Immunomodulatory treatment in unclassifiable interstitial lung disease: A retrospective study of treatment response

Background and Objective The optimal management of unclassifiable Interstitial lung disease (ILD) remains a challenge. The aim of this study was to describe pulmonary function trajectories for patients treated with immunomodulatory therapy and for untreated patients. Methods Clinical information and...

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Published in:Respirology (Carlton, Vic.) Vic.), 2023-04, Vol.28 (4), p.373-379
Main Authors: Hyldgaard, Charlotte, Torrisi, Sebastiano, Kronborg Brix‐White, Sissel, Prior, Thomas Skovhus, Ganter, Claudia, Bendstrup, Elisabeth, Kreuter, Michael
Format: Article
Language:English
Subjects:
Carbon monoxide
Corticosteroids
Humans
Immunomodulation
immunomodulatory therapy
Lung - diagnostic imaging
Lung diseases
Lung Diseases, Interstitial - drug therapy
lung fibrosis
Patients
pulmonary function
rare lung disease
Regression analysis
Respiratory function
Retrospective Studies
Tidal Volume
unclassifiable interstitial lung disease
Vital Capacity
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Summary:Background and Objective The optimal management of unclassifiable Interstitial lung disease (ILD) remains a challenge. The aim of this study was to describe pulmonary function trajectories for patients treated with immunomodulatory therapy and for untreated patients. Methods Clinical information and treatment data were obtained retrospectively at two ILD centres. Pulmonary function data were analysed using (1) mixed effects linear regression models with and without clinical covariates and (2) propensity score matching using gender, age, physiology (GAP) stage, smoking and presence of ground glass opacities. Results Sixty‐five percent of the 249 patients included received corticosteroids and/or other immunomodulators. Treated patients had lower forced vital capacity (FVC) (72% vs. 83% predicted) and diffusing capacity for carbon monoxide (DLco) (44% vs. 60% predicted). In mixed effects linear regression, the adjusted change in FVC was −0.22%, [−0.34; −0.11], and −0.15% [−0.28;‐0.012] for DLco. The difference in pulmonary function decline between treated and untreated patients was insignificant, −0.082% per month, [−0.28; 0.11], p = 0.10 for FVC and −0.14% per month, [−0.36; 0.079], p = 0.15, for DLco. In propensity score matched analysis, the difference in change in FVC was 0.039% per month, p = 0.12, and for DLco, 0.0085% per month, p = 0.7. Conclusion The pulmonary function trajectories for treated and untreated patients were parallel, despite treated patients having more severe disease at baseline. The persisting differences between the groups suggest no overall effect, although improvement or stabilization may be seen in some patients. Prospective studies are needed to define subsets of patients with unclassifiable interstitial lung disease and their optimal management. Patients with unclassifiable interstitial lung disease who received immunomodulatory therapy had similar pulmonary function trajectories as patients who did not receive therapy despite more severe disease at baseline in treated patients. This suggests a lack of overall improvement, although immunomodulatory therapy may have effect in a subgroup of patients.
ISSN:1323-7799
1440-1843
DOI:10.1111/resp.14409