:1 in reports from Japan to 1.2:1 in Israel [2, 3]. Its incidence varies from 3.6 cases/million per year in Japan to 1 - 2 per million per year worldwide [4, 5]. TA, in fact, is a panarteritis, beginning with inflammation of the adventitia with subsequent involvement of the media and intima. The characteristic pathological features include vessel wall inflammation leading to thickening, fibrosis, segmental stenosis, and aneurysm and thrombus formation. The exact etiopathogenesis is unknown [1, 6]. Since the original description, the disease has been associated with autoimmune conditions such as glomerulonephritis, systemic lupus erythematosus, juvenile idiopathic arthritis, anterior uveitis, sarcoidosis, Crohn’s disease, Wegener’s granulomatosis, Sweet syndrome, and ulcerative colitis, which may indicate immune mechanisms in the pathogenesis [7].

Rheumatic mitral stenosis is a chronic sequela of acute rheumatic fever, an autoimmune disease related to previous throat infection with group A β-hemolytic streptococcus. The prevalence of rheumatic heart disease is estimated to be 15.6 million cases worldwide [8].

We hereby describe a case of TA with rheumatic mitral stenosis. The presence of both diseases raises the possibility of common immunological basis for the pathogenesis of both diseases.

A 23-year-old female presented to us with complaints of exertional dyspnea and palpitations along with right arm claudication from last 5 years. On clinical examination, she was found to have absent brachial and radial pulse and diminished carotid artery pulse on right side. Her blood pressure was 130/80 mm Hg in left upper limb, not recordable in right upper limb. Auscultation of the patient revealed loud first heart sound, a mid-diastolic murmur at apex and a carotid bruit on right side. Erythrocyte sedimentation rate was 25 mm (first hour) by Westergren method. Chest X-ray revealed straightening of left cardiac border with pulmonary venous hypertension. Electrocardiogram revealed normal sinus rhythm with evidence of left atrial enlargement. Two-dimensional and color Doppler echocardiography revealed normal left ventricular function and features typical of mitral stenosis such as thickened valve with restricted motion of both anterior and posterior mitral leaflets and dilated left atrium. Mitral valve orifice area was 1.0 cm². Computed tomographic aortogram of the patient revealed significant stenosis of right brachiocephalic trunk, right common carotid and subcalvian arteries and narrowed descending thoracic aorta (Fig. 1).

Click for large image

Figure 1. (a) Two-dimensional transthoracic echocardiography showing parasternal short axis view at mitral valve level showing mitral valve orifice area of 1.0 cm2. (b) Continuous wave Doppler at mitral inflow showing severe mitral stenosis having mean gradient of 19 mm Hg. (c, d) Three-dimensional computed tomographic aortogram showing stenosis of brachiocephalic trunk, right common carotid artery and right subclavian artery and narrowed descending thoracic aorta segment.

Our patient fulfilled American College of Rheumatology criteria for TA [9]. TA is a form of granulomatous arteritis, which affects large- and medium-sized arteries, primarily the aorta and its large branches as well as proximal portions of pulmonary, coronary, and renal arteries that results in segmental stenosis, occlusion, dilatation, and aneurysmal formation in the affected vessels. Although of worldwide distribution, TA is comparatively more prevalent in Asian population including India [7]. In India it most commonly affects the abdominal aorta and renal arteries, in comparison to the originally diagnosed patients of Japanese origin where aortic arch and its branches are commonly involved [10]. Rheumatic heart disease is estimated to be present in 0.44 - 3.37 million people in India with mitral stenosis being the most common valvular heart disease in women in India [11]. Diagnosis of TA rests on clinical suspicion in any young patient, particularly women, with clinical manifestations of vascular ischemia and the presence of bruits, decreased or absent pulses, ischemic ulcers, or a combination of these findings.

The exact mechanism by which group A streptococcus causes acute rheumatic fever remains unexplained. Historically three hypotheses have been postulated to explain a streptococcal pathogenesis for rheumatic fever. These include: 1) direct infection (for example, by the group A streptococcus); 2) effects of a streptococcal toxin (streptolysin O); and 3) the concept of antigenic mimicry in association with an abnormal immune response. Although no mechanism has been unequivocally proven, autoimmunity, or, more precisely, antigenic mimicry appears to be the most likely explanation [12].

The precise factors in TA that are responsible for the vascular damage are unknown. The presence of increased mononuclear cells, circulating anti-endothelial cell antibodies, circulating immune complexes, and a favorable response to steroids suggests the pathogenic role of autoimmunity [13]. One can speculate that in patients with rheumatic mitral stenosis and TA, serum antibodies could recognize cardiac, brain and vessel antigens causing both conditions.

The co-occurrence of rheumatic mitral stenosis and TA has infrequently been described. Doi et al described a case report of a 44-year-old female having mitral stenosis with TA [14]. Aggarwal et al described a 21-year-old female with rheumatic mitral stenosis with aortoarteritis which was treated with percutaneous balloon mitral valvotomy for MS and stenting for aortoarteritis [15].

The concurrent occurrence of TA with rheumatic mitral stenosis in our case is whether due to etiological relation or is merely a coincidence remains to be answered. The presence of rheumatic mitral stenosis in a patient with TA raises the possibility of a common immunological basis for the pathogenesis of both diseases.

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