Revisión actualizada de enfermedades pulmonares intersticiales en enfermedades autoinmunes
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ene 14, 2025
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https://doi.org/10.36677/medicinainvestigacion.v13i1.24616
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Daniela Itzel López
http://orcid.org/0000-0001-9535-0051
Gildardo Baldomero Zambrano Tobón
http://orcid.org/0009-0002-3043-0645
http://orcid.org/0000-0001-9535-0051
Gildardo Baldomero Zambrano Tobón
http://orcid.org/0009-0002-3043-0645
Resumen
Las enfermedades del tejido conectivo (ECT) ocasionan daño orgánico, una forma común se presenta como enfermedad pulmonar intersticial (EPI), puede diagnosticarse simultáneamente a una enfermedad autoinmune o una tras el diagnóstico de otra. La EPI puede presentarse de manera más común en enfermedad mixta del tejido conectivo, esclerosis sistémica, miopatias inflamatorias idiopáticas (MSA) o artritis reumatoide (AR).
La patogénesis, progresión y pronóstico de EPI-ECT varia dependiendo de la patología acompañante.
En relación con su diagnóstico es indispensable la tomografía constrastada para evaluación sobre lesiones y de acuerdo a eso se han determinado diferentes patrones. En la mayoría de enfermedades se asocia al patrón de neumonia intersticial no específica.
Su tratamiento varía de acuerdo a la enfemedad de base. El presente trabajo muestra una revisión actualizada sobre el tema.
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LÓPEZ, Daniela Itzel; ZAMBRANO TOBÓN, Gildardo Baldomero.
Revisión actualizada de enfermedades pulmonares intersticiales en enfermedades autoinmunes.
Medicina e Investigación Universidad Autónoma del Estado de México, [S.l.], v. 13, n. 1, p. 208-216, ene. 2025.
ISSN 2594-0600.
Disponible en: <https://medicinainvestigacion.uaemex.mx/article/view/24616>. Fecha de acceso: 10 sep. 2026
doi: https://doi.org/10.36677/medicinainvestigacion.v13i1.24616.
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Esta obra está bajo licencia internacional Creative Commons Reconocimiento-NoComercial-SinObrasDerivadas 4.0.
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1. Mackintosh JA, Wells AU, Cottin V, Nicholson AG, Renzoni EA. Interstitial pneumonia with autoimmune features: challenges and controversies. Eur Respir. Rev 2021;30(162) DOI: 10.1183/16000617.0177-2021
2. European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the I. American Thoracic Society
3. Jee A, Sheeshy R, Hospkins P, Corte t, Grainge C, Troy L, Symons K, Spencer L, Reynolds P. Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia New Zealand. 2020Respirology
4. Wijsenbeek ;, Suzui A, Maher TM. Interstitial lung diseases. Lancet. 2022; 400 (10354): 769-786. DOI: 10.1016/S0140-6736(22)01052-2
5. Carrol M, Kanne J, et al. Uptdate on Interstitial Pneumonias. Clin Chest Med. 2023; Volume 0, Issue 0: 1-13. https://doi.org/10.1016/j.ccm.2023.08.015. NO
6. Jeganathan N, Sathananthan M. Connective Tissue Disease-Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment. Lung. 2020;198(5):735-759. doi: 10.1007/s00408-020-00383-w
7. Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J. 2002;19(5):794–6.
8. Toby M. Interstitial Lung Diseade A Review. 2024 JAMA. DOI:10.1001/jama.2024.3669
9. Dai Y, Wang W, Yu Y, Hu S. Rheumatoid arthritis-associated interstitial lung disease: an overview of epidemiology, pathogenesis and management. Clin Rheumatol [Internet]. 2021;40(4):1211–20. Disponible en: http://dx.doi.org/10.1007/s10067-020-05320-z
10. Shaw M, Collins B et al. Rheumatoid arthritis-associated lung disease. 2015; 24: 1-16. DOI: 10.1183/09059180.00008014
11. Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020 Mar;8(3):304-320. doi: 10.1016/S2213-2600(19)30480-1.
12. Ingegnoli F, Ughi N, Mihai C. Update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis.
Best Pract Res Clin Rheumatol 2018; 32: 223–40.
13. Jung E, Suh CH, Kim HA, Jung JY. Clinical characteristics of systemic sclerosis with interstitial lung disease. Arch Rheumatol 2018; 33: 322–27.
14. Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med [Internet]. 2020;8(3):304–20. Disponible en: http://dx.doi.org/10.1016/S2213-2600(19)30480-1
15. Vu TTT, Brown KK, Solomon JJ. Myositis-associated interstitial lung disease. Curr Opin Pulm Med. 2023 Sep 1;29(5):427-435. doi: 10.1097/MCP.0000000000001000.
16. Wu W, Guo L, Fu Y, Wang K, Zhang D, Xu W, Chen Z, Ye S. Interstitial Lung Disease in Anti-MDA5 Positive Dermatomyositis. Clin Rev Allergy Immunol. 2021 Apr;60(2):293-304. doi: 10.1007/s12016-020-08822-5.
17. Kuwana M, Gil-Vila A, Selva-O’Callaghan A. Role of autoantibodies in the diagnosis and prognosis of interstitial lung disease in autoimmune rheumatic disorders. Ther Adv Musculoskelet Dis. Published online July 27, 2021. doi:10.1177/1759720X211032457
18. Mira-Avendano I, Abril A, Burger CD, Dellaripa PF, Fischer A, Gotway MB, Lee AS, Lee JS, Matteson EL, Yi ES, Ryu JH. Enfermedad pulmonar intersticial y otras manifestaciones pulmonares en enfermedades del tejido conectivo. Mayo Clin Proc. 2019 Feb;94(2):309-325. doi: 10.1016/j.mayocp.2018.09.002.
19. Sparks JA, He X, Huang J, Fletcher EA, Zaccardelli A, Friedlander HM, et al. Rheumatoid arthritis disease activity predicting incident clinically apparent rheumatoid arthritis-associated interstitial lung disease: A prospective cohort study. Arthritis Rheumatol [Internet]. 2019;71(9):1472–82. Disponible en: http://dx.doi.org/10.1002/art.40904
20. Van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424.
21. Spagnolo P, Distler O, Ryerson CJ, Tzouvelekis A, Lee JS, Bonella F, Bouros D, Hoffmann-Vold AM, Crestani B, Matteson EL. Mechanisms of progressive fibrosis in connective tissue disease (CTD)-associated interstitial lung diseases (ILDs). Ann Rheum Dis. 2021 Feb;80(2):143-150. doi: 10.1136/annrheumdis-2020-217230.
22. GhodratiS,PugashettiJV,KadochMA, Ghasemiesfe A, Oldham JM. Diagnostic accuracy of chest radiography for detecting fibrotic interstitial lung disease. Ann Am Thorac Soc. 2022;19(11):1934- 1937. doi:10.1513/AnnalsATS.202112-1377RL
23. Hobbs S, Chung JH, Leb J, et al. Practical imaging interpretation in patients suspected of having idiopathic pulmonary fibrosis: official recommendations from the Radiology Working Group of the Pulmonary Fibrosis Foundation. Radiol Cardiothorac Imaging 2021; 3: e200279. doi:10.1148/ryct.2021200279
24. Higuero Sevilla JP, Memon A, Hinchcliff M. Learnings from clinical trials in patients with connective tissue disease-associated interstitial lung disease. Arthritis Res Ther. 2023 Jul 8;25(1):118. doi: 10.1186/s13075-023-03090-y. Erratum in: Arthritis Res Ther. 2023 Jul 27;25(1):132. doi: 10.1186/s13075-023-03123-6.
25. Mathai S, Danoff S. Management of interstitial lund disease associated with connective tissue, 2018 thbmj. DOI ;352:h6819 doi: 10.1136/bmj.h6819
26. Barnes H, Holland AE, Westall GP, Goh NS, Glaspole IN. Cyclophosphamide for connective tissue disease-associated interstitial lung disease. Cochrane Database Syst Rev. 2018 Jan 3;1(1):CD010908. doi: 10.1002/14651858.CD010908.pub2
27. Maher TM, Tudor VA, Saunders P, Gibbons MA, Fletcher SV, Denton CP, Hoyles RK, Parfrey H, Renzoni EA, Kokosi M, Wells AU, Ashby D, Szigeti M, Molyneaux PL; RECITAL Investigators. Rituximab versus intravenous cyclophosphamide in patients with connective tissue disease-associated interstitial lung disease in the UK (RECITAL): a double-blind, double-dummy, randomised, controlled, phase 2b trial. Lancet Respir Med. 2023 Jan;11(1):45-54. doi: 10.1016/S2213-2600(22)00359-9. Epub 2022 Nov 11.
28. Rahaghi FF, Hsu VM, Kaner RJ, Mayes MD, Rosas IO, Saggar R, Steen VD, Strek ME, Bernstein EJ, Bhatt N, Castelino FV, Chung L, Domsic RT, Flaherty KR, Gupta N, Kahaleh B, Martinez FJ, Morrow LE, Moua T, Patel N, Shlobin OA, Southern BD, Volkmann ER, Khanna D. Consenso experto sobre el manejo de la enfermedad pulmonar intersticial asociada a la esclerosis sistémica. Respir Res. 2023 Jan 9;24(1):6. doi: 10.1186/s12931-022-02292-3.
29. 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.
30. Tashkin DP , Elashoff R , Clements PJ , et al . Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006;354:2655–66. doi:10.1056/NEJMoa055120
31. 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;54:3962–70. doi:10.1002/art.22204
32. Ma X, Tang R, Luo M, Zeng Z, Shi Y, Tang B, Xiao R. Efficacy of mycophenolate mofetil versus cyclophosphamide in systemic sclerosis-related interstitial lung disease: a systematic review and meta-analysis. Clin Rheumatol. 2021 Aug;40(8):3185-3193. doi: 10.1007/s10067-021-05794-5.
33. Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM; SENSCIS Trial Investigators. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. N Engl J Med. 2019 Jun 27;380(26):2518-2528. doi: 10.1056/NEJMoa1903076.
34. Lui JK, Bosch NA, Gillmeyer KR, Reardon CC. Novel Therapeutic Approaches for Pulmonary Manifestations of Systemic Sclerosis. Am J Respir Crit Care Med. 2020 Sep 15;202(6):878-880. doi: 10.1164/rccm.202002-0307RR.
35. Matson SM, Baqir M, Moua T, Marll M, Kent J, Iannazzo NS, Boente RD, Donatelli JM, Dai J, Diaz FJ, Demoruelle MK, Hamblin MB, Mathai SK, Ryu JH, Pope K, Walker CM, Lee JS. Treatment Outcomes for Rheumatoid Arthritis-Associated Interstitial Lung Disease: A Real-World, Multisite Study of the Impact of Immunosuppression on Pulmonary Function Trajectory. Chest. 2023 Apr;163(4):861-869. doi: 10.1016/j.chest.2022.11.035.
36. Narváez J, Robles-Pérez A, Molina-Molina M, Vicens-Zygmunt V, Luburich P, Yañez MA, Alegre JJ, Nolla JM. Real-world clinical effectiveness of rituximab rescue therapy in patients with progressive rheumatoid arthritis-related interstitial lung disease. Semin Arthritis Rheum. 2020 Oct;50(5):902-910. doi: 10.1016/j.semarthrit.2020.08.008.
37. Matteson EL, Aringer M, Burmester GR, Mueller H, Moros L, Kolb M. Effect of nintedanib in patients with progressive pulmonary fibrosis associated with rheumatoid arthritis: data from the INBUILD trial. Clin Rheumatol. 2023 Sep;42(9):2311-2319. doi: 10.1007/s10067-023-06623-7.
38. Fujisawa T. Management of Myositis-Associated Interstitial Lung Disease. Medicina (Kaunas). 2021 Apr 3;57(4):347. doi: 10.3390/medicina57040347.
39. Leard LE, Holm AM, Valapour M, et al. Consensus document for the selection of lung transplant candidates: an update from the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2021; 40: 1349–79.
40. Luppi F, Sebastiani M, Salvarani C, Bendstrup E, Manfredi A. Acute exacerbation of interstitial lung disease associated with rheumatic disease. Nat Rev Rheumatol. 2022 Feb;18(2):85-96. doi: 10.1038/s41584-021-00721-z.
2. European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the I. American Thoracic Society
3. Jee A, Sheeshy R, Hospkins P, Corte t, Grainge C, Troy L, Symons K, Spencer L, Reynolds P. Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia New Zealand. 2020Respirology
4. Wijsenbeek ;, Suzui A, Maher TM. Interstitial lung diseases. Lancet. 2022; 400 (10354): 769-786. DOI: 10.1016/S0140-6736(22)01052-2
5. Carrol M, Kanne J, et al. Uptdate on Interstitial Pneumonias. Clin Chest Med. 2023; Volume 0, Issue 0: 1-13. https://doi.org/10.1016/j.ccm.2023.08.015. NO
6. Jeganathan N, Sathananthan M. Connective Tissue Disease-Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment. Lung. 2020;198(5):735-759. doi: 10.1007/s00408-020-00383-w
7. Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J. 2002;19(5):794–6.
8. Toby M. Interstitial Lung Diseade A Review. 2024 JAMA. DOI:10.1001/jama.2024.3669
9. Dai Y, Wang W, Yu Y, Hu S. Rheumatoid arthritis-associated interstitial lung disease: an overview of epidemiology, pathogenesis and management. Clin Rheumatol [Internet]. 2021;40(4):1211–20. Disponible en: http://dx.doi.org/10.1007/s10067-020-05320-z
10. Shaw M, Collins B et al. Rheumatoid arthritis-associated lung disease. 2015; 24: 1-16. DOI: 10.1183/09059180.00008014
11. Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020 Mar;8(3):304-320. doi: 10.1016/S2213-2600(19)30480-1.
12. Ingegnoli F, Ughi N, Mihai C. Update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis.
Best Pract Res Clin Rheumatol 2018; 32: 223–40.
13. Jung E, Suh CH, Kim HA, Jung JY. Clinical characteristics of systemic sclerosis with interstitial lung disease. Arch Rheumatol 2018; 33: 322–27.
14. Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med [Internet]. 2020;8(3):304–20. Disponible en: http://dx.doi.org/10.1016/S2213-2600(19)30480-1
15. Vu TTT, Brown KK, Solomon JJ. Myositis-associated interstitial lung disease. Curr Opin Pulm Med. 2023 Sep 1;29(5):427-435. doi: 10.1097/MCP.0000000000001000.
16. Wu W, Guo L, Fu Y, Wang K, Zhang D, Xu W, Chen Z, Ye S. Interstitial Lung Disease in Anti-MDA5 Positive Dermatomyositis. Clin Rev Allergy Immunol. 2021 Apr;60(2):293-304. doi: 10.1007/s12016-020-08822-5.
17. Kuwana M, Gil-Vila A, Selva-O’Callaghan A. Role of autoantibodies in the diagnosis and prognosis of interstitial lung disease in autoimmune rheumatic disorders. Ther Adv Musculoskelet Dis. Published online July 27, 2021. doi:10.1177/1759720X211032457
18. Mira-Avendano I, Abril A, Burger CD, Dellaripa PF, Fischer A, Gotway MB, Lee AS, Lee JS, Matteson EL, Yi ES, Ryu JH. Enfermedad pulmonar intersticial y otras manifestaciones pulmonares en enfermedades del tejido conectivo. Mayo Clin Proc. 2019 Feb;94(2):309-325. doi: 10.1016/j.mayocp.2018.09.002.
19. Sparks JA, He X, Huang J, Fletcher EA, Zaccardelli A, Friedlander HM, et al. Rheumatoid arthritis disease activity predicting incident clinically apparent rheumatoid arthritis-associated interstitial lung disease: A prospective cohort study. Arthritis Rheumatol [Internet]. 2019;71(9):1472–82. Disponible en: http://dx.doi.org/10.1002/art.40904
20. Van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424.
21. Spagnolo P, Distler O, Ryerson CJ, Tzouvelekis A, Lee JS, Bonella F, Bouros D, Hoffmann-Vold AM, Crestani B, Matteson EL. Mechanisms of progressive fibrosis in connective tissue disease (CTD)-associated interstitial lung diseases (ILDs). Ann Rheum Dis. 2021 Feb;80(2):143-150. doi: 10.1136/annrheumdis-2020-217230.
22. GhodratiS,PugashettiJV,KadochMA, Ghasemiesfe A, Oldham JM. Diagnostic accuracy of chest radiography for detecting fibrotic interstitial lung disease. Ann Am Thorac Soc. 2022;19(11):1934- 1937. doi:10.1513/AnnalsATS.202112-1377RL
23. Hobbs S, Chung JH, Leb J, et al. Practical imaging interpretation in patients suspected of having idiopathic pulmonary fibrosis: official recommendations from the Radiology Working Group of the Pulmonary Fibrosis Foundation. Radiol Cardiothorac Imaging 2021; 3: e200279. doi:10.1148/ryct.2021200279
24. Higuero Sevilla JP, Memon A, Hinchcliff M. Learnings from clinical trials in patients with connective tissue disease-associated interstitial lung disease. Arthritis Res Ther. 2023 Jul 8;25(1):118. doi: 10.1186/s13075-023-03090-y. Erratum in: Arthritis Res Ther. 2023 Jul 27;25(1):132. doi: 10.1186/s13075-023-03123-6.
25. Mathai S, Danoff S. Management of interstitial lund disease associated with connective tissue, 2018 thbmj. DOI ;352:h6819 doi: 10.1136/bmj.h6819
26. Barnes H, Holland AE, Westall GP, Goh NS, Glaspole IN. Cyclophosphamide for connective tissue disease-associated interstitial lung disease. Cochrane Database Syst Rev. 2018 Jan 3;1(1):CD010908. doi: 10.1002/14651858.CD010908.pub2
27. Maher TM, Tudor VA, Saunders P, Gibbons MA, Fletcher SV, Denton CP, Hoyles RK, Parfrey H, Renzoni EA, Kokosi M, Wells AU, Ashby D, Szigeti M, Molyneaux PL; RECITAL Investigators. Rituximab versus intravenous cyclophosphamide in patients with connective tissue disease-associated interstitial lung disease in the UK (RECITAL): a double-blind, double-dummy, randomised, controlled, phase 2b trial. Lancet Respir Med. 2023 Jan;11(1):45-54. doi: 10.1016/S2213-2600(22)00359-9. Epub 2022 Nov 11.
28. Rahaghi FF, Hsu VM, Kaner RJ, Mayes MD, Rosas IO, Saggar R, Steen VD, Strek ME, Bernstein EJ, Bhatt N, Castelino FV, Chung L, Domsic RT, Flaherty KR, Gupta N, Kahaleh B, Martinez FJ, Morrow LE, Moua T, Patel N, Shlobin OA, Southern BD, Volkmann ER, Khanna D. Consenso experto sobre el manejo de la enfermedad pulmonar intersticial asociada a la esclerosis sistémica. Respir Res. 2023 Jan 9;24(1):6. doi: 10.1186/s12931-022-02292-3.
29. 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.
30. Tashkin DP , Elashoff R , Clements PJ , et al . Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006;354:2655–66. doi:10.1056/NEJMoa055120
31. 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;54:3962–70. doi:10.1002/art.22204
32. Ma X, Tang R, Luo M, Zeng Z, Shi Y, Tang B, Xiao R. Efficacy of mycophenolate mofetil versus cyclophosphamide in systemic sclerosis-related interstitial lung disease: a systematic review and meta-analysis. Clin Rheumatol. 2021 Aug;40(8):3185-3193. doi: 10.1007/s10067-021-05794-5.
33. Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM; SENSCIS Trial Investigators. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. N Engl J Med. 2019 Jun 27;380(26):2518-2528. doi: 10.1056/NEJMoa1903076.
34. Lui JK, Bosch NA, Gillmeyer KR, Reardon CC. Novel Therapeutic Approaches for Pulmonary Manifestations of Systemic Sclerosis. Am J Respir Crit Care Med. 2020 Sep 15;202(6):878-880. doi: 10.1164/rccm.202002-0307RR.
35. Matson SM, Baqir M, Moua T, Marll M, Kent J, Iannazzo NS, Boente RD, Donatelli JM, Dai J, Diaz FJ, Demoruelle MK, Hamblin MB, Mathai SK, Ryu JH, Pope K, Walker CM, Lee JS. Treatment Outcomes for Rheumatoid Arthritis-Associated Interstitial Lung Disease: A Real-World, Multisite Study of the Impact of Immunosuppression on Pulmonary Function Trajectory. Chest. 2023 Apr;163(4):861-869. doi: 10.1016/j.chest.2022.11.035.
36. Narváez J, Robles-Pérez A, Molina-Molina M, Vicens-Zygmunt V, Luburich P, Yañez MA, Alegre JJ, Nolla JM. Real-world clinical effectiveness of rituximab rescue therapy in patients with progressive rheumatoid arthritis-related interstitial lung disease. Semin Arthritis Rheum. 2020 Oct;50(5):902-910. doi: 10.1016/j.semarthrit.2020.08.008.
37. Matteson EL, Aringer M, Burmester GR, Mueller H, Moros L, Kolb M. Effect of nintedanib in patients with progressive pulmonary fibrosis associated with rheumatoid arthritis: data from the INBUILD trial. Clin Rheumatol. 2023 Sep;42(9):2311-2319. doi: 10.1007/s10067-023-06623-7.
38. Fujisawa T. Management of Myositis-Associated Interstitial Lung Disease. Medicina (Kaunas). 2021 Apr 3;57(4):347. doi: 10.3390/medicina57040347.
39. Leard LE, Holm AM, Valapour M, et al. Consensus document for the selection of lung transplant candidates: an update from the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2021; 40: 1349–79.
40. Luppi F, Sebastiani M, Salvarani C, Bendstrup E, Manfredi A. Acute exacerbation of interstitial lung disease associated with rheumatic disease. Nat Rev Rheumatol. 2022 Feb;18(2):85-96. doi: 10.1038/s41584-021-00721-z.