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Combined pulmonary fibrosis and emphysema (CPFE) is a clinical syndrome characterized by upper lobe emphysema and lower lobe fibrosis manifested by exercise hypoxemia, normal lung volumes, and severe reduction of diffusion capacity of carbon monoxide. It has varying prevalence worldwide with a male predominance, and with smoking history of more than 40 pack-years being a common risk factor. The unique imaging features of CPFE emphasize its distinct entity, aiding in the timely detection of pulmonary hypertension and lung cancer, both of which are common complications. High-resolution computed tomography (HRCT) is an important diagnostic and prognostic tool, while lung cancer is an independent factor that alters the prognosis in CPFE patients. Treatment options for CPFE are limited, but smoking cessation, usual treatments of pulmonary fibrosis and emphysema, and avoidance of environmental exposures are encouraged.
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http://dx.doi.org/10.3390/biomedicines11061636 | DOI Listing |
Turk J Pediatr
September 2025
Department of Cardiorespiratory Physiotherapy and Rehabilitation, Faculty of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Türkiye.
Background: Vascular changes are observed in children with cystic fibrosis (cwCF), and gender-specific differences may impact arterial stiffness. We aimed to compare arterial stiffness and clinical parameters based on gender in cwCF and to determine the factors affecting arterial stiffness in cwCF.
Methods: Fifty-eight cwCF were included.
Pediatr Pulmonol
September 2025
Department of Medicine, Division of Diabetes, Endocrinology and Metabolism, University of Minnesota, Minneapolis, Minnesota, USA.
Background: The approval of cystic fibrosis transmembrane conductance regulator modulators elexacaftor/tezacaftor/ivacaftor (ETI), has significantly improved pulmonary function for people with cystic fibrosis (pwCF). However, the effects on CF-related bone disease and body composition remain unclear.
Methods: This retrospective real-world study examined adults with CF who received ETI treatment.
Clin Respir J
September 2025
Department of Thoracic Surgery, Taizhou Hospital, Taizhou, Zhejiang, China.
Background: Persistent inflammation is a crucial characteristic of idiopathic pulmonary fibrosis (IPF). Gut microbiota (GM) contribute to the occurrence and development of several pulmonary diseases through the "gut-lung axis." The genetic role of GM in IPF and the mediating effect of circulating inflammatory proteins.
View Article and Find Full Text PDFIntroduction: Interstitial pneumonia with autoimmune features (IPAF) describes a rare condition characterized by interstitial lung disease (ILD) with autoimmune manifestations in the absence of defined autoimmune rheumatic diseases (AIRD). Although the classification was established in 2015, prospective data on disease progression remain limited.
Objectives: To identify predictors of ILD progression in IPAF patients using three criteria: 1) progressive pulmonary fibrosis (PPF), 2) INBUILD criteria, 3) absolute FVC decline ≥10%.
Pulm Circ
July 2025
Division of Pulmonary, Critical Care, and Sleep Medicine Tufts Medical Center Boston Massachusetts USA.
Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction, proliferation, fibrosis, and microthrombosis of the pulmonary vasculature, which causes elevated pulmonary arterial pressure and vascular resistance leading to right ventricular failure and death. Previous treatments targeted three known pathways involved in the development of PAH: endothelin, nitric oxide, and prostacyclin. Dysfunctional signaling of the transforming growth factor-beta (TGF-β) family, via bone morphogenetic protein (BMP) receptor 2 and activin signaling, has also been implicated in PAH leading to the development of a new class of therapies.
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