{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T01:19:37Z","timestamp":1792718377929,"version":"4.5.0"},"reference-count":44,"publisher":"Walter de Gruyter GmbH","issue":"3","license":[{"start":{"date-parts":[[2024,9,1]],"date-time":"2024-09-01T00:00:00Z","timestamp":1725148800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,21]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Systemic sclerosis (SSc) or scleroderma is an autoimmune disease characterized by immune dysregulation which leads to progressive fibrosis of the skin and internal organs. Interstitial lung disease (ILD) is present in approximately 65% of patients with SSc and it accounts for approximately 40% of all SSc deaths. Risk factors associated with the development of systemic sclerosis related interstitial lung disease (SSc-ILD) include male sex, African heritage, high modified Rodnan skin score (mRSS), presence of anti-Scl-70\/Topoisomerase I antibodies, and nucleolar pattern on antinuclear antibody (ANA). The primary tool to diagnose ILD in patients with SSc is high-resolution computed tomography (HRCT). Full pulmonary function tests (PFTs) with diffusing capacity of the lungs for carbon monoxide (DLco) and ambulatory desaturation testing should be obtained following the diagnosis of SSc-ILD for disease monitoring.<\/jats:p>\n               <jats:p>The purpose of this review is to provide an updated guide for the management of SSc-ILD. Our proposed first line treatment for SSc-ILD is immunosuppressive therapy such as mycophenolate mofetil, tocilizumab, and rituximab which are discussed in depth, and we present the evidence-based data that has justified the use of these pharmacotherapies. Other immunosuppressive treatments are also reviewed, and we discuss the role of antifibrotic therapy. Finally, we dive into other avenues of treatments such as chimeric antigen receptor (CAR)-T cell therapy and hematopoietic stem cell transplant.<\/jats:p>","DOI":"10.2478\/rir-2024-0022","type":"journal-article","created":{"date-parts":[[2024,10,21]],"date-time":"2024-10-21T13:21:33Z","timestamp":1729516893000},"page":"157-165","source":"Crossref","is-referenced-by-count":9,"title":["Systemic sclerosis-associated interstitial lung disease: How to manage in 2024?"],"prefix":"10.2478","volume":"5","author":[{"given":"Rocio","family":"Bautista-Sanchez","sequence":"first","affiliation":[{"name":"Division of Rheumatology, University of Michigan , Ann Arbor, MI , Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dinesh","family":"Khanna","sequence":"additional","affiliation":[{"name":"Scleroderma Program, University of Michigan , Ann Arbor, MI , Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","published-online":{"date-parts":[[2024,10,21]]},"reference":[{"key":"2024102315010208193_j_rir-2024-0022_ref_001","doi-asserted-by":"crossref","unstructured":"Denton CP, Khanna D. Systemic Sclerosis. The Lancet. 2017;390:1685\u20131699.","DOI":"10.1016\/S0140-6736(17)30933-9"},{"key":"2024102315010208193_j_rir-2024-0022_ref_002","doi-asserted-by":"crossref","unstructured":"Allanore Y, Simms R, Distler O, et al. Nature Reviews disease primers. [Online] 2015 [cited 2024 January 17. Available from: https:\/\/doi.org\/10.1038\/nrdp.2015.2.","DOI":"10.1038\/nrdp.2015.2"},{"key":"2024102315010208193_j_rir-2024-0022_ref_003","doi-asserted-by":"crossref","unstructured":"Rahaghi FF, Hsu VM, Kaner RJ, et al. Expert consensus on the management of systemic sclerosis-associated interstitial lung disease. Respir Res. 2023;24:6.","DOI":"10.1186\/s12931-022-02292-3"},{"key":"2024102315010208193_j_rir-2024-0022_ref_004","doi-asserted-by":"crossref","unstructured":"Hoffmann-Vold AM, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. Lancet Rheumatol. 2020;2:e71\u2013e83.","DOI":"10.1016\/S2665-9913(20)30098-9"},{"key":"2024102315010208193_j_rir-2024-0022_ref_005","doi-asserted-by":"crossref","unstructured":"Bernstein EJ, Jaafar S, Assassi S, et al. Performance Characteristics of Pulmonary Function Tests for the Detection of Interstitial Lung Disease in Adults With Early Diffuse Cutaneous Systemic Sclerosis. Arthritis Rheumatol. 2020;72:1892\u20131896.","DOI":"10.1002\/art.41415"},{"key":"2024102315010208193_j_rir-2024-0022_ref_006","doi-asserted-by":"crossref","unstructured":"Roofeh D, Khanna D. Management of systemic sclerosis: the first five years. Curr Opin Rheumatol. 2020;32:228\u2013237.","DOI":"10.1097\/BOR.0000000000000711"},{"key":"2024102315010208193_j_rir-2024-0022_ref_007","doi-asserted-by":"crossref","unstructured":"Reveille JD, Solomon DH. Evidence-Based Guidelines for the Use of Immunologic Tests: Anticentromere, Scl-70, and Nucleolar Antibodies. Arthritis & Rheumatism (Arthritis Care & Research). 200;49:399\u2013412.","DOI":"10.1002\/art.11113"},{"key":"2024102315010208193_j_rir-2024-0022_ref_008","unstructured":"Petelytska l, Velauthapilla A, Tofani L, et al. Incidence and Risk Factors for New Onset of Interstitial Lung Disease in Systemic Sclerosis: A EUSTAR Analysis [Abstract]. Arthritis & Rheumatology. 2023;75. Available from: https:\/\/acrabstracts.org\/abstract\/incidence-and-risk-factors-for-new-onset-of-interstitial-lung-disease-in-systemic-sclerosis-a-eustar-analysis\/."},{"key":"2024102315010208193_j_rir-2024-0022_ref_009","unstructured":"American College of Rheumatology. American College of Rheumatology. [Online]; 2023 [cited 2024 January 19. Available from: https:\/\/assets.contentstack.io\/v3\/assets\/bltee37abb6b278ab2c\/ blt7e2cadfc7bc986fb\/interstitial-lung-disease-guideline-summary-screening-monitoring-2023.pdf."},{"key":"2024102315010208193_j_rir-2024-0022_ref_010","doi-asserted-by":"crossref","unstructured":"Pope JE, Denton CP, Johnson SR, et al. State-of-the-art evidence in the treatment of systemic sclerosis. Nat Rev Rheumatol. 2023;19:212\u2013226.","DOI":"10.1038\/s41584-023-00909-5"},{"key":"2024102315010208193_j_rir-2024-0022_ref_011","doi-asserted-by":"crossref","unstructured":"Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177:1248\u20131254.","DOI":"10.1164\/rccm.200706-877OC"},{"key":"2024102315010208193_j_rir-2024-0022_ref_012","doi-asserted-by":"crossref","unstructured":"Khanna D, Lescoat A, Roofeh D, et al. Systemic Sclerosis-Associated Interstitial Lung Disease: How to Incorporate Two Food and Drug Administration-Approved Therapies in Clinical Practice. Arthritis Rheumatol. 2022;74:13\u201327.","DOI":"10.1002\/art.41933"},{"key":"2024102315010208193_j_rir-2024-0022_ref_013","doi-asserted-by":"crossref","unstructured":"Panopoulos ST, Bournia VK, Trakada G, et al. Mycophenolate versus cyclophosphamide for progressive interstitial lung disease associated with systemic sclerosis: a 2-year case control study. Lung. 2013;191:483\u2013489.","DOI":"10.1007\/s00408-013-9499-8"},{"key":"2024102315010208193_j_rir-2024-0022_ref_014","doi-asserted-by":"crossref","unstructured":"Broen JCA, van Laar JM. Mycophenolate mofetil, azathioprine and tacrolimus: mechanisms in rheumatology. Nat Rev Rheumatol. 2020;16:167\u2013178.","DOI":"10.1038\/s41584-020-0374-8"},{"key":"2024102315010208193_j_rir-2024-0022_ref_015","doi-asserted-by":"crossref","unstructured":"Tashkin 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\u2013719.","DOI":"10.1016\/S2213-2600(16)30152-7"},{"key":"2024102315010208193_j_rir-2024-0022_ref_016","doi-asserted-by":"crossref","unstructured":"Raghu G, Montesi SB, Silver RM, et al. Treatment of Systemic Sclerosis-associated Interstitial Lung Disease: Evidencebased Recommendations. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2024;209:137\u2013152.","DOI":"10.1164\/rccm.202306-1113ST"},{"key":"2024102315010208193_j_rir-2024-0022_ref_017","doi-asserted-by":"crossref","unstructured":"Sime\u00f3n-Aznar CP, Fonollosa-Pl\u00e1 V, Tolosa-Vilella C, et al. Effect of mycophenolate sodium in scleroderma-related interstitial lung disease. 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