SynthesisArthritis research & therapy2021
Effect of combined pulmonary fibrosis and emphysema on patients with connective tissue diseases and systemic sclerosis: a systematic review and meta-analysis.
Synthesis in Arthritis research & therapy, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 15 citations in OpenAlex.
- Prognostic factors in combined pulmonary fibrosis and emphysema: a systematic review and meta-analysis.Journal of thoracic disease · 2026Article
- Pulmonary cystic destruction in systemic sclerosis and antisynthetase syndrome associated non-specific interstitial pneumonitis: a case series of a novel radiological phenotype.Rheumatology advances in practice · 2026Article
- Does combined pulmonary fibrosis and emphysema syndrome affect response to antifibrotic therapy and survival? A single-center retrospective cohort study.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2025Article
- Emphysema elevates the DETECT scores: impact on pulmonary hypertension screening and diagnosis in systemic sclerosis.Rheumatology (Oxford, England) · 2025Article
- Rheumatoid arthritis presenting by combined pulmonary fibrosis and emphysema with clubbing and pulmonary hypertension: A rare case report.Clinical case reports · 2024Article
- Emphysema and interstitial lung disease in systemic sclerosis-related pulmonary hypertension.Respiratory medicine · 2023Article
- The Role of Macrophages in Connective Tissue Disease-Associated Interstitial Lung Disease: Focusing on Molecular Mechanisms and Potential Treatment Strategies.International journal of molecular sciences · 2023Review
- Traction Bronchiectasis/Bronchiolectasis on CT Scans in Relationship to Clinical Outcomes and Mortality: The COPDGene Study.Radiology · 2022Article
- Use of machine learning models to predict prognosis of combined pulmonary fibrosis and emphysema in a Chinese population.BMC pulmonary medicine · 2022Article
- Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease.Tuberculosis and respiratory diseases · 2022Review
Corrections and comments
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Authors and funding
7 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThis study aimed to analyze the literature systematically to determine the clinical characteristics and prognosis of patients with connective tissue disease (CTD) with combined pulmonary fibrosis and emphysema (CPFE) compared to those of patients with CTD-interstitial lung disease (CTD-ILD) without emphysema.
methodsWe searched MEDLINE, EMBASE, Cochrane Library, and KoreaMed for relevant articles published before July 2019. Studies meeting all the following criteria were included: (1) original research studies evaluating the effect of CPFE on CTD, (2) studies that compared patients with CTD-CPFE to those with CTD-ILD without emphysema, and (3) studies providing data on physical capacity, pulmonary function, or death in patients with CTD. Clinical characteristics of patients with CTD-CPFE were compared with those of patients with CTD-ILD without emphysema, and the influence of CPFE on physical capacity, pulmonary function, and death was analyzed.
resultsSix studies between 2013 and 2019 were included. Two hundred ninety-nine (29.5%) and 715 (70.5%) patients had CTD-CPFE and CTD-ILD without emphysema, respectively. Regarding the type of CTD, 711 (68.3%) patients had systemic sclerosis, 263 (25.3%) rheumatoid arthritis, and 67 (6.4%) other CTDs. Patients with CTD-CPFE had a higher frequency of pulmonary hypertension and pulmonary fibrosis > 20% of the total lung volume, higher ratio of the forced vital capacity to the diffusion capacity of the lung for carbon monoxide (DLCO), lower arterial oxygen pressure at rest, and lower DLCO compared to those in patients with CTD-ILD without emphysema. In addition, more deaths occurred among those with CTD-CPFE (odds ratio, 2.95; 95% confidence interval, 1.75-4.96).
conclusionCTD-CPFE is associated with worse physical and pulmonary function and more deaths compared to those in CTD-ILD without emphysema. These findings indicate the need for increased awareness and close monitoring of patients with CTD-CPFE.
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