Efficacy of Janus kinase inhibitors in the treatment of acute exacerbations of rheumatoid arthritis–associated interstitial lung disease: a retrospective observational case series
Background: Acute exacerbation of rheumatoid arthritis-associated interstitial lung disease (AE-RA-ILD) is a life-threatening condition for which no standard therapy has been established. Although Janus kinase (JAK) inhibitors are effective for treating rheumatoid arthritis (RA) and have shown potential benefit in chronic RA-ILD, evidence supporting their use in AE-RA-ILD remains extremely limited. Methods: This retrospective observational study included consecutive patients hospitalized for AE-RA-ILD who received JAK inhibitors alongside systemic glucocorticoids between January and December 2024. Clinical characteristics, laboratory data, treatment regimens, and outcomes were extracted from medical records. High-resolution computed tomography images were quantitatively evaluated at three time points-before acute exacerbation (AE), at exacerbation onset, and after treatment-using a standardized computed tomography (CT) scoring system. Results: ratio. The total CT score increased from baseline (175.3 ± 50.3) to AE onset (247.2 ± 53.6) and subsequently decreased after treatment (220.1 ± 58.6), with notable improvement in fibrotic lesion components. All patients survived during the 3-month observation period, although four patients experienced a decline in percent predicted forced vital capacity, and three required home oxygen therapy at discharge. Conclusion: In this retrospective case series, adjunctive treatment with JAK inhibitors was associated with improvements in respiratory status and radiological findings in patients with AE-RA-ILD. These findings suggest that JAK inhibitors may represent a promising therapeutic option for this severe condition and support the need for further investigation in larger, prospective studies.
Authors
- Shinya Tomari (ORCID: https://orcid.org/0000-0001-9680-1723)
- Tomo Mihara
- Keita Fujikawa
- Takashi Kido (ORCID: https://orcid.org/0000-0001-8338-0608)
- Seiji Doi
- Momoko Okamoto
- Akinari Mizokami
- Hiroshi Mukae
- Noriho Sakamoto
- Atsushi Kawakami
- Tomohiro Koga
Institutions
- Japan Community Healthcare Organization (JP)
- Nagasaki University (JP)
Publication Details
- Journal
- Frontiers in Immunology
- Published
- 2026-04-13
- DOI
- https://doi.org/10.3389/fimmu.2026.1800161
- Primary Topic
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Type
- article
- Field-Weighted Citation Impact
- 0.00