University of Oulu

Nurmi HM, Elfving PK, Kettunen HP, Suoranta SK, Järvinen HMI, Kuittinen VAE, Purokivi MK, Kaarteenaho RL, Koskela HO. Inflammatory myositis-associated interstitial lung disease can be distinguished from that associated with other connective tissue diseases. J Thorac Dis 2023;15(2):311-322.

Inflammatory myositis-associated interstitial lung disease can be distinguished from that associated with other connective tissue diseases

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Author: Nurmi, Hanna M.1,2; Elfving, Pia K.3,4; Kettunen, Hannu-Pekka5;
Organizations: 1Division of Respiratory Medicine, Institute of Clinical Medicine, University of Eastern Finland, Kuopio, Finland
2Center of Medicine and Clinical Research, Department of Respiratory Medicine, Kuopio University Hospital, Kuopio, Finland
3Department of Medicine, Kuopio University hospital, Kuopio, Finland
4Institute of Clinical Medicine, University of Eastern Finland, Kuopio, Finland
5Department of Clinical Radiology, Kuopio University Hospital, Kuopio, Finland
6Institute of Clinical Radiology, University of Eastern Finland, Kuopio, Finland
7Research Unit of Internal Medicine, University of Oulu and Center of Internal Medicine and Respiratory Medicine, Medical Research Center Oulu, Oulu University Hospital, Oulu, Finland
Format: article
Version: published version
Access: open
Online Access: PDF Full Text (PDF, 0.7 MB)
Persistent link:
Language: English
Published: AME Publishing Company, 2023
Publish Date: 2023-08-21


Background: Acute onset of interstitial lung disease (ILD) has been described in patients with idiopathic inflammatory myositis (IIM), but controlled studies about this issue are sparse. The aim of this study was to compare disease onset, demographics, and high-resolution computed tomography (HRCT) patterns in IIM-ILD and other connective tissue disease (CTD)-ILDs.

Methods: Clinical and radiological data of 22 IIM-ILD and 132 other CTD-ILD patients was retrospectively gathered from hospital registries between January 2000 and November 2019. Data was re-assessed and compared using a multivariate analysis.

Results: Compared to other CTD-ILDs, the patients with IIM-ILD were younger (59.7 vs. 68.0 years, P=0.023), more often non-smokers (71.4% vs. 45.7%, P=0.029) and displayed radiological nonspecific interstitial pneumonia/organizing pneumonia (NSIP/OP) overlap pattern more frequently (27.3% vs. 1.5%, P<0.001). The onset of ILD was acute with patients needing intensive care significantly more often in IIM-ILD than in other CTD-ILDs (22.7% vs. 2.3%, P<0.001). In most patients ILD was diagnosed before or simultaneously with IIM presentation unlike in other CTD-ILDs (90.9% vs. 47.7%, P<0.001). In multivariate analysis, NSIP/OP overlap pattern, acute onset disease treated in intensive care unit and ILD preceding or being diagnosed simultaneously with CTD were significantly associated with IIM-ILD. The multivariate model, supplemented with age, had excellent diagnostic performance identifying IIM-ILD [area under curve (AUC) 0.845].

Conclusions: Unlike other CTD-ILDs, IIM-ILD often develops acutely, simultaneously with the systemic disease. Therefore, clinicians should consider IIM-ILD as an option of differential diagnosis in patients with acute ILD and promptly test muscle enzymes as well as comprehensive autoantibody tests.

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Series: Journal of thoracic disease
ISSN: 2072-1439
ISSN-E: 2077-6624
ISSN-L: 2072-1439
Volume: 15
Issue: 2
Pages: 311 - 322
DOI: 10.21037/jtd-22-1219
Type of Publication: A1 Journal article – refereed
Field of Science: 3121 General medicine, internal medicine and other clinical medicine
Funding: This work was supported by Foundation of the Finnish Anti-Tuberculosis Association and the North Savo Regional Fund of the Finnish Cultural Foundation to HN.
Copyright information: © Journal of Thoracic Disease. All rights reserved. This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: