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.

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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
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article

Morphological CT phenotypes in pulmonary sarcoidosis at diagnosis: splitting and lumping based on predictors of clinical progression

Daiana Stolz, Björn C. Frye, Martin Feineis, Charlott Terschluse et al.
ERJ Open Research
Sarcoidosis and Beryllium Toxicity Research
article

Morphological CT phenotypes in pulmonary sarcoidosis at diagnosis: splitting and lumping based on predictors of clinical progression

Daiana Stolz, Björn C. Frye, Martin Feineis, Charlott Terschluse, Fabian Bamberg, Louis Jouanjan, Paula Kuss, Christopher L. Schlett, Sebastian Wirtz, Prerana Agarwal, Daniel Bross Soriano, Emil Wachenfeld
article en

Abstract

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.

ERJ Open Research
Openalex Percentile: Top 50%
Sarcoidosis and Beryllium Toxicity Research
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