Phenotyping by persistent inflammation in systemic sclerosisassociated interstitial lung disease: a EUSTAR database analysis.

Guler, Sabina; Sarbu, Adela-Cristina; Stalder, Odile; Allanore, Yannick; Bernardino, Vera; Distler, Joerg; Gabrielli, Armando; Hoffmann-Vold, Anna-Maria; Matucci-Cerinic, Marco; Müller-Ladner, Ulf; Ortiz-Santamaria, Vera; Rednic, Simona; Riccieri, Valeria; Smith, Vanessa; Ullman, Susanne; Walker, Ulrich A; Geiser, Thomas K; Distler, Oliver; Maurer, Britta and Kollert, Florian (2023). Phenotyping by persistent inflammation in systemic sclerosisassociated interstitial lung disease: a EUSTAR database analysis. Thorax, 78(12), pp. 1188-1196. BMJ Publishing Group 10.1136/thorax-2023-220541

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BACKGROUND

Systemic sclerosis (SSc) is a heterogeneous disease with frequently associated interstitial lung disease (SSc-ILD). We aimed to determine the prognostic potential of phenotyping patients with SSc and SSc-ILD by inflammation and to describe disease trajectories stratified by inflammation and immunosuppressive treatment.

METHODS

Patients from the European Scleroderma Trials and Research (EUSTAR) group cohort were allocated to persistent inflammatory, intermediate and non-inflammatory phenotypes if C-reactive protein (CRP) levels were ≥5 mg/L at ≥80%, at 20-80% and at <20% of visits, respectively. Cox regression models were used to analyse mortality risk and mixed effect models to describe trajectories of FVC and diffusing capacity for carbon monoxide (DLCO) %-predicted stratified by inflammation and immunosuppressive treatment.

RESULTS

2971 patients with SSc and 1171 patients with SSc-ILD had at least three CRP measurements available. Patients with SSc-ILD with a persistent inflammatory phenotype had a 6.7 times higher risk of mortality within 5 years compared with those with a persistent non-inflammatory phenotype (95% CI 3 to 15). In the inflammatory phenotype, FVC %-predicted was declining without (-1.11 (95% CI -2.14 to -0.08)/year), but stable with immunosuppressive treatment (-0.00 (95% CI -0.92 to 0.92)/year). In the non-inflammatory phenotype, patients with and without immunosuppressive treatment had a significant decline in FVC %-predicted, which was more pronounced in those with immunosuppressive treatment (-1.26 (95% CI -1.87 to -0.64) and -0.84 (95% CI -1.35 to -0.33)/year, respectively).

CONCLUSIONS

Phenotyping by persistent inflammation provides valuable prognostic information, independent of demographics, disease duration, cutaneous subtype, treatment and SSc-ILD severity. The findings from this study support early immunosuppressive treatment in patients with SSc-ILD with persistent inflammation.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology and Immunology
04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Pneumology
04 Faculty of Medicine > Pre-clinic Human Medicine > Department of Clinical Research (DCR)
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > DBMR Forschung Mu35 > Forschungsgruppe Rheumatologie
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > DBMR Forschung Mu35 > Forschungsgruppe Rheumatologie

04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Forschungsbereich Mu50 > Forschungsgruppe Pneumologie (Erwachsene)

UniBE Contributor:

Guler, Sabina Anna, Sarbu, Adela-Cristina, Stalder, Odile, Geiser, Thomas (A), Maurer, Britta, Kollert, Florian Kim

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0040-6376

Publisher:

BMJ Publishing Group

Funders:

[206] Stiftung Lindenhof Bern = Lindenhof Foundation

Language:

English

Submitter:

Pubmed Import

Date Deposited:

09 Oct 2023 13:08

Last Modified:

20 Feb 2024 14:15

Publisher DOI:

10.1136/thorax-2023-220541

PubMed ID:

37798114

Additional Information:

Sabina Guler and Adela-Cristina Sarbu contributed equally to this paper.

Uncontrolled Keywords:

Interstitial Fibrosis Rheumatoid lung disease Systemic disease and lungs

BORIS DOI:

10.48350/186950

URI:

https://boris.unibe.ch/id/eprint/186950

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