Association of ankylosing spondylitis and Takayasu’s arteritis: a case report

Manuscript type : Case Report | Article Date : 2015/02/04

  • Autors

    1. Mirfeizi Zahra
    2. Hashemzadeh Kamila
    3. Fazlinejad Afsoun
  • Abstract

    Ankylosing spondylarthritis and Takayasu’s arteritis are two inflammatory diseases with a not yet elucidated pathogenic mechanism. Only a few cases with this association (15 cases) have been published in the literature. We report here a new case of a 20-year-old, HLA-B27 positive male patient, who was the known case of ankylosing spondylitis. He was treated with Diclofenac and Sulfasalazin and the result was acceptable. One year after the first visit he presented with palpitation, dyspnea, nightly cough, absence of pulsation in the right radial artery, anemia and an increase in erythrocyte sedimentation rate (ESR). By performing colour doppler ultrasonography and neck and abdomen magnetic resonance angiography (MRA), Takayasu disease (TA), which led to stenosis of the common carotid, subclavian artery, celiac and renal arteries, was diagnosed. This occured two years after the initiation of ankylosing spondylitis. Treatment was begun with 40 mg prednisolone per day, Methotrexate 7.5 mg per week, Amilodipin 7.5 mg per day, Atrovastatin 20 mg per day and Aspirin, which were successfully tolerated. Now, two years after the diagnosis, he is in remission and all of the drugs have been stopped.
  • Description


    Ankylosing spondylitis (AS) is a chronic inflammatory disease of unknown cause associated with human leukocyte antigen (HLA)-B27. In general population AS occurs in 1-2% of HLA-B27 positive adults.1 The male to female preponderance is 2:1.2 This disease presents with idiopathic, progressive, inflammatory arthritis of the spinal, extra axial and sacroiliac joints.1 Uveitis, prostatitis, pulmonary fibrosis, and cardiovascular lesions such as conduction defects, 3 transmural aortitis with aortic incompetence, and slight aortic widening 4,5 are also seen in AS. It is rare, however, to find a patient with AS who presents with a true aortic arch syndrome. We report here the rare case of a young man with ankylosing spondylitis who later developed Takayasu’s arteritis.


    A 20-year-old man was admitted to hospital complaining of dyspnea in November 2009. He had been a known case of ankylosing spondylitis for 4 years. His first symptoms including longstanding morning stiffness, alternative low back pain and restriction in lumbar spine began 5 years ago. However, the diagnosis and treatment was delayed. He was referred to us in November 2008 with these symptoms. Upon physical examination, bilateral sacroiliac tenderness and restriction of the spinal movement were detected. Anteroposterior pelvic x ray showed evidence of bilateral subchondral sclerosis, joint space narrowing at left sacroiliac with erosive changes and in some areas ankylosis on the edges of the right sacroiliac joint (Fig. 1). HLA-B27 was positive. Treatment with Diclofenac and sulfasalazine was initiated. After 3 months, pain, morning stiffness, and restriction of the spinal movement disappeared and ESR became normal. Four months later, he presented with complaints of vomiting and gasteroesophageal reflux. In laboratory tests, hypochrome microcytic anemia and a rise in the ESR were observed. Omeprazol was added to other drugs and gasteroesophageal endoscopy was conducted. Gastric biopsy showed chronic nonspecific deodenitis. One year after the first visit, he presented with palpitation, dyspnea and nightly cough. In physical examination, absence of pulsation in the right radial artery was noticed. Blood pressure was 110/70 in the right arm and 160/110 in the left. Bruit was heard on the right carotid artery. All arterial leg pulses were present and blood pressure in low extremities was normal. No heart murmur was heard. However, S3 was heard over the left ventricular apex. As a result, the patient was hospitalized and evaluated. At the time of admission, the patient had no low back pain and the spinal movement was normal. Laboratory tests displayed microcytic hypochrome anemia and a dramatic rise in erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Laboratory data has been shown in table 1. Chest x-ray demonstrated cardiomegaly. Colour doppler ultrasonography of common subclavian and common carotid artery revealed that the thickness of intima and media of both common carotid arteries had increased. In the right subclavian artery, a significant rise in intima media thickness was also observed. Neck and abdomen magnetic resonance angiography (MRA) were performed. This demonstrated a stenosis less than 2 cm in the right common carotid artery. In the right subclavian artery, a moderate to severe stenosis with a length of 4.5 cm was noticed (Fig.2). An original stenosis of celiac and both renal arteries were noted in abdominal MRA, but both renal arteries were affected more in terms of length and severity in comparison with celiac artery. The electrocardiogram was normal and trans-thoracic and trans-esophageal echocardiography showed mild left ventricle (LV) systolic dysfunction, mild global hypokinesia, mild aortic insufficiency and mild intimal thickening of descending aorta in mid and proximal parts and a protruding atheroma. Ejection fraction was 50%. Initially, the patient received prednisolone, Methotrexate, Amilodipin, Atrovastatin and Aspirin and after one month his clinical and laboratory data improved. Now, after two years the patient does not have any symptoms and prednisolone and Methotrexate have been stopped. His thoraco-lumbar symptoms have been resolved and he has no restriction of spinal movement.


    Association of ankylosing spondylitis with Takayasu arteritis is very rare and may be accidental. The case we presented here had the diagnostic criteria for AS of Moll and Wright.6 As we know cardiac manifestations of ankylosing spondylitis in the form of involvement of the aortic and mitral valve and conductive heart system have been documented. 7 The incidence of aortitis in AS is approximately 5% and histologically, it can resemble Takayasu disease. However, in AS it has an insidious onset and is not associated with systemic manifestation.8 AS often involves the aortic root but distal aortic root involvement has rarely been reported. 3-5,8 Thus, in our patient, there were several features that confirmed the diagnosis of Takayasu arteritis simultaneously. These features included laboratory findings, angiographic studies, and clinical course (i.e., the loss of peripheral pulses in an adult) confirming the diagnosis of Takayasu arteritis. The association of ankylosing spondylitis and Takayasu arteritis has been described in several studies as shown in Table 2. In the first study in 1966, a possible association of Takayasu’s arteritis with ankylosing spondylitis in four cases was reported. 9 In these cases radiological evidence of bilateral sacroiliitis was noticed, which was indistinguishable from ankylosing spondylitis as in our case. Two of the cases with AS and Takayasu had syndesmophyte formation of the spine. Some of the cases reported did not had sacroiliitis and it was reported that sacroiliitis in Takayasu’s arteritis is more common in females. 1, 8 One of the cases reported before had erosive seronegative peripheral arthritis 16 and there was a report of pseudoarthrosis in a patient with Takayasu’s arteritis and AS in another study. 17 In some cases, besides AS and Takayasu arteritis, ulcerative colitis or chron disease existed. 14,15 Although most of the patients described in these studies as having Takayasu"s arteritis were young women, our case was a 20 year old man. There is insufficient evidence to link ankylosing spondylitis and Takayasu disease, but in the etiology of associated spine and joint involvement in Takayasu’s arteritis, autoimmunity and genetic factors may play a role. 1, 12, 13 Our case was the sixth of TA and AS who had a positive HLA-B27. This suggests that the association of these two diseases can occur in both HLA-B27 negative AS 8-11, 14 and HLA-B27 positive AS. The spondarthritis had been diagnosed first in our patient as in some of the previously reported cases 1, 8-11, 13. This may be due to more predomininant arthicular pain in pre-pulseless phase of Takayasu.1 Only in one case report, Takayasu arteritis was the first presentation and AS developed later. 16


    We believe that some common pathophysiological background, particularly a genetic mechanism and a certain immunopathogenic process may justify the combination of these two rare conditions and this association is probably not fortuitous.
  • Reference

    1. 1.Magaro, M., Altomonte, L., Mirone, L., Zoli, A., Corvino, G. Seronegative spondarthritis associated with Takayasu’s arteritis. Ann Rheum Dis. 1988; 47:595–597.
    2. 2.Calin, A., Fries, J. F. The striking prcvalence of ankylosing spondvlitis in "heailthy" w27 positive males and females. A controlled study. N ELngl J Med. 1975; 293: 835-9.
    3. 3.Bergfeldt, L., Edmag, 0. Rays J. HLA-B27 associated heart disease. Amt1 J Med. 1984; 74: 961-7.
    4. 4.Bulklcy, B. 1i., Roherts, W.C. Ankylosing spondylitis and aortic regurgitation. Description of the characteristic cardiovascular lesion from study of eight necropsy patients. Circulation. 1973; 18: 1014-27.
    5. 5.Tuckcr, C., Fowles, R. E., Calin, A., Popp, R. L. Aortitis in ankylosing spondvlitis: cirly detection of aortic root abnormalities with two dimensional echocardiography. Ain J C-ardiol. 1982; 49: 680-6.
    6. 6.Moll J, M. H., Wright, V. Ncw York clinical criteria for ankylosing spondylitis. A,1n1 Rheuwn Dis. 1973; 32: 354-63.
    7. 7.Sandring, H., Welin, G. Aortic arch syndrome with special reference to rheumatoid arteritis. Acta Med Scnd. 1%1; 170: 1-19.
    8. 8.Cowley, ML., Hicking, P., Wells, IP., Marshall, A.J. Takayasu’s disease and bilateral sacroiliitis. Clin Exp Rheumatol. 1987; 5:67–70.
    9. 9.Paloheimo, JA., Julkunen, H., Siltanen, P., Kajander, A. Takayasu’s arteritis and ankylosing spondylitis: report of four cases. Acta Med Scand. 1966; 179:77–85.
    10. 10.Ghozlan, R., Amor, B., Debarre, F. Association d"une maladie de Takayashu et d"une spondylarthrite ankylosante, un nouveau cas. Ann Med Interne. (Paris) 1977; 128: 959-63.
    11. 11.Hull R, G., Asherson, R. A., Rennie, J. A. N., Ankylosing spondylitis aind an aortic arch syndrorme. Br Heart J. 1984; 51: 663-5.
    12. 12.Sanders, ME., Fischbein, L.C. Hypertrophic osteoarthropathy with Takayasu’s arteritis. Clin Exp Rheumatol. 1987; 5:71–74.
    13. 13.Cherin, P., Blétry, O., Ziza, JM., Kieffer, E., Arfi, S., Estrade, G., Godeau, P. Association spondylarthrite ankylosante et maladie de Takayasu. Rev Rhum. 1990; 57:33–37.
    14. 14.Aoyagi, S., Akashi, H., Kawara, T., Ishihara, K., Tanaka, A., Kanaya, S., Koga, Y., Ishikawa. R. Aortic root replacement for Takayasu arteritis associated with ulcerative colitis and ankylosing spondylitis--report of a case. Jpn Circ J. 1998; 62 (1):64-8.
    15. 15.Hilàrio, M.O., Terreri, M.T., Prismich, G., Len, C., Kihara, EN., Goldenberg, J., Solé, D. Association of ankylosing spondylitis, Crohn"s disease and Takayasu"s arteritis in a child. Clin Exp Rheumatol. 1998; 16(1):92-4.
    16. 16.Wilson ,WA., Morgan, O., Bain, B., Taylor, JE. Takayasu’s arteritis: association with Stills disease in an adult. Arthritis Rheum. 1979; 22:684.
    17. 17.Schuetz, C.T., Aeberli, D., Oertle, S., Anderson, SE. Pseudoarthrosis and ankylosis of the vertebral spine without sacroiliitis associated with Takayasu"s arteritis: review of the association. Skeletal Radiol. 2002; 31(9):554-7.