Systemic sclerosis & Raynaud's
Predicting the risk of subsequent progression in patients with systemic sclerosis-associated interstitial lung disease with progression: a multicentre observational cohort study
Main finding · Further progression (FVC decline ≥5%) from visit 2 to visit 3Result — go to the resultOdds ratio 0.28 95% CI 0.12–0.63Authors' conclusion
Systemic sclerosis-associated ILD progression does not predict further ILD progression at the next annual follow-up visit, even in an enriched population, but progression was associated with mortality.
Study context
- Population
- Patients with SSc-associated interstitial lung disease (ILD) aged 18 years or older at diagnosis fulfilling 2013 ACR-EULAR SSc criteria; Oslo University Hospital, Norway, and University Hospital Zurich, Switzerland (diagnosed 2001–2019)main cohort
- Participants
- 231 patients
- Exposure
- ILD progression (forced vital capacity (FVC) decline ≥5%) from visit 1 to visit 2 vs no progression, for further progression from visit 2 to visit 3
- Progressionn = 71
- Outcome
- Further ILD progression (primary definition: FVC decline of 5% or more) from visit 2 to visit 3
- Follow-up
- Visit 3
Methodological detailsprospectively collected data, validation in enriched cohort, multivariable logistic regression
- Prospectively collected data
- Validation in enriched cohort
- Multivariable logistic regression
Main finding
As reported in the abstract
Odds ratio
0.28
95% CI 0.12–0.63
P = 0.002
Observational data: associations, not effects.
Further findings
Further progression (visit 2 to 3) after progression (visit 1 to 2), secondary definition FVC decline ≥10%
| OR (95% CI) | P value | ||
|---|---|---|---|
| FVC decline of ≥10% (visit 1 to 2)Main cohort | 0.57 (0.16–1.99) | P = 0.38 | |
| 0.10.31310Odds ratio (log scale) |
Further progression (visit 2 to 3) after progression (visit 1 to 2), secondary definition progressive pulmonary fibrosis (PPF)
| OR (95% CI) | P value | ||
|---|---|---|---|
| PPF (visit 1 to 2)Main cohort | 0.93 (0.39–2.22) | P = 0.88 | |
| 0.20.5125Odds ratio (log scale) |
Further progression (visit 2 to 3) after progression (visit 1 to 2), secondary definition progressive fibrosing ILD (PF-ILD)
| OR (95% CI) | P value | ||
|---|---|---|---|
| PF-ILD (visit 1 to 2)Main cohort | 0.69 (0.35–1.36) | P = 0.28 | |
| 0.20.5125Odds ratio (log scale) |
Further progression after progression by FVC decline ≥5%, enriched SSc-ILD cohort (validation)
| OR (95% CI) | P value | ||
|---|---|---|---|
| FVC decline of 5% or moreEnriched cohort | 0.22 (0.06–0.87) | P = 0.031 | |
| 0.050.21520Odds ratio (log scale) |
Mortality
| HR (95% CI) | P value | ||
|---|---|---|---|
| FVC decline of 5% or moreAdjusted for other risk factors | 1.66 (1.05–2.62) | P = 0.030 | |
| 0.20.5125Hazard ratio (log scale) |
- Further progression (visit 2 to 3) after progression (visit 1 to 2), secondary definition FVC decline ≥10%: multivariable logistic regression, adjusted for risk factors and immunosuppressive treatment
- Further progression (visit 2 to 3) after progression (visit 1 to 2), secondary definition PPF: multivariable logistic regression, adjusted for risk factors and immunosuppressive treatment
- Further progression (visit 2 to 3) after progression (visit 1 to 2), secondary definition PF-ILD: multivariable logistic regression, adjusted for risk factors and immunosuppressive treatment
Further progression after progression by FVC decline ≥5%, enriched SSc-ILD cohort (validation)
- Progression by FVC decline ≥5% in enriched cohort: 41 (34%) of 121 patients
ILD progression between visit 1 and visit 2 by secondary definitions, main cohort
- FVC decline of 10% or more
- 38 (16%)
- PPF
- 39 (17%)
- PF-ILD
- 89 (39%)
Original publication
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