Disease staging and sub setting of interstitial lung disease associated with systemic sclerosis: impact on therapy

References

• Chifflot H, Fautrel B, Sordet C, et al. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum. 2008;37(4):223–235.
   PubMed Web of Science ®Google Scholar
• Barnes J, Mayes MD. Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr Opin Rheumatol. 2012;24(2):165–170.
   PubMed Web of Science ®Google Scholar
• Society AT, Society ER. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the idiopathic interstitial pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277–304.
   PubMedGoogle Scholar
• Walker UA, Tyndall A, Czirják L, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2007;66(6):754–763.
   PubMed Web of Science ®Google Scholar
• Tyndall AJ, Bannert B, Vonk M, et al. 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):1809–1815.
   PubMed Web of Science ®Google Scholar
• Briggs DC, Vaughan RW, Welsh KI, et al. Immunogenetic prediction of pulmonary fibrosis in systemic sclerosis. Lancet. 1991;338(8768):661–662.
   PubMed Web of Science ®Google Scholar
• Reveille JD, Solomon DH. Guidelines ACoRAHCoIT. Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies. Arthritis Rheum. 2003;49(3):399–412.
   PubMedGoogle Scholar
• Gilchrist FC, Bunn C, Foley PJ, et al. Class II HLA associations with autoantibodies in scleroderma: a highly significant role for HLA-DP. Genes Immun. 2001;2(2):76–81.
   PubMed Web of Science ®Google Scholar
• 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):1581–1586.
   PubMed Web of Science ®Google Scholar
• 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):560–567.
   PubMed Web of Science ®Google Scholar
• Wells AU. High-resolution computed tomography and scleroderma lung disease. Rheumatology (Oxford). 2008;47(Suppl 5):v59–v61.
   PubMedGoogle Scholar
• Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177(11):1248–1254.
   PubMed Web of Science ®Google Scholar
• Wells AU, Hansell DM, Corrin B, et al. High resolution computed tomography as a predictor of lung histology in systemic sclerosis. Thorax. 1992;47(9):738–742.
   PubMed Web of Science ®Google Scholar
• Remy-Jardin M, Giraud F, Remy J, et al. Importance of ground-glass attenuation in chronic diffuse infiltrative lung disease: pathologic-CT correlation. Radiology. 1993;189(3):693–698.
   PubMed Web of Science ®Google Scholar
• Silver RM, Miller KS, Kinsella MB, et al. Evaluation and management of scleroderma lung disease using bronchoalveolar lavage. Am J Med. 1990;88(5):470–476.
   PubMed Web of Science ®Google Scholar
• Witt C, Borges AC, John M, et al. Pulmonary involvement in diffuse cutaneous systemic sclerosis: broncheoalveolar fluid granulocytosis predicts progression of fibrosing alveolitis. Ann Rheum Dis. 1999;58(10):635–640.
   PubMed Web of Science ®Google Scholar
• Wells AU, Hansell DM, Haslam PL, et al. Bronchoalveolar lavage cellularity: lone cryptogenic fibrosing alveolitis compared with the fibrosing alveolitis of systemic sclerosis. Am J Respir Crit Care Med. 1998;157(5 Pt 1):1474–1482.
   PubMed Web of Science ®Google Scholar
• Goh NS, Veeraraghavan S, Desai SR, et al. Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum. 2007;56(6):2005–2012.
   PubMed Web of Science ®Google Scholar
• Strange C, Bolster MB, Roth MD, et al. Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med. 2008;177(1):91–98.
   PubMed Web of Science ®Google Scholar
• Peters-Golden M, Wise RA, Hochberg MC, et al. Carbon monoxide diffusing capacity as predictor of outcome in systemic sclerosis. Am J Med. 1984;77(6):1027–1034.
   PubMed Web of Science ®Google Scholar
• Morgan C, Knight C, Lunt M, et al. Predictors of end stage lung disease in a cohort of patients with scleroderma. Ann Rheum Dis. 2003;62(2):146–150.
   PubMed Web of Science ®Google Scholar
• Steen VD, Conte C, Owens GR, et al. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum. 1994;37(9):1283–1289.
   PubMedGoogle Scholar
• Wells AU, Hansell DM, Rubens MB, et al. Fibrosing alveolitis in systemic sclerosis: indices of lung function in relation to extent of disease on computed tomography. Arthritis Rheum. 1997;40(7):1229–1236.
   PubMedGoogle Scholar
• Wells AU, Denton CP. Interstitial lung disease in connective tissue disease–mechanisms and management. Nat Rev Rheumatol. 2014;10(12):728–739.
   PubMed Web of Science ®Google Scholar
• Latsi PI, Wells AU. Evaluation and management of alveolitis and interstitial lung disease in scleroderma. Curr Opin Rheumatol. 2003;15(6):748–755.
   PubMed Web of Science ®Google Scholar
• Antoniou KM, Margaritopoulos GA, Goh NS, et al. Combined pulmonary fibrosis and emphysema in scleroderma-related lung disease has a major confounding effect on lung physiology and screening for pulmonary hypertension. Arthritis Rheumatol. 2016;68(4):1004–1012.
   PubMed Web of Science ®Google Scholar
• Khanna D, Nagaraja V, Tseng CH, et al. Predictors of lung function decline in scleroderma-related interstitial lung disease based on high-resolution computed tomography: implications for cohort enrichment in systemic sclerosis-associated interstitial lung disease trials. Arthritis Res Ther. 2015;17:372.
   PubMed Web of Science ®Google Scholar
• Moore OA, Goh N, Corte T, et al. Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford). 2013;52(1):155–160.
   PubMed Web of Science ®Google Scholar
• MacGregor AJ, Canavan R, Knight C, et al. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford). 2001;40(4):453–459.
   PubMed Web of Science ®Google Scholar
• 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):1088–1093.
   PubMed Web of Science ®Google Scholar
• Hughes JM, Pride NB. In defence of the carbon monoxide transfer coefficient Kco (TL/VA). Eur Respir J. 2001;17(2):168–174.
   PubMed Web of Science ®Google Scholar
• Donato L, Giovanna Elisiana C, Giuseppe G, et al. Utility of FVC/DLCO ratio to stratify the risk of mortality in unselected subjects with pulmonary hypertension. Intern Emerg Med. 2017;12(3):319–326.
   PubMed Web of Science ®Google Scholar
• Coghlan JG, Denton CP, Grünig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340–1349.
   PubMed Web of Science ®Google Scholar
• Latsi PI, du Bois RM, Nicholson AG, et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med. 2003;168(5):531–537.
   PubMed Web of Science ®Google Scholar
• Collard HR, King TE, Bartelson BB, et al. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2003;168(5):538–542.
   PubMed Web of Science ®Google Scholar
• Flaherty KR, Mumford JA, Murray S, et al. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003;168(5):543–548.
   PubMed Web of Science ®Google Scholar
• Moore OA, Proudman SM, Goh N, et al. Quantifying change in pulmonary function as a prognostic marker in systemic sclerosis-related interstitial lung disease. Clin Exp Rheumatol. 2015;33(4 Suppl 91):S111–S116.
   PubMed Web of Science ®Google Scholar
• Goh NS, Hoyles RK, Denton CP, et al. Short term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol. 2017 Aug;69(8):1670–1678.
   PubMedGoogle Scholar
• Russell AM, Adamali H, Molyneaux PL, et al. Daily home spirometry: an effective tool for detecting progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2016;194(8):989–997.
   PubMed Web of Science ®Google Scholar
• Kim HJ, Tashkin DP, Gjertson DW, et al. Transitions to different patterns of interstitial lung disease in scleroderma with and without treatment. Ann Rheum Dis. 2016;75(7):1367–1371.
   PubMed Web of Science ®Google Scholar
• van Bon L, Affandi AJ, Broen J, et al. Proteome-wide analysis and CXCL4 as a biomarker in systemic sclerosis. N Engl J Med. 2014;370(5):433–443.
   PubMed Web of Science ®Google Scholar
• Volkmann ER, Tashkin DP, Roth MD, et al. Changes in plasma CXCL4 levels are associated with improvements in lung function in patients receiving immunosuppressive therapy for systemic sclerosis-related interstitial lung disease. Arthritis Res Ther. 2016;18(1):305.
   PubMed Web of Science ®Google Scholar
• Winstone TA, Assayag D, Wilcox PG, et al. Predictors of mortality and progression in scleroderma-associated interstitial lung disease: a systematic review. Chest. 2014;146(2):422–436.
   PubMed Web of Science ®Google Scholar
• Lopes AJ, Capone D, Mogami R, et al. Systemic sclerosis-associated interstitial pneumonia: evaluation of pulmonary function over a five-year period. J Bras Pneumol. 2011;37(2):144–151.
   PubMed Web of Science ®Google Scholar
• Roth MD, Tseng CH, Clements PJ, et al. Predicting treatment outcomes and responder subsets in scleroderma-related interstitial lung disease. Arthritis Rheum. 2011;63(9):2797–2808.
   PubMed Web of Science ®Google Scholar
• Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66(7):940–944.
   PubMed Web of Science ®Google Scholar
• Fertig N, Domsic RT, Rodriguez-Reyna T, et al. Anti-U11/U12 RNP antibodies in systemic sclerosis: a new serologic marker associated with pulmonary fibrosis. Arthritis Rheum. 2009;61(7):958–965.
   PubMedGoogle Scholar
• Launay D, Humbert M, Berezne A, et al. Clinical characteristics and survival in systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease. Chest. 2011;140(4):1016–1024.
   PubMed Web of Science ®Google Scholar
• De Santis M, Bosello SL, Peluso G, et al. Bronchoalveolar lavage fluid and progression of scleroderma interstitial lung disease. Clin Respir J. 2012;6(1):9–17.
   PubMed Web of Science ®Google Scholar
• Al-Dhaher FF, Pope JE, Ouimet JM. Determinants of morbidity and mortality of systemic sclerosis in Canada. Semin Arthritis Rheum. 2010;39(4):269–277.
   PubMed Web of Science ®Google Scholar
• 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):1789–1801.
   PubMed Web of Science ®Google Scholar
• du Bois RM, Weycker D, Albera C, et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184(4):459–466.
   PubMed Web of Science ®Google Scholar
• Wells AU, Desai SR, Rubens MB, et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med. 2003;167(7):962–969.
   PubMed Web of Science ®Google Scholar
• Steen VD, Medsger TA. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum. 1998;41(9):1613–1619.
   PubMed Web of Science ®Google Scholar
• DeMarco PJ, Weisman MH, Seibold JR, et al. Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial. Arthritis Rheum. 2002;46(11):2983–2989.
   PubMed Web of Science ®Google Scholar
• White B, Moore WC, Wigley FM, et al. Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med. 2000;132(12):947–954.
   PubMed Web of Science ®Google Scholar
• Nannini C, West CP, Erwin PJ, et al. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther. 2008;10(5):R124.
   PubMed Web of Science ®Google Scholar
• Poormoghim H, Moradi Lakeh M, Mohammadipour M, et al. Cyclophosphamide for scleroderma lung disease: a systematic review and meta-analysis. Rheumatol Int. 2012;32(8):2431–2444.
   PubMed Web of Science ®Google Scholar
• Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354(25):2655–2666.
   PubMed Web of Science ®Google Scholar
• Haubitz M, Schellong S, Göbel U, et al. Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmic antibody-associated vasculitis and renal involvement: a prospective, randomized study. Arthritis Rheum. 1998;41(10):1835–1844.
   PubMedGoogle Scholar
• Tashkin DP, Elashoff R, Clements PJ, et al. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med. 2007;176(10):1026–1034.
   PubMed Web of Science ®Google Scholar
• Nihtyanova SI, Brough GM, Black CM, et al. Mycophenolate mofetil in diffuse cutaneous systemic sclerosis–a retrospective analysis. Rheumatology (Oxford). 2007;46(3):442–445.
   PubMed Web of Science ®Google Scholar
• Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708–719.
   PubMed Web of Science ®Google Scholar
• Volkmann ER, Tashkin DP, Li N, et al. Mycophenolate versus placebo for systemic sclerosis-related interstitial lung disease: an analysis of scleroderma lung studies I and II. Arthritis Rheumatol. 2017;69:1451–1460.
   PubMed Web of Science ®Google Scholar
• Müller-Ladner U, Tyndall A, Czirjak L, et al. Ten years EULAR Scleroderma Research and Trials (EUSTAR): what has been achieved? Ann Rheum Dis. 2014;73(2):324–327.
   PubMed Web of Science ®Google Scholar
• Jenkins RG, Simpson JK, Saini G, et al. Longitudinal change in collagen degradation biomarkers in idiopathic pulmonary fibrosis: an analysis from the prospective, multicentre PROFILE study. Lancet Respir Med. 2015;3(6):462–472.
   PubMed Web of Science ®Google Scholar
• Hant FN, Ludwicka-Bradley A, Wang HJ, et al. Surfactant protein D and KL-6 as serum biomarkers of interstitial lung disease in patients with scleroderma. J Rheumatol. 2009;36(4):773–780.
   PubMed Web of Science ®Google Scholar
• Bonella F, Volpe A, Caramaschi P, et al. Surfactant protein D and KL-6 serum levels in systemic sclerosis: correlation with lung and systemic involvement. Sarcoidosis Vasc Diffuse Lung Dis. 2011;28(1):27–33.
   PubMed Web of Science ®Google Scholar
• Richeldi L, Du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–2082.
   PubMed Web of Science ®Google Scholar
• King TE, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–2092.
   PubMed Web of Science ®Google Scholar
• Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–1769.
   PubMed Web of Science ®Google Scholar
• Khanna D, Albera C, Fischer A, et al. An open-label, phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial. J Rheumatol. 2016;43(9):1672–1679.
   PubMed Web of Science ®Google Scholar
• Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford). 2010;49(2):271–280.
   PubMed Web of Science ®Google Scholar
• Daoussis D, Liossis SN, Tsamandas AC, et al. Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis. Clin Exp Rheumatol. 2012;30(2 Suppl 71):S17–S22.
   PubMed Web of Science ®Google Scholar
• Daoussis D, Melissaropoulos K, Sakellaropoulos G, et al. A multicenter, open-label, comparative study of B-cell depletion therapy with Rituximab for systemic sclerosis-associated interstitial lung disease. Semin Arthritis Rheum. 2017;46(5):625–631.
   PubMed Web of Science ®Google Scholar