Morphological CT phenotypes in pulmonary sarcoidosis at diagnosis: splitting and lumping based on predictors of clinical progression
Background Sarcoidosis may represent a broad umbrella term for various diseases that induces non-necrotizing granulomas in affected organs. A recent multinational consensus proposed distinct computed tomography phenotypes for pulmonary sarcoidosis; however, clinical implications remain unclear. Question What is the prevalence of individual phenotypes in a tertiary referral cohort, and are these phenotypes associated with airway involvement and disease progression? Materials and Methods This retrospective study included 232 biopsy-proven pulmonary sarcoidosis patients treated between 2010 and 2020. Baseline CT scans were grouped into (1) non-fibrotic/fibrotic/unclear for fibrosis and (2) normal/nodular/non-nodular. Airway involvement were analysed. Disease progression was assessed over 36 months using predefined clinical, functional and therapeutic criteria. Results Most patients had non-fibrotic phenotypes (91.0%, n=211), while fibrotic (5.6%, n=13) and unclear (3.4%, n=8) patterns were less frequent. Inter-reader agreement was moderate for individual phenotypes (κ=0.55) and good for fibrotic versus non-fibrotic classification (κ=0.79). At 36 months, 69% of patients had progressed; all fibrotic cases progressed, compared with 67% of non-fibrotic. Nodular phenotypes progressed similar to normal CT (64% versus 68%) and lower than non-nodular disease (88%). Fibrotic disease was associated with an increased risk of disease progression after adjustment for age, sex, BMI, and baseline lung function (HR 2.31; 95% CI 1.21–4.38; p=0.038). Answer CT-based seems feasible but my require refinement. Fibrotic patterns predict progression, while “nodular” carries a risk similar to normal CT. Further radiological splitting may not reflect distinct disease behavior.
Authors
- Daiana Stolz (ORCID: https://orcid.org/0000-0003-0099-882X)
- Björn C. Frye (ORCID: https://orcid.org/0000-0003-3520-1868)
- Martin Feineis
- Charlott Terschluse
- Fabian Bamberg (ORCID: https://orcid.org/0000-0002-7460-3942)
- Louis Jouanjan (ORCID: https://orcid.org/0000-0001-7476-5557)
- Paula Kuss (ORCID: https://orcid.org/0009-0001-2005-4431)
- Christopher L. Schlett (ORCID: https://orcid.org/0000-0002-1576-1481)
- Sebastian Wirtz (ORCID: https://orcid.org/0000-0003-4037-0615)
- Prerana Agarwal (ORCID: https://orcid.org/0000-0002-6784-6727)
- Daniel Bross Soriano
- Emil Wachenfeld
Publication Details
- Journal
- ERJ Open Research
- Published
- 2025-12-18
- DOI
- https://doi.org/10.1183/23120541.00632-2025
- Primary Topic
- Sarcoidosis and Beryllium Toxicity Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00