Abstract
Lung complications have the highest mortality of any of the disease-related complications of systemic sclerosis following improvements in outcome of scleroderma renal crisis. Currently these complications are spread between lung fibrosis and pulmonary arterial hypertension, with a particularly poor survival when both of these lung processes occur concurrently. Since there is symptomatic overlap between the lung complications and other manifestations of SSc that lead to breathlessness and limited exercise capacity, the differential diagnosis of pulmonary complications is important. In addition, therapeutic options for lung complications have increased as evidence supporting current treatment strategies grows; new options for the treatment of pulmonary arterial hypertension emerge and potential use of antifibrotic agents that are now licensed for the treatment of idiopathic pulmonary fibrosis for other forms of lung fibrosis such as systemic sclerosis.
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References
Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66(7):940–4.
Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, Das C, Elliot CA, Johnson M, DeSoyza J, Torpy C, Goldsmith K, Hodgkins D, Hughes RJ, Pepke-Zaba J, Coghlan JG. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;179(2):151–7.
Derrett-Smith EC, Nihtyanova SI, Harvey J, Salama AD, Denton CP. Revisiting ANCA-associated vasculitis in systemic sclerosis: clinical, serological and immunogenetic factors. Rheumatology (Oxford). 2013;52(10):1824–31.
Wells AU, Steen V, Valentini G. Pulmonary complications: one of the most challenging complications of systemic sclerosis. Rheumatology (Oxford). 2009;48 Suppl 3:iii40–4.
Frauenfelder T, Winklehner A, Nguyen TD, Dobrota R, Baumueller S, Maurer B, Distler O. Screening for interstitial lung disease in systemic sclerosis: performance of high-resolution CT with limited number of slices: a prospective study. Ann Rheum Dis. 2014;73(12):2069–73.
Desai SR, Veeraraghavan S, Hansell DM, Nikolakopolou A, Goh NS, Nicholson AG, Colby TV, Denton CP, Black CM, du Bois RM, Wells AU. 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.
Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P, Haslam PL, Vassilakis DA, Black CM, du Bois RM. 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.
Goh NS, Desai SR, Anagnostopoulos C, Hansell DM, Hoyles RK, Sato H, Denton CP, Black CM, du Bois RM, Wells AU. Increased epithelial permeability in pulmonary fibrosis in relation to disease progression. Eur Respir J. 2011;38(1):184–90.
De Lauretis A, Sestini P, Pantelidis P, Hoyles R, Hansell DM, Goh NS, Zappala CJ, Visca D, Maher TM, Denton CP, Ong VH, Abraham DJ, Kelleher P, Hector L, Wells AU, Renzoni EA. Serum interleukin 6 is predictive of early functional decline and mortality in interstitial lung disease associated with systemic sclerosis. J Rheumatol. 2013;40(4):435–46.
Nihtyanova SI, Schreiber BE, Ong VH, Rosenberg D, Moinzadeh P, Coghlan JG, Wells AU, Denton CP. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol. 2014;66(6):1625–35.
Coghlan JG, Denton CP, Grünig E, Bonderman D, Distler O, Khanna D, Müller-Ladner U, Pope JE, Vonk MC, Doelberg M, Chadha-Boreham H, Heinzl H, Rosenberg DM, McLaughlin VV, Seibold JR, DETECT study group. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340–9.
Nihtyanova SI, Tang EC, Coghlan JG, Wells AU, Black CM, Denton CP. Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 2010;103(2):109–15.
Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, Corte TJ, Sander CR, Ratoff J, Devaraj A, Bozovic G, Denton CP, Black CM, du Bois RM, Wells AU. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177(11):1248–54.
Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I, Czirjak L, Clements P, Denton C, Farge D, Fligelstone K, Földvari I, Furst DE, Müller-Ladner U, Seibold J, Silver RM, Takehara K, Toth BG, Tyndall A, Valentini G, van den Hoogen F, Wigley F, Zulian F, Matucci-Cerinic M; EUSTAR Co-Authors. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009 May;68(5):620–8.
Volkmann ER, Saggar R, Khanna D, Torres B, Flora A, Yoder L, Clements PJ, Elashoff RM, Ross DJ, Agrawal H, Borazan N, Furst DE, Saggar R. Improved transplant-free survival in patients with systemic sclerosis-associated pulmonary hypertension and interstitial lung disease. Arthritis Rheumatol. 2014;66(7):1900–8.
Keir GJ, Maher TM, Hansell DM, Denton CP, Ong VH, Singh S, Wells AU, Renzoni EA. Severe interstitial lung disease in connective tissue disease: rituximab as rescue therapy. Eur Respir J. 2012;40(3):641–8.
Williams MH, Das C, Handler CE, Akram MR, Davar J, Denton CP, Smith CJ, Black CM, Coghlan JG. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart. 2006;92(7):926–32.
Pulido T, Adzerikho I, Channick RN, Delcroix M, Galiè N, Ghofrani HA, Jansa P, Jing ZC, Le Brun FO, Mehta S, Mittelholzer CM, Perchenet L, Sastry BK, Sitbon O, Souza R, Torbicki A, Zeng X, Rubin LJ, Simonneau G, SERAPHIN Investigators. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369(9):809–18.
Fox BD, Shimony A, Langleben D, Hirsch A, Rudski L, Schlesinger R, Eisenberg MJ, Joyal D, Hudson M, Boutet K, Serban A, Masetto A, Baron M. High prevalence of occult left heart disease in scleroderma-pulmonary hypertension. Eur Respir J. 2013;42(4):1083–91.
O’Callaghan DS, Dorfmuller P, Jaïs X, Mouthon L, Sitbon O, Simonneau G, Humbert M, Montani D. Pulmonary veno-occlusive disease: the bête noire of pulmonary hypertension in connective tissue diseases? Presse Med. 2011;40(1 Pt 2):e65–78.
Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Müller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumánovics G, Coleiro B, Feierl E, Szucs G, Von Mühlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kötter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809–15.
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Denton, C.P. (2017). Overview of Lung Involvement: Diagnosis, Differential Diagnosis and Monitoring. In: Varga, J., Denton, C., Wigley, F., Allanore, Y., Kuwana, M. (eds) Scleroderma. Springer, Cham. https://doi.org/10.1007/978-3-319-31407-5_23
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DOI: https://doi.org/10.1007/978-3-319-31407-5_23
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