ArticleRespiratory research2024
Clinical, radiological and histopathological features of patients with familial pulmonary fibrosis.
Article in Respiratory research, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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6 citing papers in PubMed.
- Integrated machine learning risk model for predicting radiation pneumonitis in lung cancer patients with interstitial lung disease.Annals of medicine · 2026Article
- Prevalence and outcomes of pleuroparenchymal fibroelastosis in patients with non-tuberculous mycobacterial pulmonary disease: a French retrospective cohort study.Journal of thoracic disease · 2026Article
- B and T cells in lung tissue are associated with smoking and forced vital capacity in idiopathic pulmonary fibrosis and familial pulmonary fibrosis.Respiratory research · 2026Article
- Gut microbiota dysbiosis and metabolic abnormalities promote oxidative stress and fibrosis in idiopathic pulmonary fibrosis.Scientific reports · 2026Article
- Bronchoalveolar lavage cell differential count is associated with survival and radiological UIP pattern in familial pulmonary fibrosis.BMC pulmonary medicine · 2026Article
- Time-of-day radiotherapy alters radiation pneumonitis risk but not survival in lung cancer patients with interstitial lung disease.Therapeutic advances in medical oncology · 2026Article
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Abstract
backgroundIn familial pulmonary fibrosis (FPF) at least two biological relatives are affected. Patients with FPF have diverse clinical features. RESEARCH QUESTION: We aimed to characterize demographic and clinical features, re-evaluate high-resolution computed tomography (HRCT) scans and histopathology of surgical lung biopsies, assess survival and investigate the suitability of risk prediction models for FPF patients. STUDY
designA retrospective cohort study.
methodsFPF data (n = 68) were collected from the medical records of Oulu University Hospital (OUH) and Oulaskangas District Hospital between 1 Jan 2000 and 11 Jan 2023. The inclusion criterion was pulmonary fibrosis (PF) (ICD 10-code J84.X) and at least one self-reported relative with PF. Clinical information was gathered from hospital medical records. HRCT scans and histology were re-evaluated.
resultsThirty-seven (54.4%) of the patients were men, and 31 (45.6%) were women. The mean ages of the women and men were 68.6 and 61.7 years, respectively (p = 0.003). Thirty-seven (54.4%) patients were nonsmokers. The most common radiological patterns were usual interstitial pneumonia (UIP) (51/75.0%), unclassifiable (8/11.8%) and nonspecific interstitial pneumonia (NSIP) (3/4.4%). Pleuroparenchymal fibroelastosis (PPFE) was observed as a single or combined pattern in 13.2% of the patients. According to the 2022 guidelines for idiopathic pulmonary fibrosis (IPF), the patients were categorized as UIP (31/45.6%), probable UIP (20/29.4%), indeterminate for UIP (7/10.3%) or alternative diagnosis (10/14.7%). The histopathological patterns were UIP (7/41.2%), probable UIP (1/5.9%), indeterminate for UIP (8/47.2%) and alternative diagnosis (1/5.9%). Rare genetic variants were found in 9 patients; these included telomerase reverse transcriptase (TERT, n = 6), telomerase RNA component (TERC, n = 2) and regulator of telomere elongation helicase 1 (RTEL1, n = 1). Half of the patients died (n = 29) or underwent lung transplantation (n = 5), with a median survival of 39.9 months. The risk prediction models composite physiology index (CPI), hazard ratio (HR) 1.07 (95.0% CI 1.04-1.10), and gender-age-physiology index (GAP) stage I predicted survival statistically significantly (p<0.001) compared to combined stages II and III.
conclusionsThis study confirmed the results of earlier studies showing that FPF patients' radiological and histopathological patterns are diverse. Moreover, radiological and histological features revealed unusual patterns and their combinations.
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