ArticleRespiratory research2022
Characterization of the PF-ILD phenotype in patients with advanced pulmonary sarcoidosis.
Article in Respiratory research, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 27 citations in OpenAlex.
- Advanced Pulmonary Sarcoidosis: Treatment and Monitoring.Diagnostics (Basel, Switzerland) · 2025Review
- Fibrotic Pulmonary Sarcoidosis-From Pathogenesis to Management.Journal of clinical medicine · 2025Review
- Family history of pulmonary fibrosis impacts prognosis in patients with sarcoidosis.ERJ open research · 2025Article
- Sarcoidosis versus Granulomatous and Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency: A Comparative Review.Biomedicines · 2024Review
- Sarcoidosis-Associated Pulmonary Hypertension.Journal of clinical medicine · 2024Review
- High-Risk Sarcoidosis: A Focus on Pulmonary, Cardiac, Hepatic and Renal Advanced Diseases, as Well as on Calcium Metabolism Abnormalities.Diagnostics (Basel, Switzerland) · 2024Review
- Establishing a Diagnosis of Pulmonary Sarcoidosis.Journal of clinical medicine · 2023Article
- Cluster features in fibrosing interstitial lung disease and associations with prognosis.BMC pulmonary medicine · 2023Article
- Phenotypes of Sarcoidosis-Associated Pulmonary Hypertension-A Challenging Mystery.Diagnostics (Basel, Switzerland) · 2023Review
- Sarcoidosis-associated pulmonary fibrosis: joining the dots.European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Molecular Mechanism in the Development of Pulmonary Fibrosis in Patients with Sarcoidosis.International journal of molecular sciences · 2023Review
- Lymphocyte Subsets and Pulmonary Nodules to Predict the Progression of Sarcoidosis.Biomedicines · 2023Article
- Genetics in Idiopathic Pulmonary Fibrosis: A Clinical Perspective.Diagnostics (Basel, Switzerland) · 2022Review
- A clinical predictive model for the long-term survival of progressive fibrosis interstitial lung disease patients.Therapeutic advances in respiratory diseaseArticle
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
Abstract
backgroundAdvanced pulmonary sarcoidosis causes significant morbidity and can lead to death. Large trials demonstrated efficacy of antifibrotics in patients with progressive fibrosing interstitial lung diseases (PF-ILD), including a few with sarcoidosis. To date, little is known about this progressive fibrosing phenotype in sarcoidosis. Diffusion capacity of carbon monoxide (DLCO) may be a useful functional marker to screen for advanced pulmonary sarcoidosis. In this study, we describe a cohort with advanced pulmonary sarcoidosis and we gain insights in the progressive fibrosing phenotype in sarcoidosis.
methodsPatients with sarcoidosis and a DLCO < 50% predicted were included in this retrospective cohort study. First measurement of DLCO < 50% predicted was the baseline. Lung function data, HRCT, pulmonary hypertension (PH) and mortality were collected. Patients with > 10% fibrosis on HRCT meeting the criteria for ILD-progression within 24 months were labelled as PF-ILD. With Cox-regression analysis predictors of mortality were established.
results106 patients with a DLCO < 50% predicted were included. Evolution of forced vital capacity (FVC) varied widely between patients from - 34% to + 45% after 2 years follow-up, whereas change in DLCO varied between - 11% and + 26%. Fourteen patients (15%) met the PF-ILD criteria, of whom 6 (43%) died within 10 years versus 10 (13%) in the non PF-ILD group (p = 0.006). PH was present 12 (11%), 56 (53%) demonstrated > 10% fibrosis on HRCT. Independent predictors of mortality and lung transplantation in the whole cohort are PH, PF-ILD and UIP-like pattern.
conclusionIn conclusion, within this group with advanced pulmonary sarcoidosis disease course varied widely from great functional improvement to death. PF-ILD patients had higher mortality rate than the mortality in the overall pulmonary sarcoidosis group. Future research should focus on the addition of antifibrotics in these patients. Trial registration retrospectively registered.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.