1. Department of Clinical Immunology, 2. Department of Pulmonary Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
Volume 29, Issue 3, September 2018
Sign in to download the Issue in PDF format.
34812741
Mediterr J Rheumatol 2018;29(3):140-7
Article Links
Interstitial lung disease in Systemic sclerosis: insights into pathogenesis and evolving therapies
References
- Nihtyanova S I, Tang E C, Coghlan J G, Wells A U, Black C M, Denton C P. Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM 2010;103:109-15. [https://doi.org/10.1093/qjmed/hcp174] [PMID: 19966313]
- Giacomelli R, Liakouli V, Berardicurti O, Ruscitti P, Di Benedetto P, Carubbi F, et al. Interstitial lung disease in systemic sclerosis: current and future treatment. Rheumatol Int 2017;37:853-63. [https://doi.org/10.1007/s00296-016-3636-7] [PMID: 28063071]
- Gasparyan A Y, Ayvazyan L, Blackmore H, Kitas G D. Writing a narrative biomedical review: considerations for authors, peer reviewers, and editors. Rheumatol Int 2011;31:1409-17. [https://doi.org/10.1007/s00296-011-1999-3] [PMID: 21800117]
- Rocha L F, Luppino Assad A P, Marangoni R G, Del Rio A P T, Marques-Neto J F, Sampaio-Barros PD. Systemic sclerosis and silica exposure: a rare association in a large Brazilian cohort. Rheumatol Int 2016;36:697-702. [https://doi.org/10.1007/s00296-015-3412-0] [PMID: 26759224]
- Tian R, Zhu Y, Yao J, Meng X, Wang J, Xie H, et al. NLRP3 participates in the regulation of EMT in bleomycin-induced pulmonary fibrosis. Exp Cell Res 2017;357:328-34. [https://doi.org/10.1016/j.yexcr.2017.05.028] PMID: 28591554
- Martinod K, Witsch T, Erpenbeck L, Savchenko A, Hayashi H, Cherpokova D, et al. Peptidylarginine deiminase 4 promotes age-related organ fibrosis. J Exp Med 2017;214:439-58. [https://doi.org/10.1084/jem.20160530] [PMID: 28031479]
- Henderson J, O’Reilly S. Inflammasome lights up in systemic sclerosis. Arthritis Res Ther 2017;19:205. [https://doi.org/10.1186/s13075-017-1420-z] [PMID: 28923085]
- Altorok N, Tsou P-S, Coit P, Khanna D, Sawalha A H. Genome-wide DNA methylation analysis in dermal fibroblasts from patients with diffuse and limited systemic sclerosis reveals common and subset-specific DNA methylation aberrancies. Ann Rheum Dis 2015;74:1612-20. [https://doi.org/10.1136/annrheumdis-2014-205303] [PMID: 24812288]
- Munger J S, Huang X, Kawakatsu H, Griffiths M J, Dalton S L, Wu J, et al. The integrin alpha v beta 6 binds and activates latent TGF beta 1: a mechanism for regulating pulmonary inflammation and fibrosis. Cell 1999;96:319-28. [PMID: 10025398]
- Carracedo S, Lu N, Popova SN, Jonsson R, Eckes B, Gullberg D. The fibroblast integrin alpha11beta1 is induced in a mechanosensitive manner involving activin A and regulates myofibroblast differentiation. J Biol Chem 2010;285:10434–45. [https://doi.org/10.1074/jbc.M109.078766] [PMID: 20129924]
- Olewicz-Gawlik A, Danczak-Pazdrowska A, Kuznar-Kaminska B, Gornowicz-Porowska J, Katulska K, Trzybulska D, et al. Interleukin-17 and interleukin-23: importance in the pathogenesis of lung impairment in patients with systemic sclerosis. Int J Rheum Dis 2014;17:664-70. [https://doi.org/10.1111/1756-185X.12290] [PMID: 24467649]
- Rolla G, Fusaro E, Nicola S, Bucca C, Peroni C, Parisi S, et al. Th-17 cytokines and interstitial lung involvement in systemic sclerosis. J Breath Res 2016;10:046013. [https://doi.org/10.1088/1752-7155/10/4/046013] [PMID: 27869103]
- Vandecasteele E, De Pauw M, De Keyser F, Decuman S, Deschepper E, Piette Y, et al. Six-minute walk test in systemic sclerosis: A systematic review and meta-analysis. Int J Cardiol 2016;212:265-73. [https://doi.org/10.1016/j.ijcard.2016.03.084] PMID: 27057932
- Betteridge Z E, Woodhead F, Lu H, Shaddick G, Bunn C C, Denton C P, et al. Brief Report: Anti-Eukaryotic Initiation Factor 2B Autoantibodies Are Associated With Interstitial Lung Disease in Patients With Systemic Sclerosis. Arthritis Rheumatol Hoboken NJ 2016;68:2778-83. [https://doi.org/10.1002/art.39755] [PMID: 27273608]
- Ishikawa N, Hattori N, Yokoyama A, Kohno N. Utility of KL-6/MUC1 in the clinical management of interstitial lung diseases. Respir Investig 2012;503-13. [https://doi.org/10.1016/j.resinv.2012.02.001] [PMID: 22554854]
- Schutyser E, Richmond A, Van Damme J. Involvement of CC chemokine ligand 18 (CCL18) in normal and pathological processes. J Leukoc Biol 2005;78:14-26. [https://doi.org/10.1189/jlb.1204712] [PMID: 15784687]
- Hoffmann-Vold A-M, Tennøe AH, Garen T, Midtvedt Ø, Abraityte A, Aaløkken TM, et al. High Level of Chemokine CCL18 Is Associated With Pulmonary Function Deterioration, Lung Fibrosis Progression, and Reduced Survival in Systemic Sclerosis. Chest 2016;150:299-306. [https://doi.org/10.1016/j.chest.2016.03.004] [PMID: 26997242]
- Kowal-Bielecka O, Chwiesko-Minarowska S, Bernatowicz P L, Allanore Y, Radstake T, Matucci-Cerinic M, et al. The arachidonate 5-lipoxygenase activating protein gene polymorphism is associated with the risk of scleroderma-related interstitial lung disease: a multicentre European Scleroderma Trials and Research group (EUSTAR) study. Rheumatologly (Oxford) 2017;56:844-[52. https://doi.org/10.1093/rheumatology/kew499] [PMID: 28160477]
- Mak A C Y, Tang P L F, Cleveland C, Smith M H, Kari Connolly M, Katsumoto T R, et al. Brief Report: Whole-Exome Sequencing for Identification of Potential Causal Variants for Diffuse Cutaneous Systemic Sclerosis. Arthritis Rheumatol Hoboken NJ 2016;68:2257-62. [https://doi.org/10.1002/art.39721] [PMID: 27111861]
- Salaffi F, Carotti M, Di Donato E, Di Carlo M, Ceccarelli L, Giuseppetti G. Computer-Aided Tomographic Analysis of Interstitial Lung Disease (ILD) in Patients with Systemic Sclerosis (SSc). Correlation with Pulmonary Physiologic Tests and Patient-Centred Measures of Perceived Dyspnea and Functional Disability. PLoS One 2016;11:e0149240. [https://doi.org/10.1371/journal.pone.0149240] [PMID: 26930658]
- Kim H J, Tashkin D P, Gjertson D W, Brown M S, Kleerup E, Chong S, et al. Transitions to different patterns of interstitial lung disease in scleroderma with and without treatment. Ann Rheum Dis 2016;75:1367-71. https://doi.org/10.1136/annrheumdis-2015-208929. [PMID: 26757749]
- Molberg Ø, Hoffmann-Vold A-M. Interstitial lung disease in systemic sclerosis: progress in screening and early diagnosis. Curr Opin Rheumatol 2016;28:613-8. [https://doi.org/10.1097/BOR.0000000000000323] [PMID: 27387267]
- Pinal-Fernandez I, Pineda-Sanchez V, Pallisa-Nuñez E, Simeon-Aznar C P, Selva-O’Callaghan A, Fonollosa-Pla V, et al. Fast 1.5 T chest MRI for the assessment of interstitial lung disease extent secondary to systemic sclerosis. Clin Rheumatol 2016;35:2339-45. [https://doi.org/10.1007/s10067-016-3267-0] [PMID: 27107755]
- Buda N, Piskunowicz M, Porzezińska M, Kosiak W, Zdrojewski Z. Lung Ultrasonography in the Evaluation of Interstitial Lung Disease in Systemic Connective Tissue Diseases: Criteria and Severity of Pulmonary Fibrosis - Analysis of 52 Patients. Ultraschall Med Stuttg Ger 1980 2016;37:379-85. [https://doi.org/10.1055/s-0041-110590] [PMID: 26713499]
- Wang Y, Gargani L, Barskova T, Furst DE, Cerinic M M. Usefulness of lung ultrasound B-lines in connective tissue disease-associated interstitial lung disease: a literature review. Arthritis Res Ther 2017;19:206. [https://doi.org/10.1055/s-0041-110590] [PMID: 26713499]
- Gigante A, Rossi Fanelli F, Lucci S, Barilaro G, Quarta S, Barbano B, et al. Lung ultrasound in systemic sclerosis: correlation with high-resolution computed tomography, pulmonary function tests and clinical variables of disease. Intern Emerg Med 2016;11:213-7. [https://doi.org/10.1007/s11739-015-1329-y] [PMID: 26494471]
- Tomiyama F, Watanabe R, Ishii T, Kamogawa Y, Fujita Y, Shirota Y, et al. High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis. Tohoku J Exp Med 2016;239:297-305. [https://doi.org/10.1620/tjem.239.297] [PMID: 27487743]
- Goh N S, Hoyles R K, Denton C P, Hansell D M, Renzoni E A, Maher T M, et al. Short-Term Pulmonary Function Trends Are Predictive of Mortality in Interstitial Lung Disease Associated With Systemic Sclerosis. Arthritis Rheumatol Hoboken NJ 2017;69:1670-8. [https://doi.org/10.1002/art.40130] [PMID: 28426895]
- Simeón-Aznar C P, Fonollosa-Plá V, Tolosa-Vilella C, Espinosa-Garriga G, Campillo-Grau M, Ramos-Casals M, et al. Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death. Medicine (Baltimore) 2015;94:e1728. [https://doi.org/10.1097/MD.0000000000001728] [PMID: 26512564]
- Khanna D, Tseng C-H, Farmani N, Steen V, Furst D E, Clements PJ, et al. Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung Study Placebo Group. Arthritis Rheum 2011;63:3078-85. [https://doi.org/10.1002/art.30467] [PMID: 21618205]
- Fischer A, du Bois R. Interstitial lung disease in connective tissue disorders. Lancet Lond Engl 2012;380:689-98. [https://doi.org/10.1016/S0140-6736(12)61079-4] [PMID: 22901890]
- Okamoto M, Fujimoto K, Sadohara J, Furuya K, Kaieda S, Miyamura T, et al. A retrospective cohort study of outcome in systemic sclerosis-associated interstitial lung disease. Respir Investig 2016;54:445-53. [https://doi.org/10.1016/j.resinv.2016.05.004] [PMID: 27886856]
- Tashkin D P, Elashoff R, Clements P J, Goldin J, Roth M D, Furst D E, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006;354:2655-66. [https://doi.org/10.1056/NEJMoa055120] [PMID: 16790698]
- Hoyles R K, Ellis R W, Wellsbury J, Lees B, Newlands P, Goh N S L, et al. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 2006;54:3962-70. [https://doi.org/10.1002/art.22204] [PMID: 17133610]
- Nadashkevich O, Davis P, Fritzler M, Kovalenko W. A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol 2006;25:205-12. [https://doi.org/10.1007/s10067-005-1157-y] [PMID: 16228107]
- Tashkin D P, Elashoff R, Clements P J, Roth M D, Furst D E, Silver R M, 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;176:1026-34. [https://doi.org/10.1164/rccm.200702-326OC] [PMID: 17717203]
- Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 2009;68:620-8. [https://doi.org/10.1136/ard.2008.096677] [PMID: 19147617]
- Tashkin D P, Roth M D, Clements P J, Furst D E, Khanna D, Kleerup E C, 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;4:708-19. [https://doi.org/10.1016/S2213-2600(16)30152-7] [PMID: 27469583]
- Paone C, Chiarolanza I, Cuomo G, Ruocco L, Vettori S, Menegozzo M, et al. Twelve-month azathioprine as maintenance therapy in early diffuse systemic sclerosis patients treated for 1-year with low dose cyclophosphamide pulse therapy. Clin Exp Rheumatol 2007;25:613-6. [PMID: 17888219]
- Iudici M, Cuomo G, Vettori S, Bocchino M, Sanduzzi Zamparelli A, Cappabianca S, et al. Low-dose pulse cyclophosphamide in interstitial lung disease associated with systemic sclerosis (SSc-ILD): efficacy of maintenance immunosuppression in responders and non-responders. Semin Arthritis Rheum 2015;44:437-44. [https://doi.org/10.1016/j.semarthrit.2014.09.003] [PMID: 25282395]
- Domiciano D S, Bonfá E, Borges C T L, Kairalla R A, Capelozzi V L, Parra E, et al. A long-term prospective randomized controlled study of non-specific interstitial pneumonia (NSIP) treatment in scleroderma. Clin Rheumatol 2011;30:223-9. [https://doi.org/10.1007/s10067-010-1493-4] [PMID: 20544245]
- Daoussis D, Liossis S-N C, Tsamandas A C, Kalogeropoulou C, Kazantzi A, Sirinian C, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford) 2010;49:271-80. [https://doi.org/10.1093/rheumatology/kep093] [PMID: 19447770]
- Jordan S, Distler J H W, Maurer B, Huscher D, van Laar J M, Allanore Y, et al. Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group. Ann Rheum Dis 2015;74:1188-94. [https://doi.org/10.1136/annrheumdis-2013-204522] [PMID: 24442885]
- Saunders P, Tsipouri V, Keir G J, Ashby D, Flather M D, Parfrey H, et al. Rituximab versus cyclophosphamide for the treatment of connective tissue disease-associated interstitial lung disease (RECITAL): study protocol for a randomised controlled trial. Trials 2017;18:275. [https://doi.org/10.1186/s13063-017-2016-2] [PMID: 28619061]
- Burt R K, Shah S J, Dill K, Grant T, Gheorghiade M, Schroeder J, et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet Lond Engl 2011;378:498-506. [https://doi.org/10.1016/S0140-6736(11)60982-3] [PMID: 21777972]
- van Laar J M, Farge D, Sont J K, Naraghi K, Marjanovic Z, Larghero J, et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 2014;311:2490-8. [https://doi.org/10.1001/jama.2014.6368] [PMID: 25058083]
- Sullivan K M, Goldmuntz E A, Keyes-Elstein L, McSweeney P A, Pinckney A, Welch B, et al. Myeloablative Autologous Stem-Cell Transplantation for Severe Scleroderma. N Engl J Med 2018;378:35-47. [PMID: 29298160]
- Distler O, Brown K K, Distler J H W, Assassi S, Maher T M, Cottin V, et al. Design of a randomised, placebo-controlled clinical trial of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCISTM). Clin Exp Rheumatol 2017;35 Suppl 106:75-81. [PMID: 28664834]
- Fraticelli P, Gabrielli B, Pomponio G, Valentini G, Bosello S, Riboldi P, et al. Low-dose oral imatinib in the treatment of systemic sclerosis interstitial lung disease unresponsive to cyclophosphamide: a phase II pilot study. Arthritis Res Ther 2014;16:R144. [https://doi.org/10.1186/ar4606] [PMID: 25007944]
- Sabnani I, Zucker M J, Rosenstein E D, Baran D A, Arroyo L H, Tsang P, et al. A novel therapeutic approach to the treatment of scleroderma-associated pulmonary complications: safety and efficacy of combination therapy with imatinib and cyclophosphamide. Rheumatol Oxf Engl 2009;48:49-52. [https://doi.org/10.1093/rheumatology/ken369] [PMID: 18815156]
- Seibold J R, Denton C P, Furst D E, Guillevin L, Rubin L J, Wells A, et al. Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis. Arthritis Rheum 2010;62:2101-8. [https://doi.org/10.1002/art.27466] [PMID: 20506355]
- Launay D, Savale L, Berezne A, Le Pavec J, Hachulla E, Mouthon L, et al. Lung and heart-lung transplantation for systemic sclerosis patients. A monocentric experience of 13 patients, review of the literature and position paper of a multidisciplinary Working Group. Presse Medicale Paris Fr 1983 2014;43:e345-63. [https://doi.org/10.1016/j.lpm.2014.01.020] [PMID: 25027464]
![](images/delimiter.png)