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Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort

Scheidegger, Moritz; Boubaya, Marouane; Garaiman, Alexandru; Barua, Imon; Becker, Mike; Bjørkekjær, Hilde Jenssen; Bruni, Cosimo; Dobrota, Rucsandra; Fretheim, Håvard; Jordan, Suzana; Midtvedt, Oyvind; Mihai, Carina; Hoffmann-Vold, Anna-Maria; Distler, Oliver; Elhai, Muriel (2024). Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort. RMD Open, 10(1):e003658.

Abstract

BACKGROUND

Interstitial lung disease (ILD) is the leading cause of death in systemic sclerosis (SSc). According to expert statements, not all SSc-ILD patients require pharmacological therapy.

OBJECTIVES

To describe disease characteristics and disease course in untreated SSc-ILD patients in two well characterised SSc-ILD cohorts.

METHODS

Patients were classified as treated if they had received a potential ILD-modifying drug. ILD progression in untreated patients was defined as (1) decline in forced vital capacity (FVC) from baseline of ≥10% or (2) decline in FVC of 5%-9% associated with a decline in diffusing capacity for carbon monoxide (DLCO)≥15% over 12±3 months or (3) start of any ILD-modifying treatment or (4) increase in the ILD extent during follow-up. Multivariable logistic regression was performed to identify factors associated with non-prescription of ILD-modifying treatment at baseline. Prognostic factors for progression in untreated patients were tested by multivariate Cox regression.

RESULTS

Of 386 SSc-ILD included patients, 287 (74%) were untreated at baseline. Anticentromere antibodies (OR: 6.75 (2.16-21.14), p=0.001), limited extent of ILD (OR: 2.39 (1.19-4.82), p=0.015), longer disease duration (OR: 1.04 (1.00-1.08), p=0.038) and a higher DLCO (OR: 1.02 (1.01-1.04), p=0.005) were independently associated with no ILD-modifying treatment at baseline. Among 234 untreated patients, the 3 year cumulative incidence of progression was 39.9% (32.9-46.2). Diffuse cutaneous SSc and extensive lung fibrosis independently predicted ILD progression in untreated patients.

CONCLUSION

As about 40% of untreated patients show ILD progression after 3 years and effective and safe therapies for SSc-ILD are available, our results support a change in clinical practice in selecting patients for treatment.

Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Rheumatology Clinic and Institute of Physical Medicine
Dewey Decimal Classification:610 Medicine & health
Scopus Subject Areas:Health Sciences > Rheumatology
Health Sciences > Immunology and Allergy
Life Sciences > Immunology
Language:English
Date:9 January 2024
Deposited On:07 Feb 2024 07:04
Last Modified:31 Oct 2024 02:36
Publisher:BMJ Publishing Group
ISSN:2056-5933
OA Status:Gold
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1136/rmdopen-2023-003658
PubMed ID:38199606
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  • Language: English
  • Licence: Creative Commons: Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)

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