Ankylosing spondylitis is a chronic inflammatory condition that usually affects young men. Cardiac dysfunction and pulmonary disease are well-known and commonly reported extra-articular manifestation, associated with ankylosing spondylitis (AS). AS has also been reported to be specifically associated with aortitis, aortic valve diseases, conduction disturbances, cardiomyopathy and ischemic heart disease. The pulmonary manifestations of the disease include fibrosis of the upper lobes, interstitial lung disease, ventilatory impairment due to chest wall restriction, sleep apnea, and spontaneous pneumothorax. They are many reports detailing pathophysiology, hypothesized mechanisms leading to these derangements, and estimated prevalence of such findings in the AS populations. At this time, there are no clear guidelines regarding a stepwise approach to screen these patients for cardiovascular and pulmonary complications. 1. Cardiac Manifestations of Ankylosing Spondylitis Introduction Ankylosing spondylitis is a chronic and inflammatory condition, affecting the spine, sacroiliac, and peripheral joints. This entity most often affects young men and may lead to spinal vertebral fusion. Human leukocyte antigen (HLA)-B27 is present in the majority of patients with AS and is reported to contribute to the pathophysiologic manifestations of this condition [1]. It has been estimated that cardiac manifestations in patients with AS are found in 2–10% of patients. It was initially in the 1930s when aortitis found in a group of patients with spondylitis. It is widely accepted today that not only is aortic pathology linked to AS, but there is also risk for conduction defects, valvular regurgitation, and cardiomyopathy, associated with this entity [1]. This is especially important, given that in many patients, cardiac changes may begin prior to the onset of clinical symptoms [2, 3]. 1.1. Valvular Disease The presence of aortic root and valve disease in ankylosing spondylitis is related to the duration of the underlying disease. Aortic disease and aortic regurgitation may, however, predate the onset of any joint symptoms, and the presence of ankylosing spondylitis as an underlying cause may not be initially appreciated [1]. One of the first pathophysiologic descriptions of valvular disease in AS was put forward by Bulkley and Roberts, who studied autopsy findings in eight patients with AS. They noted aortic root dilatation along with fibrous proliferation along the intima [4]. Further examination demonstrated a cellular inflammatory process coupled with platelet aggregation
References
[1]
I. Moyssakis, E. Gialafos, V. A. Vassiliou et al., “Myocardial performance and aortic elasticity are impaired in patients with ankylosing spondylitis,” Scandinavian Journal of Rheumatology, vol. 38, no. 3, pp. 216–221, 2009.
[2]
A. Yildirir, S. Aksoyek, M. Calguneri, A. Oto, and S. Kes, “Echocardiographic evidence of cardiac involvement in ankylosing spondylitis,” Clinical Rheumatology, vol. 21, no. 2, pp. 129–134, 2002.
[3]
M. Caliskan, D. Erdogan, H. Gullu et al., “Impaired coronary microvascular and left ventricular diastolic functions in patients with ankylosing spondylitis,” Atherosclerosis, vol. 196, no. 1, pp. 306–312, 2008.
[4]
B. H. Bulkley and W. C. Roberts, “Ankylosing spondylitis and aortic regurgitation. Description of the characteristic cardiovascular lesion from study of eight necropsy patients,” Circulation, vol. 48, no. 5, pp. 1014–1027, 1973.
[5]
C. A. Roldan, J. Chavez, P. W. Wiest, C. R. Qualls, and M. H. Crawford, “Aortic root disease and valve disease associated with ankylosing spondylitis,” Journal of the American College of Cardiology, vol. 32, no. 5, pp. 1397–1404, 1998.
[6]
J. Ka？mierczak, M. Peregud-Pogorzelska, J. Biernawska, et al., “Cardiac arrhythmias and conduction disturbances in patients with ankylosing spondylitis,” Angiology, vol. 58, no. 6, pp. 751–756, 2008.
[7]
V. K. Dik, M. J. L. Peters, P. A. Dijkmans et al., “The relationship between disease-related characteristics and conduction disturbances in ankylosing spondylitis,” Scandinavian Journal of Rheumatology, vol. 39, no. 1, pp. 38–41, 2010.
[8]
A. Yildirir, S. Aksoyek, M. Calguneri et al., “QT dispersion as a predictor of arrhythmic events in patients with ankylosing spondylitis,” Rheumatology, vol. 39, no. 8, pp. 875–879, 2000.
[9]
E. Toussirot, M. Bahjaoui-Bouhaddi, J. C. Poncet et al., “Abnormal autonomic cardiovascular control in ankylosing spondylitis,” Annals of the Rheumatic Diseases, vol. 58, no. 8, pp. 481–487, 1999.
[10]
A. Yildirir, S. Aksoyek, M. Calguneri et al., “No evidence of cardiac autonomic involvement in ankylosing spondylitis, as assessed by heart rate variability,” Clinical Rheumatology, vol. 20, no. 3, pp. 185–188, 2001.
[11]
B. A. Gould, J. Turner, D. H. Keeling, P. Hickling, and A. J. Marshall, “Myocardial dysfunction in ankylosing spondylitis,” Annals of the Rheumatic Diseases, vol. 51, no. 2, pp. 227–232, 1992.
[12]
M. J. L. Peters, I. Visman, M. M. J. Nielen et al., “Ankylosing spondylitis: a risk factor for myocardial infarction?” Annals of the Rheumatic Diseases, vol. 69, no. 3, pp. 579–581, 2010.
[13]
H. Divecha, N. Sattar, A. Rumley, L. Cherry, G. D. O. Lowe, and R. Sturrock, “Cardiovascular risk parameters in men with ankylosing spondylitis in comparison with non-inflammatory control subjects: relevance of systemic inflammation,” Clinical Science, vol. 109, no. 2, pp. 171–176, 2005.
[14]
S. Heeneman and M. J. A. P. Daemen, “Cardiovascular risks in spondyloarthritides,” Current Opinion in Rheumatology, vol. 19, no. 4, pp. 358–362, 2007.
[15]
F. Brunner, A. Kunz, U. Weber, et al., “Ankylosing Spondylitis and Heart Abnormalities: do cardiac conduction disorders, valve regurgitation and diastolic dysfunction occur more often in male patients with diagnosed ankylosing spondylitis for over 15 years than in the normal population?” Clinical Rheumatology, vol. 25, pp. 24–29, 2005.
[16]
C. Dunham and F. Kautz, “Sondylarthritis Ankylopoietica, A Review and Report of Twenty Cases,” The American Journal of the Medical Sciences, vol. 201, pp. 232–250, 1941.
[17]
S. Baser, S. Cubukcu, S. Ozkurt, N. Sabir, B. Akdag, and E. Diri, “Pulmonary involvement starts in early stage ankylosing spondylitis,” Scandinavian Journal of Rheumatology, vol. 35, no. 4, pp. 325–327, 2006.
[18]
E. C. Rosenow, C. V. Strimlan, J. R. Muhm, and R. H. Ferguson, “Pleuropulmonary manifestations of ankylosing spondylitis,” Mayo Clinic Proceedings, vol. 52, no. 10, pp. 641–649, 1977.
[19]
I. P. Casserly, H. M. Fenlon, E. Breatnach, and S. M. Sant, “Lung findings on high-resolution computed tomography in idiopathic ankylosing spondylitis-correlation with clinical findings, pulmonary function testing and plain radiography,” British Journal of Rheumatology, vol. 36, no. 6, pp. 677–682, 1997.
[20]
P. D. Sampaio-Barros, E. M. F. P. Cerqueira, S. M. Rezende et al., “Pulmonary involvement in ankylosing spondylitis,” Clinical Rheumatology, vol. 26, no. 2, pp. 225–230, 2007.
[21]
K. Turetschek, W. Ebner, D. Fleischmann, et al., “Early pulmonary involvement in ankylosing spondylitis: assessment with Thin-section CT,” Clinical Radiology, vol. 33, pp. 632–636, 2000.
[22]
N. Kanathur and T. Lee-Chiong, “Pulmonary Manifestations of Ankylosing Spondylitis,” Clinics in Chest Medicine, vol. 31, no. 3, pp. 547–554, 2010.
[23]
W. M. Rumancik, H. Firooznia, M. S. Davis Jr., et al., “Fibrobullous disease of the upper lobes: an extraskeletal manifestation of ankylosing spondylitis,” Journal of Computed Tomography, vol. 8, no. 3, pp. 225–229, 1984.
[24]
A. K. Sil, “Lung changes in ankylosing spondylitis,” Chest, vol. 61, no. 4, pp. 406–407, 1972.
[25]
D. Davies, “Lung fibrosis in ankylosing spondylitis,” Thorax, vol. 27, no. 2, p. 262, 1972.
[26]
D. Thai, R. S. Ratani, S. Salama, and R. M. Steiner, “Upper lobe fibrocavitary disease in a patient with back pain and stiffness,” Chest, vol. 118, no. 6, pp. 1814–1816, 2000.
[27]
B. A. Scobie, “The lungs in ankylosing spondylitis,” British Medical Journal, vol. 4, pp. 492–493, 1971.
[28]
C. K. Tan, C. C. Lai, C. H. Chou, and P. O. R. Hsueh, “Mycobacterium celatum pulmonary infection mimicking pulmonary tuberculosis in a patient with ankylosing spondylitis,” International Journal of Infectious Diseases, vol. 13, no. 6, pp. e459–e462, 2009.
[29]
W. P. U. Kennedy, L. J. Milne, W. Blyth, and G. K. Crompton, “Two unusual organisms, Aspergillus terreus and Metschnikowia pulcherrima, associated with the lung disease of ankylosing spondylitis,” Thorax, vol. 27, no. 5, pp. 604–610, 1972.
[30]
H. H. Ho, M. C. Lin, K. H. Yu, C. M. Wang, Y. J. J. Wu, and J. I. Y. Chen, “Pulmonary tuberculosis and disease-related pulmonary apical fibrosis in ankylosing spondylitis,” Journal of Rheumatology, vol. 36, no. 2, pp. 355–360, 2009.
[31]
L. T. Tanoue, “Pulmonary involvement in collagen vascular disease: a review of the pulmonary manifestations of the Marfan syndrome, ankylosing spondylitis, Sj？gren's syndrome, and relapsing polychondritis,” Journal of Thoracic Imaging, vol. 7, no. 2, pp. 62–77, 1992.
[32]
A. A. Cohen, E. A. Natelson, R. E. Fechner, et al., “Fibrosing interstitial pneumonitis in ankylosing spondylitis,” Chest, vol. 59, no. 4, pp. 369–371, 1971.
[33]
A. S. Souza, N. L. Müller, E. Marchiori, L. V. Soares-Souza, and M. De Souza Rocha, “Pulmonary abnormalities in ankylosing spondilitis: inspiratory and expiratory high-resolution CT findings in 17 patients,” Journal of Thoracic Imaging, vol. 19, no. 4, pp. 259–263, 2004.
[34]
U. Dincer, E. Cakar, M. Z. Kiralp, E. Bozkanat, H. Kilac, and H. Dursun, “The pulmonary involvement in rheumatic diseases: pulmonary effects of ankylosing spondylitis and its impact on functionality and quality of life,” Tohoku Journal of Experimental Medicine, vol. 212, no. 4, pp. 423–430, 2007.
[35]
A. El Maghraoui, S. Chaouir, A. Abid et al., “Lung findings on thoracic high-resolution computed tomography in patients with ankylosing spondylitis. Correlations with disease duration, clinical findings and pulmonary function testing,” Clinical Rheumatology, vol. 23, no. 2, pp. 123–128, 2004.
[36]
C. C. Lee, S. H. Lee, I. J. Chang et al., “Spontaneous pneumothorax associated with ankylosing spondylitis,” Rheumatology, vol. 44, no. 12, pp. 1538–1541, 2005.
[37]
H. Kaneda, Y. Saito, M. Okamoto, T. Maniwa, K. I. Minami, and H. Imamura, “Bilaterally repeated spontaneous pneumothorax with ankylosing spondylitis,” General Thoracic and Cardiovascular Surgery, vol. 55, no. 6, pp. 266–269, 2007.
[38]
？. Solak, F. Fidan, ü. Dündar, et al., “The prevalence of obstructive sleep apnoea syndrome in ankylosing spondylitis patients,” Rheumatology, vol. 48, no. 4, pp. 433–435, 2009.
[39]
S. Garrett, T. Jenkinson, L. G. Kennedy, H. Whitelock, P. Gaisford, and A. Calin, “A new approach to defining disease status in ankylosing spondylitis: the bath ankylosing spondylitis disease activity index,” Journal of Rheumatology, vol. 21, no. 12, pp. 2286–2291, 1994.
[40]
N. Erb, D. Karokis, J. P. Delamere, M. J. Cushley, and G. D. Kitas, “Obstructive sleep apnoea as a cause of fatigue in ankylosing spondylitis,” Annals of the Rheumatic Diseases, vol. 62, no. 2, pp. 183–184, 2003.
[41]
A. Deodhar, J. Braun, R. D. Inman, et al., “Golimumab reduces sleep disturbance in patients with active ankylosing spondylitis: results from a randomized, placebo-controlled trial,” Arthritis Care & Research, vol. 62, no. 9, pp. 1266–1271, 2010.
[42]
M. Rudwaleit, K. Gooch, B. Michel, et al., “Adalimumab improves sleep and sleep quality in patients with active ankylosing spondylitis,” The Journal of Rheumatology, vol. 38, no. 1, pp. 79–86, 2011.
[43]
D. van der Heijde, A. Kivitz, M. H. Schiff et al., “Efficacy and safety of adalimumab in patients with ankylosing spondylitis: results of a multicenter, randomized, double-blind, placebo-controlled trial,” Arthritis and Rheumatism, vol. 54, no. 7, pp. 2136–2146, 2006.
[44]
R. D. Inman, J. C. Davis, D. Van Der Heijde et al., “Efficacy and safety of golimumab in patients with ankylosing spondylitis: results of a randomized, double-blind, placebo-controlled, phase III trial,” Arthritis and Rheumatism, vol. 58, no. 11, pp. 3402–3412, 2008.
