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.

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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
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article

Efficacy of Janus kinase inhibitors in the treatment of acute exacerbations of rheumatoid arthritis–associated interstitial lung disease: a retrospective observational case series

Shinya Tomari, Tomo Mihara, Keita Fujikawa, Takashi Kido et al.
Frontiers in Immunology
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
article

Efficacy of Janus kinase inhibitors in the treatment of acute exacerbations of rheumatoid arthritis–associated interstitial lung disease: a retrospective observational case series

Shinya Tomari, Tomo Mihara, Keita Fujikawa, Takashi Kido, Seiji Doi, Momoko Okamoto, Akinari Mizokami, Hiroshi Mukae, Noriho Sakamoto, Atsushi Kawakami, Tomohiro Koga
article en

Abstract

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.

Frontiers in ImmunologyVol. 17
Japan Community Healthcare Organization (JP), Nagasaki University (JP)
Good health and well-being
Openalex Percentile: Top 27%
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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