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. 2025 Nov 19.
doi: 10.1007/s00330-025-12106-x. Online ahead of print.

CT features of interstitial lung disease in systemic lupus erythematosus and overlap lupus-other connective tissue disease

Aurore Sajust de Bergues de Escalup  1 , Arthur Mageau  2 , Lou Deneuville  3 , Karim Sacre  2 , Antoine Khalil  4 , Nathalie Costedoat-Chalumeau  5 , Eric Hachulla  6 , Yurdagul Uzunhan  7 , Erwan Le Tallec  8 , Jacques Cadranel  9 , Sylvain Marchand-Adam  10 , David Montani  11 , Martine Reynaud-Gaubert  12 , Grégoire Prévot  13 , Guillaume Beltramo  14 , Bruno Crestani  15 , Vincent Cottin  16 , Raphaël Borie  15 , Marie-Pierre Debray  17 ; OrphaLung Network
Collaborators, Affiliations

CT features of interstitial lung disease in systemic lupus erythematosus and overlap lupus-other connective tissue disease

Aurore Sajust de Bergues de Escalup et al. Eur Radiol. .

Abstract

Objectives: To describe initial and follow-up CT features of chronic interstitial lung disease (cILD) associated with systemic lupus erythematosus (SLE), focusing on variant signs of lung fibrosis.

Materials and methods: This multicentric retrospective study included 76 patients (72 females, 95%, median age: 46 years) with both cILD and SLE, including 46 patients having another connective tissue disease (CTD) associated with lupus. Two radiologists independently reviewed chest CTs for ILD pattern and variant fibrosis signs. Radiologic progression of ILD was analyzed in 52 patients. Pulmonary function tests were collected at baseline and follow-up if available. Data were analyzed using Fisher's exact and Wilcoxon's rank sum tests. Inter-reader agreement was assessed using Cohen's Kappa coefficients.

Results: The most frequent ILD patterns were non-specific interstitial pneumonia (NSIP) in 31/76 (41%) patients, among which 5 showed overlap organizing pneumonia features, indeterminate in 18/76 (24%) patients, and usual interstitial pneumonia in 10/76 (13%) patients. The overall inter-reader agreement for the CT pattern was moderate (κ = 0.52). Any variant sign of fibrosis, including anterior-upper-lobe sign, exuberant-honeycombing, island-like fibrosis, and straight-edge sign, was observed in 14/76 (18%) patients, with an overall inter-reader agreement of 0.62, highly variable according to the sign considered. After 100 months of median follow-up, CT showed signs of progressive ILD in 36/52 (69%) patients, with poor correlation between functional and radiological progression.

Conclusion: SLE-associated cILD most often showed an NSIP or indeterminate pattern, commonly associated with variant signs of lung fibrosis. After a median follow-up of 100 months, 69% of patients showed CT signs of progression.

Key points: Question Radiologic presentation and progression of cILD in SLE remain poorly defined, limiting the ability to guide long-term clinical management. Findings NSIP (more common in isolated lupus) and indeterminate patterns predominated, with variant fibrosis signs of variable reproducibility. Two-thirds of patients showed progressive ILD. Clinical relevance CT helps characterize and monitor lupus-associated interstitial lung disease, but limited pattern reproducibility and weak correlation with functional decline support the need for multidisciplinary evaluation and individualized follow-up strategies.

Keywords: Computed tomography; Connective tissue disease; Fibrosis variants; Interstitial lung disease; Systemic lupus erythematosus.

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Conflict of interest statement

Compliance with ethical standards. Guarantor: The scientific guarantor of this publication is Marie-Pierre Debray. Conflict of interest: The authors of this manuscript declare relationships with the following companies: Nathalie Costedoat-Chalumeau declares grants from Roche and UCB and participation to data safety monitoring board or advisory board for BMS; Yurdagul Uzunhan declares grants from Oxyvie, consulting fees from Boehringer-Ingelheim and Pfizer, payments for honoraria or lectures from Sanofi, Boehringer-Ingelheim, CSL Vifor, participation on data safety monitoring board or advisory board for Boehringer-Ingelheim, support for attending meetings from Oxyvie and Boehringer-Ingelheim; Jacques Cadranel declares consulting fees from AstraZeneca, Boehringer-Ingelheim, Takeda, Johnson and Johnson, MSD, Pfizer, and Roche; David Montani declares grants from Gossamer and Merck, consulting fees from Ferrer and Merck MSD, payments for honoraria and lectures from Bayer, Boehringer-Ingelheim, Chiesi, GSK, Jazz Pharmacenticals, and Ferrer and Merck MSD; Martine Reunaud-Gaubert declares consulting fees from Ferrer and Merck MSD, payments or honoraria or lectures from Sanofi, Boehringer-Ingelheim, and Ferrer; Guillaume Beltramo declares payments for honoraria or lectures from Boehringer-Ingelheim, support for attending meetings from Boehringer-Ingelheim, Sanofi and Asdia medical; Bruno Crestani declares grants from Boehringer-Ingelheim, consulting fees from BMS, Boehringer-Ingelheim, Chiesi, CSL Behring, GSK, Sanofi, payments for honoraria or lectures and support for attending meetings from AstraZeneca, BMS, Boehringer-Ingelheim, Roche, Sanofi, support for attending meetings from GSK and Novartis, participation on data safety monitoring board or advisory board for BMS, Boehringer-Ingelheim, Horizon, Sanofi, and is president of the board of trustee of the Fondation du Souffle; Vincent Cottin declares consulting fees from Abbvie, AstraZeneca, Avalyn, Boehringer-Ingelheim, BMS/Celgene, CSL, Ferrer/ United Therapeutics, Gossamer, GSK, Liquidia, Pliant, PureTech, Roche, Roivant, Sanofi, Shionogi, payments for honoraria or lectures from Boehringer-Ingelheim, Ferrer/United Therapeutics, Roche, Sanofi, support for attending meetings from Boehringer-Ingelheim, Sanofi, participation on data safety monitoring board for GSK and Molecure and participation to adjudication committee for Fibrogen; Raphaël Borie declares consulting fees from Boehringer-Ingelheim, Ferrer, Sanofi, payments for honoraria or lectures and support for attending meetings from Boehringer-Ingelheim; Marie-Pierre Debray declares payments for honoraria or lectures from Boehringer-Ingelheim, Sanofi, Bracco and support for attending meetings from Boehringer-Ingelheim Aurore Sajust de Bergues de Escalup, Arthur Mageau, Lou Deneuville, Karim Sacre, Antoine Khalil, Eric Hachulla, Erwan Le Tallec, Sylvain Marchand-Adam, Grégoire Prévot declare no relationships with any companies, whose products or services may be related to the subject matter of the article. Statistics and biometry: One of the authors, Arthur Mageau, has significant statistical expertise. Informed consent: Written informed consent was not required for this observational non-interventional study. It was waived by the Institutional Review Board. Ethical approval: Institutional Review Board approval was obtained. Study subjects or cohorts overlap: This study is an ancillary study of the previously published study by Deneuville et al (Deneuville L, Mageau A, Debray MP, Respirology 2024;29:713–721). Most patients included in this manuscript were reported in the study by Deneuville et al, which described characteristics of patients with interstitial lung disease associated with lupus (89 patients) and analyzed factors associated with prognosis, but provided very limited information on imaging findings. This study focuses on imaging findings, both at presentation and on last follow-up. It gives a detailed description of the CT findings at presentation, including several variant signs of lung fibrosis. It analyzes CT signs of progressive disease over a long time period and compares CT to functional progression. Methodology: Retrospective Multicentric Observational

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