{"doi":"10.1002/art.41933","title":"Systemic Sclerosis–Associated Interstitial Lung Disease: How to Incorporate Two Food and Drug Administration–Approved Therapies in Clinical Practice","abstract":"Systemic sclerosis (SSc; scleroderma) has the highest individual mortality of all rheumatic diseases, and interstitial lung disease (ILD) is among the leading causes of SSc-related death. Two drugs are now approved by the US Food and Drug Administration (FDA) and indicated for slowing the rate of decline in pulmonary function in patients with SSc-associated ILD (SSc-ILD): nintedanib (a tyrosine kinase inhibitor) and tocilizumab (the first biologic agent targeting the interleukin-6 pathway in SSc). In addition, 2 generic drugs with cytotoxic and immunoregulatory activity, mycophenolate mofetil and cyclophosphamide, have shown comparable efficacy in a phase II trial but are not FDA-approved for SSc-ILD. In light of the heterogeneity of the disease, the optimal therapeutic strategy for the management of SSc-ILD is still to be determined. The objectives of this review are 2-fold: 1) review the body of research focused on the diagnosis and treatment of SSc-ILD; and 2) propose a practical approach for diagnosis, stratification, management, and therapeutic decision-making in this clinical context. This review presents a practical classification of SSc patients in terms of disease severity (subclinical versus clinical ILD) and associated risk of progression (low versus high risk). The pharmacologic and nonpharmacologic options for first- and second-line therapy, as well as potential combination approaches, are discussed in light of the recent approval of tocilizumab for SSc-ILD.","journal":"Arthritis & Rheumatology","year":2021,"id":148606,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":123,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9546,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2021-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":632333,"name":"Alain Lescoat","orcid":"0000-0003-2081-8558","position":1,"is_corresponding":false},{"id":623917,"name":"David Roofeh","orcid":"0000-0002-9750-5771","position":2,"is_corresponding":false},{"id":332664,"name":"Elana J. Bernstein","orcid":"0000-0001-5560-6390","position":3,"is_corresponding":false},{"id":309299,"name":"Ella A. Kazerooni","orcid":"0000-0001-5859-8744","position":4,"is_corresponding":false},{"id":348233,"name":"Michael D. Roth","orcid":"0000-0003-2194-6578","position":5,"is_corresponding":false},{"id":33125,"name":"Fernando J. Martínez","orcid":"0000-0002-2412-3182","position":6,"is_corresponding":false},{"id":278241,"name":"Kevin R. Flaherty","orcid":"0000-0001-7686-0291","position":7,"is_corresponding":false},{"id":7275,"name":"Christopher P. Denton","orcid":"0000-0003-3975-8938","position":8,"is_corresponding":false},{"id":249352,"name":"Dinesh Khanna","orcid":"0000-0003-1412-4453","position":0,"is_corresponding":true}],"reference_count":88,"raw_metadata":null,"created_at":"2026-07-18T23:42:46.224799Z","pmid":"34313399","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}