Novel Therapeutic Approaches in Connective Tissue Disease-Associated Interstitial Lung Disease

Erica Mulcaire-Jones; Janelle Vu Pugashetti; Justin M. Oldham; Dinesh Khanna

doi:10.1055/s-0044-1786155

Seminars in Respiratory and Critical Care Medicine, Inhaltsverzeichnis

Semin Respir Crit Care Med 2024; 45(03): 435-448
DOI: 10.1055/s-0044-1786155

Review Article

Novel Therapeutic Approaches in Connective Tissue Disease-Associated Interstitial Lung Disease

Authors

Erica Mulcaire-Jones

¹Division of Rheumatology, Internal Medicine, University of Michigan, Ann Arbor, Michigan
Janelle Vu Pugashetti

²Division of Pulmonary and Critical Care Medicine, Internal Medicine, University of Michigan, Ann Arbor, Michigan
Justin M. Oldham

²Division of Pulmonary and Critical Care Medicine, Internal Medicine, University of Michigan, Ann Arbor, Michigan
Dinesh Khanna

¹Division of Rheumatology, Internal Medicine, University of Michigan, Ann Arbor, Michigan

Abstract

Connective tissue diseases (CTD) comprise a group of autoimmune diseases that can affect multiple organs in the body including the lungs. The most common form of pulmonary involvement is interstitial lung disease (ILD). CTD-associated ILD (CTD-ILD) can take one of several courses including nonprogressive, chronically progressive, or rapidly progressive. Chronically and rapidly progressive patterns are associated with increased mortality. Limited randomized controlled trial data are available for treatment of CTD-ILD, with most data coming from systemic sclerosis-related ILD. The current first-line treatment for all CTD-ILD is immunosuppression with consideration of antifibrotics, stem cell transplant, and lung transplant in progressive disease. In this article, we review data for ILD treatment options in systemic sclerosis, rheumatoid arthritis, myositis, and primary Sjögren's syndrome-related ILDs.

Keywords

connective tissue disease - interstitial lung disease - rheumatoid arthritis - systemic sclerosis - idiopathic inflammatory myositis - primary Sjögren's syndrome - treatment

Volltext

Referenzen

References
1 Jeganathan N, Sathananthan M. Connective tissue disease-related interstitial lung disease: prevalence, patterns, predictors, prognosis, and treatment. Lung 2020; 198 (05) 735-759
2 Joy GM, Arbiv OA, Wong CK. et al. Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis. Eur Respir Rev 2023; 32 (167) 220210
3 Hyldgaard C, Bendstrup E, Pedersen AB, Pedersen L, Ellingsen T. Interstitial lung disease in connective tissue diseases: survival patterns in a population-based cohort. J Clin Med 2021; 10 (21) 4830
4 Ahmed S, Handa R. Management of connective tissue disease-related interstitial lung disease. Curr Pulmonol Rep 2022; 11 (03) 86-98
5 Pope JE, Denton CP, Johnson SR, Fernandez-Codina A, Hudson M, Nevskaya T. State-of-the-art evidence in the treatment of systemic sclerosis. Nat Rev Rheumatol 2023; 19 (04) 212-226
6 Raghu G, Montesi SB, Silver RM. et al. Treatment of systemic sclerosis-associated interstitial lung disease: evidence-based recommendations. an official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med 2024; 209 (02) 137-152
7 Raghu G, Remy-Jardin M, Richeldi L. et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2022; 205 (09) e18-e47
8 Kawano-Dourado L, Lee JS. Management of connective tissue disease-associated interstitial lung disease. Clin Chest Med 2021; 42 (02) 295-310
9 On behalf of ACR Board of Directors. 2023 American College of Rheumatology (ACR) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Disease - Guideline Summary. August 12, 2023 ( https://assets.contentstack.io/v3/assets/bltee37abb6b278ab2c/bltaedebda97a351d47/interstitial-lung-disease-guideline-summary-treatment-2023.pdf )
10 Denton CP, Khanna D. Systemic sclerosis. Lancet 2017; 390 (10103): 1685-1699
11 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 (02) 223-240
12 Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med 2020; 8 (03) 304-320
13 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
14 Nihtyanova SI, Schreiber BE, Ong VH. et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol 2014; 66 (06) 1625-1635
15 Hoffmann-Vold AM, Allanore Y, Alves M. et al; EUSTAR collaborators. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis 2021; 80 (02) 219-227
16 Steen V, Domsic RT, Lucas M, Fertig N, Medsger Jr TA. A clinical and serologic comparison of African American and Caucasian patients with systemic sclerosis. Arthritis Rheum 2012; 64 (09) 2986-2994
17 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
18 Giacomelli R, Valentini G, Salsano F. et al. Cyclophosphamide pulse regimen in the treatment of alveolitis in systemic sclerosis. J Rheumatol 2002; 29 (04) 731-736
19 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 (12) 3962-3970
20 Pakas I, Ioannidis JP, Malagari K, Skopouli FN, Moutsopoulos HM, Vlachoyiannopoulos PG. Cyclophosphamide with low or high dose prednisolone for systemic sclerosis lung disease. J Rheumatol 2002; 29 (02) 298-304
21 White B, Moore WC, Wigley FM, Xiao HQ, Wise RA. Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 2000; 132 (12) 947-954
22 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 (25) 2655-2666
23 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 (09) 708-719
24 Volkmann ER, Tashkin DP, Li N. et al. Mycophenolate mofetil versus placebo for systemic sclerosis-related interstitial lung disease: an analysis of scleroderma lung studies I and II. Arthritis Rheumatol 2017; 69 (07) 1451-1460
25 Jaafar S, Lescoat A, Huang S. et al. Clinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis: a longitudinal analysis of an observational prospective multicenter US cohort. Arthritis Res Ther 2021; 23 (01) 170
26 Khanna D, Lin CJF, Furst DE. et al. focuSSced investigators. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med 2020; 8 (10) 963-974
27 Roofeh D, Lin CJF, Goldin J. et al. focuSSced Investigators. Tocilizumab prevents progression of early systemic sclerosis-associated interstitial lung disease. Arthritis Rheumatol 2021; 73 (07) 1301-1310
28 Khanna D, Lin CJF, Furst DE. et al. Long-term safety and efficacy of tocilizumab in early systemic sclerosis-interstitial lung disease: open-label extension of a phase 3 randomized controlled trial. Am J Respir Crit Care Med 2022; 205 (06) 674-684
29 Ebata S, Yoshizaki A, Oba K. et al. Safety and efficacy of rituximab in systemic sclerosis (DESIRES): a double-blind, investigator-initiated, randomised, placebo-controlled trial. Lancet Rheumatol 2021; 3 (07) e489-e497
30 Kuzumi A, Ebata S, Fukasawa T. et al. Long-term outcomes after rituximab treatment for patients with systemic sclerosis: follow-up of the DESIRES trial with a focus on serum immunoglobulin levels. JAMA Dermatol 2023; 159 (04) 374-383
31 Distler O, Highland KB, Gahlemann M. et al. SENSCIS Trial Investigators. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 2019; 380 (26) 2518-2528
32 Acharya N, Sharma SK, Mishra D, Dhooria S, Dhir V, Jain S. Efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease-a randomised controlled trial. Rheumatol Int 2020; 40 (05) 703-710
33 Khanna DS, Bernstein E, Goldin J, Tashkin D, Roth M. Combination therapy of mycophenolate mofetil and pirfenidone vs. mycophenolate alone: results from the Scleroderma Lung Study III - abstract number: 0520. ACR Convergence 2022
34 Roth M. Scleroderma Lung Study III - Combining Pirfenidone With Mycophenolate (SLSIII). ClinicalTrials.gov. https://clinicaltrials.gov/study/NCT03221257
35 van Laar JM, Farge D, Sont JK. et al. EBMT/EULAR Scleroderma Study Group. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 2014; 311 (24) 2490-2498
36 Sullivan KM, Goldmuntz EA, Keyes-Elstein L. et al. SCOT Study Investigators. Myeloablative autologous stem-cell transplantation for severe scleroderma. N Engl J Med 2018; 378 (01) 35-47
37 A Multicenter Trial to Evaluate the Efficacy, Safety, Tolerability and Pharmacokinetics of HZN-825 in Patients with Diffuse Cutaneous Systemic Sclerosis. clinicaltrials.gov.
38 Phase 2 Safety and Efficacy Study of Tulisokibart (MK-7240/PRA023) in Subjects With Systemic Sclerosis Associated With Interstitial Lung Disease (SSc-ILD) (MK-7240-007) (ATHENA-SSc-ILD). clinicaltrials.gov.
39 A Study of the Efficacy and Safety of Belimumab in Adults With Systemic Sclerosis Associated Interstitial Lung Disease (BLISSc-ILD). National Institute of Health. (clincaltrials.gov).
40 Determine Effectiveness of Anifrolumab In SYstemic Sclerosis (DAISY). National Institute of Health. (clincaltrials.gov).
41 A Study Evaluating the Efficacy and Safety of Vixarelimab in Participants With Idiopathic Pulmonary Fibrosis and in Participants With Systemic Sclerosis-Associated Interstitial Lung Disease. National Institute of Health. clinicaltrials.gov.
42 Platform Clinical Study for Conquering Scleroderma (CONQUEST). National Institute of Health. clinicaltrials.gov.
43 Singh JA, Saag KG, Bridges Jr SL. et al. 2015 American College of Rheumatology guideline for the treatment of rheumatoid arthritis. Arthritis Rheumatol 2016; 68 (01) 1-26
44 Kay J, Upchurch KS. ACR/EULAR 2010 rheumatoid arthritis classification criteria. Rheumatology (Oxford) 2012; 51 (6, Suppl 6) vi5-vi9
45 Brown KK, Roger S. Rheumatoid lung disease. Proc Am Thorac Soc 2007; 4 (05) 443-448
46 Bongartz T, Nannini C, Medina-Velasquez YF. et al. Incidence and mortality of interstitial lung disease in rheumatoid arthritis: a population-based study. Arthritis Rheum 2010; 62 (06) 1583-1591
47 Olson AL, Swigris JJ, Sprunger DB. et al. Rheumatoid arthritis-interstitial lung disease-associated mortality. Am J Respir Crit Care Med 2011; 183 (03) 372-378
48 Narváez J, Aburto M, Seoane-Mato D. et al. Screening criteria for interstitial lung disease associated to rheumatoid arthritis: expert proposal based on Delphi methodology. Reumatol Clín (Engl Ed) 2023; 19 (02) 74-81
49 Juge PA, Granger B, Debray MP. et al. A risk score to detect subclinical rheumatoid arthritis-associated interstitial lung disease. Arthritis Rheumatol 2022; 74 (11) 1755-1765
50 Doyle TJ, Patel AS, Hatabu H. et al. Detection of rheumatoid arthritis-interstitial lung disease is enhanced by serum biomarkers. Am J Respir Crit Care Med 2015; 191 (12) 1403-1412
51 Solomon JJ, Ryu JH, Tazelaar HD. et al. Fibrosing interstitial pneumonia predicts survival in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Respir Med 2013; 107 (08) 1247-1252
52 Solomon JJ, Chung JH, Cosgrove GP. et al. Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J 2016; 47 (02) 588-596
53 Jacob J, Hirani N, van Moorsel CHM. et al. Predicting outcomes in rheumatoid arthritis related interstitial lung disease. Eur Respir J 2019; 53 (01) 1800869
54 Kelly CA, Nisar M, Arthanari S. et al. Rheumatoid arthritis related interstitial lung disease - improving outcomes over 25 years: a large multicentre UK study. Rheumatology (Oxford) 2021; 60 (04) 1882-1890
55 Matson SM, Baqir M, Moua T. et al. 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; 163 (04) 861-869
56 Md Yusof MY, Kabia A, Darby M. et al. Effect of rituximab on the progression of rheumatoid arthritis-related interstitial lung disease: 10 years' experience at a single centre. Rheumatology (Oxford) 2017; 56 (08) 1348-1357
57 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 (08) 2099-2108
58 Lopez-Olivo MA, Amezaga Urruela M, McGahan L, Pollono EN, Suarez-Almazor ME. Rituximab for rheumatoid arthritis. Cochrane Database Syst Rev 2015; 1: CD007356
59 Maxwell LJ, Singh JA. Abatacept for rheumatoid arthritis: a Cochrane systematic review. J Rheumatol 2010; 37 (02) 234-245
60 Fernández-Díaz C, Castañeda S, Melero-González RB. et al. Abatacept in interstitial lung disease associated with rheumatoid arthritis: national multicenter study of 263 patients. Rheumatology (Oxford) 2020; 59 (12) 3906-3916
61 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 (05) 977-982
62 Solomon JJ, Danoff SK, Woodhead FA. et al. TRAIL1 Network Investigators. Safety, tolerability, and efficacy of pirfenidone in patients with rheumatoid arthritis-associated interstitial lung disease: a randomised, double-blind, placebo-controlled, phase 2 study. Lancet Respir Med 2023; 11 (01) 87-96
63 AbatacePt in Rheumatoid Arthritis-ILD (APRIL). National Institute of Health. clinicaltrials.org.
64 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 (09) 1085-1090
65 Tardella M, Di Carlo M, Carotti M, Ceccarelli L, Giovagnoni A, Salaffi F. A retrospective study of the efficacy of JAK inhibitors or abatacept on rheumatoid arthritis-interstitial lung disease. Inflammopharmacology 2022; 30 (03) 705-712
66 Tofacitinib in the Treatment of Rheumatoid Arthritis-related Interstitial Lung Disease. (RAILDTo). National Institute of Health. clinicaltrials.gov.
67 Effects of Tofacitinib vs Methotrexate on Rheumatoid Arthritis Interstitial Lung Disease (PULMORA). National Institute of Health. clinicaltrials.gov.
68 Connors GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years?. Chest 2010; 138 (06) 1464-1474
69 Fujisawa T, Hozumi H, Kono M. et al. Prognostic factors for myositis-associated interstitial lung disease. PLoS One 2014; 9 (06) e98824
70 Fujisawa T. Management of myositis-associated interstitial lung disease. Medicina (Kaunas) 2021; 57 (04) 347
71 Chen Z, Wang X, Ye S. Tofacitinib in amyopathic dermatomyositis-associated interstitial lung disease. N Engl J Med 2019; 381 (03) 291-293
72 Ye S, Chen XX, Lu XY. et al. Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study. Clin Rheumatol 2007; 26 (10) 1647-1654
73 Won Huh J, Soon Kim D, Keun Lee C. et al. Two distinct clinical types of interstitial lung disease associated with polymyositis-dermatomyositis. Respir Med 2007; 101 (08) 1761-1769
74 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 (05) 896-906
75 Mira-Avendano IC, Parambil JG, Yadav R. et al. A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis/dermatomyositis. Respir Med 2013; 107 (06) 890-896
76 Hanaoka H, Iida H, Kiyokawa T, Takakuwa Y, Kawahata K. Mycophenolate mofetil treatment with or without a calcineurin inhibitor in resistant inflammatory myopathy. Clin Rheumatol 2019; 38 (02) 585-590
77 Koyama RVL, Braga TKK, da Silva Dias GA, Fujihara S, Fuzii HT, Yoshikawa GT. Hypomyopathic dermatomyositis associated with interstitial lung disease and good response to mycophenolate mofetil: case-based review. Clin Rheumatol 2017; 36 (08) 1919-1926
78 Keir GJ, Maher TM, Hansell DM. et al. Severe interstitial lung disease in connective tissue disease: rituximab as rescue therapy. Eur Respir J 2012; 40 (03) 641-648
79 Sem M, Molberg O, Lund MB, Gran JT. Rituximab treatment of the anti-synthetase syndrome: a retrospective case series. Rheumatology (Oxford) 2009; 48 (08) 968-971
80 Bauhammer J, Blank N, Max R. et al. Rituximab in the treatment of Jo1 antibody-associated antisynthetase syndrome: anti-Ro52 positivity as a marker for severity and treatment response. J Rheumatol 2016; 43 (08) 1566-1574
81 Doyle TJ, Dhillon N, Madan R. et al. Rituximab in the treatment of interstitial lung disease associated with antisynthetase syndrome: a multicenter retrospective case review. J Rheumatol 2018; 45 (06) 841-850
82 Jang Y, Yoon HY, Kim HS. The efficacy and safety of rituximab in patients with idiopathic inflammatory myopathy-associated interstitial lung disease: case series. J Clin Med 2023; 12 (10) 3406
83 Allenbach Y, Guiguet M, Rigolet A. et al. Efficacy of rituximab in refractory inflammatory myopathies associated with anti- synthetase auto-antibodies: an open-label, phase II trial. PLoS One 2015; 10 (11) e0133702
84 He C, Li W, Xie Q, Yin G. Rituximab in the treatment of interstitial lung diseases related to anti-melanoma differentiation-associated gene 5 dermatomyositis: a systematic review. Front Immunol 2022; 12: 820163
85 Hallowell RW, Danoff SK. Diagnosis and management of myositis-associated lung disease. Chest 2023; 163 (06) 1476-1491
86 Go DJ, Park JK, Kang EH. et al. Survival benefit associated with early cyclosporine treatment for dermatomyositis-associated interstitial lung disease. Rheumatol Int 2016; 36 (01) 125-131
87 Cavagna L, Caporali R, Abdì-Alì L, Dore R, Meloni F, Montecucco C. Cyclosporine in anti-Jo1-positive patients with corticosteroid-refractory interstitial lung disease. J Rheumatol 2013; 40 (04) 484-492
88 Labirua-Iturburu A, Selva-O'Callaghan A, Martínez-Gómez X, Trallero-Araguás E, Labrador-Horrillo M, Vilardell-Tarrés M. Calcineurin inhibitors in a cohort of patients with antisynthetase-associated interstitial lung disease. Clin Exp Rheumatol 2013; 31 (03) 436-439
89 Oddis CV, Sciurba FC, Elmagd KA, Starzl TE. Tacrolimus in refractory polymyositis with interstitial lung disease. Lancet 1999; 353 (9166): 1762-1763
90 Takada K, Nagasaka K, Miyasaka N. Polymyositis/dermatomyositis and interstitial lung disease: a new therapeutic approach with T-cell-specific immunosuppressants. Autoimmunity 2005; 38 (05) 383-392
91 Fujisawa T, Hozumi H, Kamiya Y. et al. Prednisolone and tacrolimus versus prednisolone and cyclosporin A to treat polymyositis/dermatomyositis-associated ILD: a randomized, open-label trial. Respirology 2021; 26 (04) 370-377
92 Kameda H, Nagasawa H, Ogawa H. et al. Combination therapy with corticosteroids, cyclosporin A, and intravenous pulse cyclophosphamide for acute/subacute interstitial pneumonia in patients with dermatomyositis. J Rheumatol 2005; 32 (09) 1719-1726
93 Tsuji H, Nakashima R, Hosono Y. et al. Multicenter prospective study of the efficacy and safety of combined immunosuppressive therapy with high-dose glucocorticoid, tacrolimus, and cyclophosphamide in interstitial lung diseases accompanied by anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Arthritis Rheumatol 2020; 72 (03) 488-498
94 Hornig J, Weinhage T, Schmidt LH. et al. [Response of dermatomyositis with lung involvement to Janus kinase inhibitor treatment]. Z Rheumatol 2018; 77 (10) 952-957
95 Ishikawa Y, Kasuya T, Fujiwara M, Kita Y. Tofacitinib for recurrence of antimelanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis after remission: a case report. Medicine (Baltimore) 2020; 99 (37) e21943
96 Kato M, Ikeda K, Kageyama T. et al. Successful treatment for refractory interstitial lung disease and pneumomediastinum with multidisciplinary therapy including tofacitinib in a patient with anti-MDA5 antibody-positive dermatomyositis. J Clin Rheumatol 2021; 27 (8S): S574-S577
97 Kurasawa K, Arai S, Namiki Y. et al. Tofacitinib for refractory interstitial lung diseases in anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis. Rheumatology (Oxford) 2018; 57 (12) 2114-2119
98 Wang Y, Luo J, Lv X. et al. Tofacitinib for new-onset adult patients with anti-melanoma differentiation-associated 5 gene antibody positive dermatomyositis. Clin Rheumatol 2023; 42 (07) 1847-1853
99 Li S, Li S, Wang J. et al. Efficacy and safety of tofacitinib in anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis. J Clin Rheumatol 2023; 29 (06) 281-284
100 Ida T, Furuta S, Takayama A. et al. Efficacy and safety of dose escalation of tofacitinib in refractory anti-MDA5 antibody-positive dermatomyositis. RMD Open 2023; 9 (01) e002795
101 Baumann Benvenuti F, Dudler J. Long-lasting improvement of refractory antisynthetase syndrome with tocilizumab: a report of two cases. RMD Open 2023; 9 (04) e003599
102 Su CF, Liao HT, Tsai CY. Tocilizumab and rituximab for anti-MDA-5 positive amyopathic dermatomyositis complicated with macrophage activation syndrome and progressive fibrosing interstitial lung disease. Scand J Rheumatol 2022; 51 (02) 166-168
103 Teng F, Peng JM, Wang Q, Tian XL, Huo Z, Weng L. Successful treatment with tocilizumab in a patient with rapidly progressive interstitial lung disease with positive anti-melanoma differentiation-associated gene-5 antibody. Chin Med J (Engl) 2020; 134 (08) 999-1000
104 Murphy SM, Lilleker JB, Helliwell P, Chinoy H. The successful use of tocilizumab as third-line biologic therapy in a case of refractory anti-synthetase syndrome. Rheumatology (Oxford) 2016; 55 (12) 2277-2278
105 Li T, Guo L, Chen Z. et al. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis. Sci Rep 2016; 6: 33226
106 Gandiga PC, Ghetie D, Anderson E, Aggrawal R. Intravenous immunoglobulin in idiopathic inflammatory myopathies: a practical guide for clinical use. Curr Rheumatol Rep 2023; 25 (08) 152-168
107 Hallowell RW, Amariei D, Danoff SK. Intravenous immunoglobulin as potential adjunct therapy for interstitial lung disease. Ann Am Thorac Soc 2016; 13 (10) 1682-1688
108 Huapaya JA, Hallowell R, Silhan L. et al. Long-term treatment with human immunoglobulin for antisynthetase syndrome-associated interstitial lung disease. Respir Med 2019; 154: 6-11
109 Suzuki Y, Hayakawa H, Miwa S. et al. Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis. Lung 2009; 187 (03) 201-206
110 Diot E, Carmier D, Marquette D, Marchand-Adam S, Diot P, Lesire V. IV immunoglobulin might be considered as a first-line treatment of severe interstitial lung disease associated with polymyositis. Chest 2011; 140 (02) 562-563
111 Hamada-Ode K, Taniguchi Y, Kimata T. et al. High-dose intravenous immunoglobulin therapy for rapidly progressive interstitial pneumonitis accompanied by anti-melanoma differentiation-associated gene 5 antibody-positive amyopathic dermatomyositis. Eur J Rheumatol 2015; 2 (02) 83-85
112 Wang LM, Yang QH, Zhang L. et al. Intravenous immunoglobulin for interstitial lung diseases of anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Rheumatology (Oxford) 2022; 61 (09) 3704-3710
113 Bakewell CJ, Raghu G. Polymyositis associated with severe interstitial lung disease: remission after three doses of IV immunoglobulin. Chest 2011; 139 (02) 441-443
114 Fujita Y, Fukui S, Suzuki T. et al. Anti-MDA5 antibody-positive dermatomyositis complicated by autoimmune-associated hemophagocytic syndrome that was successfully treated with immunosuppressive therapy and plasmapheresis. Intern Med 2018; 57 (23) 3473-3478
115 Sasaki N, Ishii A, Kurabayashi T. et al. Early initiation of plasma exchange therapy for a patient with anti-MDA5 autoantibody-positive dermatomyositis developing rapidly progressive interstitial lung disease. Mod Rheumatol Case Rep 2021; 5 (01) 87-94
116 Shirakashi M, Nakashima R, Tsuji H. et al. Efficacy of plasma exchange in anti-MDA5-positive dermatomyositis with interstitial lung disease under combined immunosuppressive treatment. Rheumatology (Oxford) 2020; 59 (11) 3284-3292
117 Abatacept for the Treatment of Myositis-associated Interstitial Lung Disease (ATtackMy-ILD). National Institute of Health. clinicaltrials.gov.
118 Myositis Interstitial Lung Disease Nintedanib Trial (MINT). National Institute of Health (NIH). https://clinicaltrials.gov/
119 Negrini S, Emmi G, Greco M. et al. Sjögren's syndrome: a systemic autoimmune disease. Clin Exp Med 2022; 22 (01) 9-25
120 Luppi F, Sebastiani M, Silva M. et al. Interstitial lung disease in Sjogren's syndrome: a clinical review. Clin Exp Rheumatol 2020; 38 Suppl 126(4): 291-300
121 Sambataro G, Ferro F, Orlandi M. et al. Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjӧgren's syndrome: a systematic review from the Italian Society of Rheumatology. Autoimmun Rev 2020; 19 (02) 102447
122 Palm O, Garen T, Berge Enger T. et al. Clinical pulmonary involvement in primary Sjogren's syndrome: prevalence, quality of life and mortality–a retrospective study based on registry data. Rheumatology (Oxford) 2013; 52 (01) 173-179
123 Ramos-Casals M, Brito-Zerón P, Seror R. et al. EULAR Sjögren Syndrome Task Force. Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology (Oxford) 2015; 54 (12) 2230-2238
124 Dong X, Zhou J, Guo X. et al. A retrospective analysis of distinguishing features of chest HRCT and clinical manifestation in primary Sjögren's syndrome-related interstitial lung disease in a Chinese population. Clin Rheumatol 2018; 37 (11) 2981-2988
125 Seror R, Bowman SJ, Brito-Zeron P. et al. EULAR Sjögren's syndrome disease activity index (ESSDAI): a user guide. RMD Open 2015; 1 (01) e000022
126 Seror R, Ravaud P, Mariette X. et al. EULAR Sjögren's Task Force. EULAR Sjogren's syndrome patient reported index (ESSPRI): development of a consensus patient index for primary Sjogren's syndrome. Ann Rheum Dis 2011; 70 (06) 968-972
127 Amlani B, Elsayed G, Barvalia U. et al. Treatment of primary Sjögren's syndrome-related interstitial lung disease: a retrospective cohort study. Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (02) 136-147
128 Roca F, Dominique S, Schmidt J. et al. Interstitial lung disease in primary Sjögren's syndrome. Autoimmun Rev 2017; 16 (01) 48-54
129 Seror R, Nocturne G, Mariette X. Current and future therapies for primary Sjögren syndrome. Nat Rev Rheumatol 2021; 17 (08) 475-486
130 Devauchelle-Pensec V, Pennec Y, Morvan J. et al. Improvement of Sjögren's syndrome after two infusions of rituximab (anti-CD20). Arthritis Rheum 2007; 57 (02) 310-317
131 Gottenberg JE, Cinquetti G, Larroche C. et al. Club Rhumatismes et Inflammations and the French Society of Rheumatology. Efficacy of rituximab in systemic manifestations of primary Sjogren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry. Ann Rheum Dis 2013; 72 (06) 1026-1031
132 Chen MH, Chen CK, Chou HP, Chen MH, Tsai CY, Chang DM. Rituximab therapy in primary Sjögren's syndrome with interstitial lung disease: a retrospective cohort study. Clin Exp Rheumatol 2016; 34 (06) 1077-1084
133 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 (05) 640-646
134 Oldham JM, Lee C, Valenzi E. et al. Azathioprine response in patients with fibrotic connective tissue disease-associated interstitial lung disease. Respir Med 2016; 121: 117-122
135 Saunders P, Tsipouri V, Keir GJ. 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 (01) 275
136 Mankikian J, Caille A, Reynaud-Gaubert M. et al. EVER-ILD investigators and the OrphaLung network. Rituximab and mycophenolate mofetil combination in patients with interstitial lung disease (EVER-ILD): a double-blind, randomised, placebo-controlled trial. Eur Respir J 2023; 61 (06) 2202071
137 Flaherty KR, Wells AU, Cottin V. et al. INBUILD Trial Investigators. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 2019; 381 (18) 1718-1727
138 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 (06) 1039-1047
139 Behr J, Prasse A, Kreuter M. et al. RELIEF investigators. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med 2021; 9 (05) 476-486
140 Wang J, Wang X, Qi X. et al. The efficacy and safety of pirfenidone combined with immunosuppressant therapy in connective tissue disease-associated interstitial lung disease: a 24-week prospective controlled cohort study. Front Med (Lausanne) 2022; 9: 871861
141 Müller F, Taubmann J, Bucci L. et al. CD19 CAR T-cell therapy in autoimmune disease - a case series with follow-up. N Engl J Med 2024; 390 (08) 687-700
142 Safety and Efficacy of CD19 Targeted CAR-T Therapy for Refractory Autoimmune Disease. clinicaltrials.gov.