Abstract
Purpose of Review
The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren’s syndrome.
Recent Findings
Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population. Interstitial pneumonia with autoimmune features (IPAF) is a newly described entity that encompasses interstitial lung disease in patients with clinical, serologic, or morphologic features suggestive of but not diagnostic of collagen vascular disease; these patients are thought to have better outcomes than idiopathic interstitial pneumonias.
Summary
Interstitial lung disease and pulmonary hypertension determine the prognosis in collagen vascular disease patients. IPAF is a new term to label patients with possible collagen vascular disease-related interstitial lung disease. Collagen vascular disease patients are at increased risk for various malignancies.
Similar content being viewed by others
References
Recently published papers of particular interest have been highlighted as: • Of importance
Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6):733–48.
Mueller-Mang C, Grosse C, Schmid K, et al. What every radiologist should know about idiopathic interstitial pneumonias. Radiographics. 2007;27(3):595–615.
Hansell DM, Bankier AA, MacMahon H, et al. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008;246(3):697–722.
Lynch DA. Lung disease related to collagen vascular disease. J Thorac Imaging. 2009;24(4):299–309.
Tansey D, Wells AU, Colby TV, et al. Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology. 2004;44(6):585–96.
Tanaka N, Kim JS, Newell JD, et al. Rheumatoid arthritis-related lung diseases: CT findings. Radiology. 2004;232(1):81–91.
Kim EA, Lee KS, Johkoh T et al. Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. Radiographics. 2002;22 Spec No:S151-65.
Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003;58(2):143–8.
Enomoto Y, Takemura T, Hagiwara E, et al. Features of usual interstitial pneumonia in patients with primary Sjögren’s syndrome compared with idiopathic pulmonary fibrosis. Respir Investig. 2014;52(4):227–35.
Song JW, Lee HK, Lee CK, et al. Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2013;30(2):103–12.
Kim EJ, Elicker BM, Maldonado F, et al. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J. 2010;35(6):1322–8.
Olson AL, Swigris JJ, Sprunger DB, et al. Rheumatoid arthritis-interstitial lung disease-associated mortality. Am J Respir Crit Care Med. 2011;183(3):372–8.
Gabbay E, Tarala R, Will R, et al. Interstitial lung disease in recent onset rheumatoid arthritis. Am J Respir Crit Care Med. 1997;156(2 Pt 1):528–35.
Lee HK, Kim DS, Yoo B, et al. Histopathologic pattern and clinical features of rheumatoid arthritis-associated interstitial lung disease. Chest. 2005;127(6):2019–27.
Aquino SL, Webb WR, Golden J. Bronchiolitis obliterans associated with rheumatoid arthritis: findings on HRCT and dynamic expiratory CT. J Comput Assist Tomogr. 1994;18(4):555–8.
Franquet T. High-resolution CT of lung disease related to collagen vascular disease. Radiol Clin N Am. 2001;39(6):1171–87.
D’Angelo WA, Fries JF, Masi AT, et al. Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med. 1969;46(3):428–40.
Desai SR, Veeraraghavan S, Hansell DM, et al. CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology. 2004;232(2):560–7.
Launay D, Remy-Jardin M, Michon-Pasturel U, et al. High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. J Rheumatol. 2006;33(9):1789–801.
Hunninghake GW, Fauci AS. Pulmonary involvement in the collagen vascular diseases. Am Rev Respir Dis. 1979;119(3):471–503.
Noël V, Lortholary O, Casassus P, et al. Risk factors and prognostic influence of infection in a single cohort of 87 adults with systemic lupus erythematosus. Ann Rheum Dis. 2001;60(12):1141–4.
Devaraj A, Wells AU, Hansell DM. Computed tomographic imaging in connective tissue diseases. Semin Respir Crit Care Med. 2007;28(4):389–97.
Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005;52(12):3792–800.
Bi H, Er B. “Unexplained” dyspnoea and shrinking lungs in systemic lupus erythematosus. Br Med J. 1965;1(5445):1273–7.
Schwarz MI. Pulmonary and cardiac manifestations of polymyositis-dermatomyositis. J Thorac Imaging. 1992;7(2):46–54.
Akira M, Hara H, Sakatani M. Interstitial lung disease in association with polymyositis-dermatomyositis: long-term follow-up CT evaluation in seven patients. Radiology. 1999;210(2):333–8.
Mino M, Noma S, Taguchi Y, et al. Pulmonary involvement in polymyositis and dermatomyositis: sequential evaluation with CT. AJR Am J Roentgenol. 1997;169(1):83–7.
Arakawa H, Yamada H, Kurihara Y, et al. Nonspecific interstitial pneumonia associated with polymyositis and dermatomyositis: serial high-resolution CT findings and functional correlation. Chest. 2003;123(4):1096–103.
• Debray MP, Borie R, Revel MP, et al. Interstitial lung disease in anti-synthetase syndrome: initial and follow-up CT findings. Eur J Radiol. 2015;84(3):516–23. This article describes the features of interstitial diseases and how it evolves in patients with anti-synthetase syndrome; the most frequently detected patterns are nonspecific interstitial pneumonia and organizing pneumonia
Koyama M, Johkoh T, Honda O, et al. Pulmonary involvement in primary Sjögren’s syndrome: spectrum of pulmonary abnormalities and computed tomography findings in 60 patients. J Thorac Imaging. 2001;16(4):290–6.
• Fischer A, Antoniou KM, Brown KK, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46(4):976–87. This is the most recent official ATS/ERS consensus statement on interstitial pneumonia with autoimmune features to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity
Assayag D, Kim EJ, Elicker BM, et al. Survival in interstitial pneumonia with features of autoimmune disease: a comparison of proposed criteria. Respir Med. 2015;109(10):1326–31.
Alhamad EH, Cal JG, AlBoukai AA, et al. Autoimmune symptoms in idiopathic pulmonary fibrosis: clinical significance. Clin Respir J. 2016;10(3):350–8.
Ahmad K, Barba T, Gamondes D, et al. Interstitial pneumonia with autoimmune features: clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. Respir Med. 2017;123:56–62.
Hoeper MM. Definition, classification, and epidemiology of pulmonary arterial hypertension. Semin Respir Crit Care Med. 2009;30(4):369–75.
Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D42–50.
Condliffe R, Kiely DG, Peacock AJ, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;179(2):151–7.
Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D34–41.
Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010;122(2):156–63.
Kawut SM, Taichman DB, Archer-Chicko CL, et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest. 2003;123(2):344–50.
Chung L, Liu J, Parsons L, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010;138(6):1383–94.
Hunzelmann N, Genth E, Krieg T, et al. The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology (Oxford). 2008;47(8):1185–92.
Mukerjee D, St George D, Coleiro B, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62(11):1088–93.
Min HK, Lee JH, Jung SM, et al. Pulmonary hypertension in systemic lupus erythematosus: an independent predictor of patient survival. Korean J Intern Med. 2015;30(2):232–41.
Dawson JK, Goodson NG, Graham DR, et al. Raised pulmonary artery pressures measured with Doppler echocardiography in rheumatoid arthritis patients. Rheumatology (Oxford). 2000;39(12):1320–5.
van Wolferen SA, Marcus JT, Boonstra A, et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J. 2007;28(10):1250–7.
Tincani A, Rebaioli CB, Taglietti M, et al. Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus. Rheumatology (Oxford). 2006;45(Suppl 4):iv8–13.
Wang SH, Chang YS, Liu CJ, et al. Incidence and risk analysis of aortic aneurysm and aortic dissection among patients with systemic lupus erythematosus: a nationwide population-based study in Taiwan. Lupus. 2014;23(7):665–71.
Chung WS, Lin CL, Chang SN, et al. Systemic lupus erythematosus increases the risks of deep vein thrombosis and pulmonary embolism: a nationwide cohort study. J Thromb Haemost. 2014;12(4):452–8.
Lambova S. Cardiac manifestations in systemic sclerosis. World J Cardiol. 2014;6(9):993–1005.
Ali H, Ng KR, Low AH. A qualitative systematic review of the prevalence of coronary artery disease in systemic sclerosis. Int J Rheum Dis. 2015;18(3):276–86.
• Mavrogeni S, Markousis-Mavrogenis G, Koutsogeorgopoulou L, et al. Cardiovascular magnetic resonance imaging: clinical implications in the evaluation of connective tissue diseases. J Inflamm Res. 2017;10:55–61. This article reviews the magnetic resonance imaging features of cardiovascular involvement in patients with collagen vascular diseases
Dankó K, Ponyi A, Constantin T, et al. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases. Medicine (Baltimore). 2004;83(1):35–42.
Shovman O, Tiosano S, Comaneshter D, et al. Aortic aneurysm associated with rheumatoid arthritis: a population-based cross-sectional study. Clin Rheumatol. 2016;35(11):2657–61.
Gyöngyösi M, Pokorny G, Jambrik Z, et al. Cardiac manifestations in primary Sjögren’s syndrome. Ann Rheum Dis. 1996;55(7):450–4.
Wechsler RJ, Steiner RM, Spirn PW, et al. The relationship of thoracic lymphadenopathy to pulmonary interstitial disease in diffuse and limited systemic sclerosis: CT findings. AJR Am J Roentgenol. 1996;167(1):101–4.
Wooten M. Systemic sclerosis and malignancy: a review of the literature. South Med J. 2008;101(1):59–62.
Bernatsky S, Ramsey-Goldman R, Labrecque J, et al. Cancer risk in systemic lupus: an updated international multi-centre cohort study. J Autoimmun. 2013;42:130–5.
Jeong YJ, Lee KS, Chung MP, et al. Amyloidosis and lymphoproliferative disease in Sjögren syndrome: thin-section computed tomography findings and histopathologic comparisons. J Comput Assist Tomogr. 2004;28(6):776–81.
Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med. 2002;165(12):1581–6.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of Interest
Drs. Hamza Jawad, Sebastian McWilliams, and Sanjeev Bhalla declare that they have no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Additional information
This article is part of the Topical Collection on Imaging
Rights and permissions
About this article
Cite this article
Jawad, H., McWilliams, S.R. & Bhalla, S. Cardiopulmonary Manifestations of Collagen Vascular Diseases. Curr Rheumatol Rep 19, 71 (2017). https://doi.org/10.1007/s11926-017-0697-x
Published:
DOI: https://doi.org/10.1007/s11926-017-0697-x