{
  "abstract": "Background: Anti-synthetase syndrome-associated ILD (ASyS-ILD) spans a spectrum from active inflammation to established fibrosis; IL-6 pathway involvement supports a rationale for tocilizumab, yet phenotype-specific efficacy data are lacking.\n\nMethods: Thirty-two patients with refractory ASyS-ILD received intravenous tocilizumab (8 mg/kg every 4 weeks) for ≥ 3 months. Patients were stratified into three phenotypes: glucocorticoid-dependent (n = 8, unable to taper prednisone below 15 mg/day without relapse), glucocorticoid-resistant (n = 18, failure to achieve remission despite ≥ 3 months of induction therapy), and progressive pulmonary fibrosis (PPF, n = 6, fulfilling ≥ 2 symptom/physiological/radiological progression criteria within 12 months on stable immunosuppression without active inflammation). Changes in lung function, high-resolution computed tomography (HRCT) scores and glucocorticoid dosage were analyzed using generalised estimating equations and paired tests.\n\nResults: Improvements in forced vital capacity (FVC)% predicted, diffusing capacity for carbon monoxide (DLCO)% predicted, and HRCT scores were observed at 3 months and sustained at 6 months. Among the glucocorticoid-dependent group, 7/8 patients (87.5%) tapered prednisone to ≤ 10 mg/day. Sixteen of 18 glucocorticoid-resistant patients (88.9%) achieved treatment response. In the PPF subgroup, aggregate lung function did not improve from baseline; however, 5/6 patients (83.3%) showed FVC trajectories shifting from decline toward stabilisation or improvement (mean slope difference, + 0.81% predicted/month; 95% CI, -0.11 to 1.73; p = .073). No severe adverse events or treatment-related deaths occurred.\n\nConclusions: In this retrospective study, tocilizumab was associated with glucocorticoid tapering and reduced disease activity in glucocorticoid-dependent and -resistant patients. In the PPF subgroup, lung function did not change significantly on between-timepoint analysis, and a slope-based analysis suggested possible attenuation of decline that did not reach statistical significance. These hypothesis-generating findings provide a phenotype-based rationale for prospective controlled trials.\n\nSupplementary Information: The online version contains supplementary material available at https://doi.org/10.1186/s12931-026-03839-4.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Rheumatology; Beijing Key Laboratory of Precision Diagnosis, Treatment and Innovative Translation in Inflammatory Myopathies, China-Japan Friendship Hospital, No.2 Yinghua East Road, Chaoyang District, Beijing, 100029 China"
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 12,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Hanbo Yang"
    },
    {
      "affs": [
        0
      ],
      "name": "Yongpeng Ge"
    },
    {
      "affs": [
        0
      ],
      "name": "Sizhao Li"
    },
    {
      "affs": [
        0
      ],
      "name": "WenLi Li"
    },
    {
      "affs": [
        0
      ],
      "name": "Linrong He"
    },
    {
      "affs": [
        0
      ],
      "name": "Fang Chen"
    },
    {
      "affs": [
        0
      ],
      "name": "Wei Jiang"
    },
    {
      "affs": [
        0
      ],
      "name": "Xiaoqin Luo"
    },
    {
      "affs": [
        0
      ],
      "name": "Xiaoming Shu"
    },
    {
      "affs": [
        0
      ],
      "name": "Qinglin Peng"
    },
    {
      "affs": [
        0
      ],
      "name": "Guochun Wang"
    },
    {
      "affs": [
        0
      ],
      "name": "Xin Lu"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/e3c707rd9v986.bib",
  "cited_by": 0,
  "doi": "10.1186/s12931-026-03839-4",
  "fulltext_url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC13733139/",
  "id": "e3c707rd9v986",
  "issns": [
    "1465-9921",
    "1465-993X"
  ],
  "landing_url": "https://doi.org/10.1186/s12931-026-03839-4",
  "language": "en",
  "licence": "CC-BY-NC-ND-4.0",
  "licence_label": "CC BY-NC-ND 4.0",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by-nc-nd/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "heading",
      "Research"
    ],
    [
      "openalex",
      "2745|Inflammatory Myopathies and Dermatomyositis"
    ]
  ],
  "openalex": "W7172228777",
  "pdf_url": null,
  "pmcid": "PMC13733139",
  "pmid": "42983497",
  "published": "2026-08-03",
  "publisher": "BMC",
  "ris": "https://research.draconitic.com/paper/e3c707rd9v986.ris",
  "sources": [
    {
      "first_seen": "2026-11-08T12:22:59Z",
      "native_id": "PMC13733139",
      "source": "pmc",
      "url": "https://doi.org/10.1186/s12931-026-03839-4"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2745|Inflammatory Myopathies and Dermatomyositis",
      "subfield": "general"
    }
  ],
  "title": "Tocilizumab in refractory anti-synthetase syndrome-associated interstitial lung disease:a phenotype-stratified cohort study",
  "title_text": "Tocilizumab in refractory anti-synthetase syndrome-associated interstitial lung disease:a phenotype-stratified cohort study",
  "type": "peer-reviewed",
  "updated_at": "2026-11-08T12:22:59Z",
  "url": "https://research.draconitic.com/paper/e3c707rd9v986",
  "venue": "Respiratory Research",
  "versions": []
}