#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#
preLekára.sk / Journals / Czech Rheumatology / 2022 - 3

Interstitial lung disease in systemic connective tissue diseases – risk factors, diagnostic and treatment

Česká verzia

Authors: P. Horák 1;  M. Žurková 2;  A. Skoumalová 1;  M. Žurek 1;  M. Skácelová 1
Authors place of work: III. interní klinika – nefrologická, revmatologická a endokrinologická LF UP a FN, Olomouc 1;  Klinika plicních nemocí a tuberkulózy LF UP a FN, Olomouc 2
Published in the journal: Čes. Revmatol., 30, 2022, No. 3, p. 121-133.
Category: Review Article

Summary

The tissues of the airways and lungs are among the frequently affected structures in systemic connective tissue diseases. Interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and fibrosis of the lung parenchyma. They are classified based on histopathological, radiological, and clinical parameters. The distinction between idiopathic pulmonary fibrosis and fibrosis accompanying systemic connective tissue diseases is important from the point of view of prognosis, as secondary forms have a more favorable course, although their impact is also serious. Interstitial lung involvement occurs most often in systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and rheumatoid arthritis, less often in systemic lupus erythematosus and Sjögren's syndrome. It also occurs frequently in microscopic polyangiitis. The prevalence, histopathological type, and mortality of these conditions vary significantly between rheumatic diseases. The cooperation of a rheumatologist, pulmonologist, and radiologist as well as screening strategies play a vital role in early diagnosis. Screening strategies are important for early diagnosis. Functional lung examination and imaging methods, especially high-resolution computed tomography, are irreplaceable examination methods. A number of anti-inflammatory drugs are available for therapy. For inflammatory forms of the disorders, it is possible to use glucocorticoids, cyclophosphamide, mycophenolate mofetil, azathioprine, calcineurin inhibitors, B-depletion therapy, or tocilizumab. The effect of these drugs is insufficient in predominantly fibrotic forms, where the use of antifibrotic drugs is an emerging therapy. Nintedanib is a drug newly registered for the indication of progressive interstitial lung involvement in systemic connective tissue diseases with broad efficacy evidence based on randomized, double-blinded, placebo-controlled trials.

Keywords:

interstitial lung disease – Connective tissue – immunosuppression – antifibrotic therapy

Zdroje
  1. Fischer A, du Bois R. Interstitial lung disease in connective tissue disorders. Lancet 2012; 380: 689–698.
  2. Shao T, Shi X, Yang S, et al. Interstitial Lung Disease in Connective Tissue Disease: A Common Lesion With Heterogeneous Mechanisms and Treatment Considerations. Front Immunol 2021; 12: 684699.
  3. Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner society white paper. Lancet Respir Med 2018; 6: 138–153.
  4. Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2018; 198: e44–e68.
  5. Ha YJ, Lee YJ, Kang EH. Lung Involvements in Rheumatic Diseases: Update on the Epidemiology, Pathogenesis, Clinical Features, and Treatment. Biomed Res Int 2018; 6930297.
  6. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med 2000; 161: 646–664.
  7. Verschakelen JA. The role of high-resolution computed tomography in the work-up of interstitial lung disease. Curr Opin Pulm Med 2010; 16: 503–510.
  8. Doyle TJ, Dellaripa PF. Lung Manifestations in the Rheumatic Diseases. Chest 2017; 152: 1283–1295.
  9. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 2007; 66: 940–944.
  10. Nieto MA, Rodriguez-Nieto MJ, Sanchez-Pernaute O, et al. Mortality rate in rheumatoid arthritis-related interstitial lung disease: the role of radiographic patterns. BMC Pulm Med 2021; 30: 205.
  11. Wuyts WA, Papiris S, Manali E, et al. The burden of progressive fibrosing interstitial lung dinase: A DELPHI approach. Adv Ther 2020; 37: 3246–3264.
  12. Olson A, Hartmann N, Patnaik P, et al. Estimation of the Prevalence of Progressive Fibrosing Interstitial Lung Diseases: Systematic Literature Review and Data from a Physician Survey. Adv Ther 2021; 38: 854–867.
  13. Nihtyanova SI, Schreiber BE, Ong VH, et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol 2014; 66: 1625–1635.
  14. Jaeger VK, Wirz EG, Allanore Y, et al. Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study. PLoS One 2016; 11: e0163894.
  15. Hoffmann-Vold AM, Maher TM, Philpot EE, et al. Assessment of recent evidence for the management of patients with systemic sclerosis-associated interstitial lung disease: a systematic review. ERJ Open Res 2021; 7: 00235–2020.
  16. Fujisawa T. Management of myositis-associated interstitial lung disease. Medicina (Kaunas) 2021; 57: 347.
  17. Nakashima, R, Hosono Y, Mimori T. Clinical significance and new detection system of autoantibodies in myositis with interstitial lung disease. Lupus 2016; 25: 925–933.
  18. Ye S, Chen XX, Lu XY. Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study. Clin Rheumatol 2007; 26: 1647–1654.
  19. McPherson M, Economidou S, Liampas A, et al. Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: A systematic review. Semin Arthritis Rheum 2022; 53: 151959.
  20. Highland KH, Silver RM. Pulmonary mnifestation in Mixed Connective Tissue Disease. In: Dellaripa P, Fisher A, Flaherty K. Pulmonary manifestations of rheumatic diseasaes. A comprehensive guide. Springer 2014; 72–82.
  21. Kondoh Y, Makino S, Ogura T, et al. 2020 guide for the diagnosis and treatment of interstitial lung disease associated with connective tissue disease. Respir Investig 2021; 59: 709–740.
  22. Kadura S, Raghu G. Rheumatoid arthritis-interstitial lung disease: manifestations and current concepts in pathogenesis and management. Eur Respir Rev 2021; 30: 210011.
  23. Solomon JJ, Chung JH, Cosgrove GP, et al. Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J 2016; 47: 588–596. doi: 10.1183/13993003.00357- 2015.
  24. Gutsche M, Rosen GD, Swigris JJ. Connective tissue disease- -associated interstitial lung disease: a review. Current Respiratory Care Reports 2012; 1: 224–232.
  25. Lopez Velazquez M, Highland KB. Pulmonary manifestations of systemic lupus erythematosus and Sjögren‘s syndrome. Curr Opin Rheumatol 2018; 30: 449–464.
  26. Keane MP, Lynch JP III. Pleuropulmonary manifestations of systemic lupus erythematosus Thorax 2000; 55: 159–166.
  27. Mira-Avendano C Abril A. Pulmonary manifestations of Sjögren syndrome, systemic lupus erythematosus, and mixed connective tissue disease. Rheum Dis Clin N Am 2015; 41: 263–277.
  28. 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: 976–987.
  29. Yamakawa H, Ogura T, Kameda H, et al. Decision-Making Strategy for the Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease (RA-ILD). J Clin Med 2021; 10: 3806.
  30. . Rojas-Serrano J, Herrera-Bringas D, Pérez-Román DI, et al. Rheumatoid arthritis-related interstitial lung disease (RA-ILD): methotrexate and the severity of lung disease are associated to prognosis. Clin Rheumatol 2017; 36: 1493–1500.
  31. Juge PA, Lee JS, Lau J, et al. Methotrexate and rheumatoid arthritis associated interstitial lung disease. Eur Respir J 2021; 57: 2000337.
  32. Robles-Pérez A, Luburich P, Bolivar S, et al. A prospective study of lung disease in a cohort of early rheumatoid arthritis patients. Sci Rep 2020; 10: 15640.
  33. Ibfelt EH, Jacobsen RK, Kopp TI, et al. Methotrexate and risk of interstitial lung disease and respiratory failure in rheumatoid arthritis: a nationwide population-based study. Rheumatology (Oxford) 2021; 60: 346–352.
  34. Li L, Liu R, Zhang Y, et al. A retrospective study on the predictive implications of clinical characteristics and therapeutic management in patients with rheumatoid arthritis-associated interstitial lung disease. Clin Rheumatol 2020; 39: 1457–1470.
  35. Wells AU. New insights into the treatment of CTD-ILD. Nat Rev Rheumatol 2021; 17: 79–80.
  36. Doubková M. Česká pneumologická a ftizeologická společnost. Guidelines. Intersticiální plicní postižení a systémová onemocnění pojiva-doporučení diagnostiky a léčby, aktualiace 2021. https://www.pneumologie.cz/guidelines, Accessed 20. 7. 2022.
  37. Yamano Y, Taniguchi H, Kondoh Y, et al. Multidimensional improvement in connective tissue disease-associated interstitial lung disease: Two courses of pulse dose methylprednisolone 132 PŘEHLEDOVÝ ČLÁNEK followed by low-dose prednisone and tacrolimus. Respirology 2018; 23: 1041–1048.
  38. 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: 103–112.
  39. Hoyles RK, Ellis RW, Wellsbury J, 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: 3962–3970.
  40. Tashkin DP, Elashoff R, Clements PJ, et al. Scleroderma Lung Study Research Group. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006; 354: 2655–2666.
  41. Roth MD, Tseng CH, Clements PJ, et al. Scleroderma Lung Study Research Group. Predicting treatment outcomes and responder subsets in scleroderma-related interstitial lung disease. Arthritis Rheum 2011; 63: 2797–2808.
  42. Ge Y, Peng Q, Zhang S, et al. Cyclophosphamide treatment for idiopathic inflammatory myopathies and related interstitial lung disease: a systematic review. Clin Rheumatol 2015; 34: 99–105.
  43. Nakamura K, Ohbe H, Ikeda K, et al. Intravenous cyclophosphamide in acute exacerbation of rheumatoid arthritis-related interstitial lung disease: A propensity-matched analysis using a nationwide inpatient database. Semin Arthritis Rheum 2021; 51: 977–982.
  44. Tashkin DP, Roth MD, Clements PJ, et al. Sclerodema Lung Study II Investigators. 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: 708–719.
  45. Simeón-Aznar CP, Fonollosa-Plá V, Tolosa-Vilella C, et al. Effect of mycophenolate sodium in scleroderma-related interstitial lung disease. Clin Rheumatol 2011; 30: 1393–1398.
  46. Fischer A, Brown KK, Du Bois RM, et al. Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease. J Rheumatol 2013; 40: 640–646.
  47. Huapaya JA, Silhan L, Pinal-Fernandez I, et al. Long-term treatment with azathioprine and mycophenolate mofetil for myositis- related interstitial lung disease. Chest 2019; 156: 896–906.
  48. Kelly C, Young A, Ahmad Y, et al. The effect of steroids, azathioprine and mycophenolate on the risk of death in rheumatoid arthritis. Rheumatology 2016; 55: i99–i100.
  49. Cassone G, Sebastiani M, Vacchi C, et al. Efficacy and safety of mycophenolate mofetil in the treatment of rheumatic disease- related interstitial lung disease: a narrative review. Drugs Context 2021; 10: 2020-8-8.
  50. Fujisawa T, Hozumi, H, Kamiya, Ye, et al. Prednisolone and tacrolimus versus prednisolone and cyclosporin A to treat polymyositis/ dermatomyositis-associated ILD: A randomized, open-label trial. Respirology 2021; 26: 370–377.
  51. Cassone G, Manfredi A, Vacchi C, et al. Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease: Lights and Shadows. J Clin Med 2020; 9: 1082.
  52. Caldas MMVDF, Azevedo KPMD, Nunes ACDF, et al. Is rituximab effective for systemic sclerosis? A systematic review and meta- -analysis. Advances in Rheumatology 2021; 61: 15.
  53. Ge Y, Li S, Tian X, et al. Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis responds to rituximab therapy. Clin Rheumatol 2021; 40: 2311–2317.
  54. Mao MM, Xia S, Guo BP, et al. Ultra-low dose rituximab as add- on therapy in anti-MDA5-positive patients with polymyositis / dermatomyositis associated ILD. Respir Med 2020; 172: 105983.
  55. Fui A, Bergantini L, Selvi E, et al. Rituximab therapy in interstitial lung disease associated with rheumatoid arthritis. Intern Med J 2020; 50: 330–336.
  56. Vadillo C, Nieto MA, Romero-Bueno F, et al. Efficacy of rituximab in slowing down progression of rheumatoid arthritis-related interstitial lung disease: data from the NEREA Registry. Rheumatology (Oxford) 2020; 59: 2099–2108.
  57. Khanna D, Denton CP, Jahreis A, et al. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet 2016; 387: 2630–2640.
  58. Khanna D, Denton CP, Lin CJF, et al. Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). Ann Rheum Dis 2018; 77: 212–220.
  59. Khanna D, Lin CJF, Furst DE, et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med 2020; 8: 963–974.
  60. Manfredi A, Cassone G, Furini F, et al. Tocilizumab therapy in rheumatoid arthritis with interstitial lung disease: a multicentre retrospective study. Intern Med J 2020; 50: 1085–1090.
  61. Wollin L, Wex E, Pautsch A, et al. Mode of action of nintedanib in the treatment of idiopathic pulmonary fibrosis. Eur Respir J 2015; 45: 1434–1445.
  62. Distler O, Highland KB, Gahlemann M, et al. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. N Engl J Med 2019; 380: 2518–2528.
  63. Seibold JR, Maher TM, Highland KB, et al. Safety and tolerability of nintedanib in patients with systemic sclerosis-associated interstitial lung disease: data from the SENSCIS trial. Ann Rheum Dis 2020; 79: 1478–1484.
  64. Wells AU, Flaherty KR, Brown KK, et al. Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel- -group trial. Lancet Respir Med 2020; 8: 453–460.
  65. Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive interstitial lung diseases: data from the whole INBUILD trial. Eur Respir J 2022; 59: 2004538.
  66. Matteson EL, Kelly C, Distler JHW, et al. INBUILD trial investigators. Nintedanib in patients with autoimmune disease-related progressive fibrosing interstitial lung diseases: subgroup analysis of the INBUILD trial. Arthritis Rheumatol 2022; 74: 1039–1047.
  67. Acharya N, Sharma SK, Mishra D, et al. Efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease – a randomised controlled trial. Rheumatol Int 2020; 40: 703–710.
  68. Li T, Guo L, Chen Z, et al. Treatment of Rheumatoid Arthritis- Associated Interstitial Lung Disease: Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis. Sci Rep 2016; 6: 33226.
  69. NCT03221257. Scleroderma lung study III – combining pirfenidone with mycophenolate. https://clinicaltrials.gov/ct2/show/ NCT03221257?term=NCT03221257&draw=2&rank=1. Published 2017. Accessed June 29, 2022.
  70. Solomon JJ, Danoff SK, Goldberg HJ, et al. The design and rationale of the trail1 trial: a randomized double-blind phase 2 clinical trial of pirfenidone in rheumatoid arthritis-associated interstitial lung disease. Adv Ther 2019; 36: 3279–3287.
  71. NCT02821689. Pirfenidone in progressive interstitial lung disease associated with clinically amyopathic dermatomyositis – full text view. ClinicalTrials.gov. https://clinicaltrials.gov/ct2/ show/NCT02821689. Accessed June 29, 2022.
  72. Burt RK, Shah SJ, Dill K, et al. Autologous non-myeloablative haemopoietic tem-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. 
  73. van Laar JM, Farge D, Sont JK, et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 2014; 311: 2490–2798.
  74. Sullivan, KM, Goldmuntz EA, Keyes-Elstein L, et al. Myeloablative Autologous Stem-Cell Transplantation for Severe Scleroderma, NEJM 2018; 378: 35–47.
  75. Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 2017; 76: 1327–1339.
  76. Farge D, Burt RK, Oliveira MC, et al. Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners. Bone Marrow Transplant 2017; 52: 1495–1503.
  77. Valentová-Bartáková L, Fila F, Kováříková Z. Česká pneumologická a ftizeologická společnost. Guidelines. Doporučení k předtransplantačnímu období, indikace a kontraindikace k transplantaci plic. Aktualizace 2019, http://www.pneumologie. cz/guidelines, Accessed 20. 7. 2022.
  78. Pradère P, Tudorache I, Magnusson J, et al. Lung transplantation for scleroderma lung disease: An international, multicenter, observational cohort study. J Heart Lung Transplant 2018; 37: 903–911. doi: 10.1016/j.healun.2018.03.003.
  79. Miele CH, Schwab K, Saggar R, et al. Lung Transplant Outcomes in Systemic Sclerosis with Significant Esophageal Dysfunction. A Comprehensive Single-Center Experience. Ann Am Thorac Soc 2016; 13: 793–802.
Štítky
Dermatology & STDs Paediatric rheumatology Rheumatology
Článek Úvodník
Článek Prof. MUDr. Eduard Cmunt – průkopník české revmatologie
Článek Risk of cardiac and ocular toxicity of synthetic antimalarials in rheumatic diseases
Článek Comorbidities in spondyloarthritits
Článek Prevalence of spondyloarthritis in patients with acute anterior uveitis
Článek Fertility disorder associated with undifferentiated connective tissue disease
Článok vyšiel v časopise

Czech Rheumatology

Číslo 3
2022 Číslo 3
Najčítanejšie tento týždeň
Najčítanejšie v tomto čísle
Prihlásenie
Zabudnuté heslo

Zadajte e-mailovú adresu, s ktorou ste vytvárali účet. Budú Vám na ňu zasielané informácie k nastaveniu nového hesla.

Prihlásenie

Nemáte účet?  Registrujte sa

#ADS_BOTTOM_SCRIPTS#