Systemic sclerosis-associated interstitial lung disease: review of the literature
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Keywords

interstitial lung disease, systemic sclerosis, pulmonary fibrosis, antibodies

How to Cite

[1]
Dacheva, R. and Monov, S. 2020. Systemic sclerosis-associated interstitial lung disease: review of the literature. Revmatologiia (Bulgaria). 28, 4 (Dec. 2020), 59-71. DOI:https://doi.org/10.35465/28.4.2020.pp59-71.

Abstract

Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive sclerosis, affecting the skin and the internal organs, vasculopathy, and immune dysregulation. Interstitial pneumonias are a group of diffuse parenchymal lung diseases (DPLDs), or also called interstitial lung diseases (ILDs). ILD is a leading complication of SSc and is a leading cause of mortality in patients with SSc. The pathogenesis of lung injury involves three major processes - persistent endothelial cell damage, immune system activation, and fibroblast activation leading to the accumulation of extracellular matrix and tissue damage. A comprehensive approach is required to properly diagnose and to select appropriate therapy.

https://doi.org/10.35465/28.4.2020.pp59-71
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References

    1. Abraham DJ, Varga J. 2005. Scleroderma: from cell and molecular mechanism to disease model. Trends Immunol. 26:587-595
    2. Jogannes WJ Bijlsma, Jose Antinio P da Silva, Eric Hachulla et al., Textbook on rheumatic diseases, 2012, 546-567
    3.Abraham, DJ, T. Krieg, J. Distler et al. Overview of pathogenesis of systemic sclerosis, Rheumatology Oxford, 2009; 48, Suppl. 3: iii3-7
    4. Katsumoto TR, Whitfield ML, Connolly MK, The pathogenesis of systemic sclerosis, Annu Rev Pathol, 2011, 6:509-37
    5. Eric Hachulla, Laszlo Czirjak, Textbook on Systemic Sclerosis, EULAR 2013, 53-59
    6/ Bordon A., M.Dueymes, Y. Levy, The binding of some human antiendothelial cell antibodies induces endothelial cell apoptosis, J.Clin Invest, 1998 May 15, 101(10): 2029-2035
    7. American Thoracic Society/European Respiratory Society. International multidisciplinary consensus classification of idiopathic interstitial pneumonias, Am J Respir Crit Care Med 2002; 165 (2) 277-304
    8. An official American Thoracic Society/European Respiratory Society Statement: Update of the International multidisciplinary consensus classification of the idiopathic interstial pneumonias. Am J Respir Crit Care Med 2013; 188 (6): 733-748.
    9. Katerina M. Antoniou, George A. Margaritopoulos, Sara Tomassetti et al. Interstitial lung disease, European Respiratory Review 2014 23:40-54
    10. Daniel Simoes Oliveira, Jose de Arimatela Araujo Filho, Antonio Fernando Lins Paica et al. Idiopathic interstitial pneumonias: review of the latest American Thoracic Society/European Respiratory Society classification, Radiol Bras 2018, Sep-Oct; 51(5): 321-327
    11. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007; 66(7):940-944.
    12. Honda, N., M. Jinnin, I. Kajihara et al., TGF-beta-mediated downregulation ot microRNA-196a contributes to the constitutive upregulated type I collagen expression in scleroderma dermal fibroblasts. J Immunol 2012; 188:3323-3331
    13. Susanna Cappelli, Silvia Bellando Randone, Gianna Camiciottoli et al. Interstitial Lung Disease in Systemiv Sclerosis: where do we stand, European Respiratory Review, 2015, 24:411-419
    14. John Varga, David Abraham, Systemic sclerosis: a prototypic multisystem fibrotic disorder, The Journal of Clinical Investigation, 117, 557-567
    15. Mehdi Mirsaeidi, Pamela Barletta, Marilyn K. Glassberg, Systemic Sclerosis Associated Interstitial Lung Disease: New Direction in Disease Management, Frontiers in Medicine (Lausanne), 2019:6:248
    16. Park JH, Kim DS, Park IN et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes, Am J Respir Crit Care Med, 2007 Apr 1; 175 (7): 705-11
    17. Ganesh Raghu, Harold R. Collard, Jim J Egan et al. An official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management, Am J Respir Crit Care Med, 2011 Mar 15; 186(6): 788-824
    18. Solomon JJ, Chartrand S, Fischer A., Current approach to connective tissue disease-associated interstitial lung disease, Curr Opin Pulm Med, 2014 Sep 20(5): 449-56
    19. Apostolos Perelas, Richard M Silver, Andrea V Arrossi, et al. Systemic sclerosis-associated interstitial lung dissease, Lancet Respir Med 2020, Volume 8, Issue 3, 304-320, https://doi.org/10.1016/S2213-2600(19)30480-1
    20.Steen VD, Owens GR, Fino GJ et al. Pulmonary Involvement in systemic sclerosis (scleroderma). Arthritis Rheum, 1985 Jul;28(7):759-67
    21. Assassi S, Sharif R, Lasky RE et al. Predictors of interstitial lung disease in early systemic sclerosis: a prospective longitudinal study of the GENISOS cohort. Arthritis Res Ther 12, R166 (2010) https://doi.org/10.1186/ar3125
    22. Ysamat Marfa R, Benito YsamatA., Espejo Perez S., et al. Lung disease associated with connective tissue disease. Radiology 2013;55:107-117
    23. 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; 69:1809-15
    24. Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system, American Journal of Respiratory and Critical Care Medicine, 2008; 177(11); 1248-54
    25. Jeong-Hwa Hwang, Shigeki Misumi, Hakan Sahin, et al. Computed Tomographic Features of Idiopatic Fibrosing Interstitial Pneumonia: Comparison With Pulmonary Fibrosis Related to Collagen Vascular Disease; Journal of Computer Asisted Tomography. 33(3):410-415
    26. Joo Hun Park, Dong Soon Kim, I-Nae Park, et al. Prognosis of Fibrotic Interstitial Pneumonia, American Journal of Respiratory and Critical Care Medicinie, Vol 175, No 7
    27. Kim EA, Lee KS, Johkoh T., et al. Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings, Radiographics, 2003 Sep-Oct; 23(5):1340
    28. J Fujita, T Yoshinouchi, Y Ohtsuki, Non-specific interstitial pneumonia as pulmonary involvement of systemic sclerosis, Ann Rheum Dis 2001; 60:281–28
    29. Katznestein AL., Fiorelli RF., Nonspecific interstitial pneumonia/fibrosis: histologic features and clinical significance, Am J Surg Pathol, 1994;18:136-147
    30. Seth J Kligerman, Steve Groshong, Kevin K. Brown, et al. Nonspecific Interstitial Pneumonia: Radiologic, Clinical, and Parhologic Consideration, Jan 1 2009, vol 29, no 1
    31. Solomon JJ, Olson AL, Fischer A. et al. Scleroderma lung disease, Eur Respir Rev, 2013 Mar 1;22(127):6-19
    32. Steen, V.D., Autoantibodies in Systemic Sclerosis, Seminars in Arthritis and Rhematism, 2005, 35(1), 35-42
    33. Vasquez-Abad D, Wallace S, Senecal JL et al. Anticentromere autoantibodies. Evaluation of an ELISA using recombinant fusion protein CENT-B as antigen, Arhtritis Rheum., 1994;37:248-252
    34. Sato S., Fujimoto M, Inh H, et al. Antibodies to centromere and centriole in scleroderma spectrum disorders, Dermatology, 1994; 189:23-26
    35. Clegg DO, Williams HJ, Singer JZ et al. Early undifferentiated connective tissue disease. II. The frequency of circulation antinuclear antibodies in patients with early rheumatic disease, J Rheumatol., 1991;18:1340-26
    36. Kane GC, Varga J, Conant EF, et al. Lung involvement in systemic sclerosis (scleroderma): relation to classification based on extent of skin involvement or autoantibody status, Respir Med, 1996 Apr; 90(4): 223-30
    37. Steen VD., Powell DL, Medsger TA., Jr Clinical correlations and prognosis based on serum autoantibodies in patients with progressive systemic sclerosis. Arthritis Rheum. 1988; 31:196-203
    38. Arnett FC, Reveille JD, Goldstein R et al. Autoantibodies to fibrillarin in systemic sclerosis (scleroderma): an immunogenetic, serologic and clinical analysis. Arthritis Rheum. 1996;39:1151-1160
    39. Kuwana M, Kaburaki J, Okano Y, et al. Clinical and prognostic associations based on serum antinuclear antibodies in Japanese patients with systemic sclerosis, Arhtritis Rheum. 1994;37:75-83
    40. Reveille JD, Solomon DH, American College of Rheumatology, Ad Hoc Committee on Immunological Testing Guidelines Evidence-based guidelines for the use of fimmunologic laboratory tests: anti-centromere, Scl-70 and nucleolar antibodies. Arthritis Rheum. 2003 Jun 15;49(3):399-412
    41. Spencet-Green G, Alter D, Welch HG. Test performance in systemic sclerosis: anti-centromere and anti-Scl-70 antibodies. Am J Med. 1998;103:242-248
    42. Ferri C, Valentini G, Cozzi F, et al. Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine. 2002; 81:139-153.
    43. Riboldi P, Asero R, Origgi L et al. Antinuclear antibodies in progressive systemic sclerosis. Clin Exp Rheum. 1985;3:205-211
    44. Silver KC, Silver RM, Management of systemic-sclerosis-associated interstitial lung disease. Rheum Dis Clin N Am. 2015; 41:439-57
    45. Denton CP, Khanna D., Systemic sclerosis. Lancet 2017 Oct 7; 390(10103): 1685-1699  
    46. Vincent-Cottin, Kevin K., Brown, Interstitial lung disease associated with systemic sclerosis (SSc-ILD), Respir Res 20, 13(2019). https://doi.org/10.1186/s12931-019-0980-7
    47. Tashin 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; 4:708-19
    48.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): S17-22.
    49. Roth M. Scleroderma Lung Study III – Combining Pirfenidone With Mycophenolate (SLSIII).  https://clinicaltrials.gov/ct2/show/NCT03221257 
    50.Huang J. Beyer, Palumbo-Zerr K, Zhang Y. et al. Nintedanib inhibits fibroblast activation and ameliorates fibrosis in preclinical models of systemic sclerosis. Ann Rheum Dis. 2016; 75:883-90
    51. Huang J, Maier C,Zhang Y et al. Nintedanib inhibits macrophage activation and ameliorates vascular and fibrotic manifestations in the Fra2 mouse model of systemic sclerosis. Ann Rheum Dis. 2017;76:1941-8
    52. Wollin L, Maillet I, Quesniaux V et al. Antifibrotic and anti-inflammatory activity of the tyrosine kinase inhibitor nintedanib in experimental models of lung fibrosis. J Pharmacol Exp Ther. 2014; 349:209-20
    53. Distler O., Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 2019; 380:2518-28
    54.Burt RK, Shah SJ, Dill K et al. Autologous non-myeloablative haemopoetic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet. 2011; 378:498-506
    55. Kowal Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 2017; 76: 1327-39.
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