Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort
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 c...
| Main Authors: | , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMJ Publishing Group
2024-02-01
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| Series: | RMD Open |
| Online Access: | https://rmdopen.bmj.com/content/10/1/e003658.full |
| _version_ | 1797222232371494912 |
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| author | Oliver Distler Cosimo Bruni Anna-Maria Hoffmann-Vold Suzana Jordan Carina Mihai Muriel Elhai Marouane Boubaya Rucsandra Dobrota Øyvind Midtvedt Mike Becker Alexandru Garaiman Håvard Fretheim Imon Barua Moritz Scheidegger Hilde Jenssen Bjørkekjær |
| author_facet | Oliver Distler Cosimo Bruni Anna-Maria Hoffmann-Vold Suzana Jordan Carina Mihai Muriel Elhai Marouane Boubaya Rucsandra Dobrota Øyvind Midtvedt Mike Becker Alexandru Garaiman Håvard Fretheim Imon Barua Moritz Scheidegger Hilde Jenssen Bjørkekjær |
| author_sort | Oliver Distler |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-08T15:30:19Z |
| format | Article |
| id | doaj.art-475ee06828c44af2aa3d17552fc41486 |
| institution | Directory Open Access Journal |
| issn | 2056-5933 |
| language | English |
| last_indexed | 2024-04-24T13:18:03Z |
| publishDate | 2024-02-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | RMD Open |
| spelling | doaj.art-475ee06828c44af2aa3d17552fc414862024-04-04T17:10:09ZengBMJ Publishing GroupRMD Open2056-59332024-02-0110110.1136/rmdopen-2023-003658Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohortOliver Distler0Cosimo Bruni1Anna-Maria Hoffmann-Vold2Suzana Jordan3Carina Mihai4Muriel Elhai5Marouane Boubaya6Rucsandra Dobrota7Øyvind Midtvedt8Mike Becker9Alexandru Garaiman10Håvard Fretheim11Imon Barua12Moritz Scheidegger13Hilde Jenssen Bjørkekjær14Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Clinical Research, CHU Avicenne, APHP, Bobigny, FranceDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Rheumatology, Oslo University Hospital, Oslo, NorwayDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Rheumatology, Oslo University Hospital, Oslo, NorwayDepartment of Rheumatology, Oslo University Hospital, Oslo, NorwayDepartment of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, SwitzerlandDepartment of Rheumatology, Hospital of Southern Norway, Kristiansand, NorwayBackground 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.https://rmdopen.bmj.com/content/10/1/e003658.full |
| spellingShingle | Oliver Distler Cosimo Bruni Anna-Maria Hoffmann-Vold Suzana Jordan Carina Mihai Muriel Elhai Marouane Boubaya Rucsandra Dobrota Øyvind Midtvedt Mike Becker Alexandru Garaiman Håvard Fretheim Imon Barua Moritz Scheidegger Hilde Jenssen Bjørkekjær Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort RMD Open |
| title | Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort |
| title_full | Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort |
| title_fullStr | Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort |
| title_full_unstemmed | Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort |
| title_short | Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort |
| title_sort | characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real life two centre cohort |
| url | https://rmdopen.bmj.com/content/10/1/e003658.full |
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