A proportion of patients with certain types of interstitial lung disease (ILD), including chronic hypersensitivity pneumonitis and ILDs associated with autoimmune diseases, develop a progressive fibrosing phenotype that shows similarities in clinical course to idiopathic pulmonary fibrosis. Irrespective of the clinical diagnosis, these progressive fibrosing ILDs show commonalities in the underlying pathogenetic mechanisms that drive a self-sustaining process of pulmonary fibrosis. The natural history of progressive fibrosing ILDs is characterized by decline in lung function, worsening of symptoms and health-related quality of life, and early mortality. Greater impairment in forced vital capacity or diffusion capacity of the lungs for carbon monoxide, and a greater extent of fibrotic changes on a computed tomography scan, are predictors of mortality in patients with fibrosing ILDs. However, the course of these diseases is heterogenous and cannot accurately be predicted for an individual patient. Data from ongoing clinical trials and patient registries will provide a better understanding of the clinical course and impact of progressive fibrosing ILDs.

• Klíčová slova
• Connective tissue diseases, Mortality, Pulmonary fibrosis, Rheumatic diseases, Systemic sclerosis, Vital capacity,
• MeSH
• hypersenzitivní pneumonitida diagnostické zobrazování   epidemiologie   MeSH
• idiopatická plicní fibróza diagnostické zobrazování   epidemiologie   MeSH
• intersticiální plicní nemoci diagnostické zobrazování   epidemiologie   MeSH
• lidé MeSH
• mortalita trendy   MeSH
• progrese nemoci * MeSH
• rizikové faktory MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

BACKGROUND: Extrinsic allergic alveolitis (EAA) patients form heterogenous group with different clinical manifestation and different prognosis. We aimed to determine how to phenotype distinct EAA subgroups. Predictive role of the bronchoalveolar lavage fluid (BALF) IL-4Rα concentration at the time of diagnosis with regard to the clinical behavior in EAA patients was studied. METHODS: Concentrations of MMP-7, IL-4Rα, TNF-α, and PAR-2 were measured in the BALF od 71 EAA patients at the time of diagnosis. Lung functions and outcome data were assessed at 12 months after the diagnosis. Correlations between the BALF protein concentration, cell profile, lung functions and patient outcome were determined. RESULTS: We found positive correlations between BALF IL-4Rα concentration and BALF eosinophils (p = 0,006), negative correlation between IL-4Rα BALF concentration and diffusing capacity (DLco) (p = 0,003), negative correlation between IL-4Rα BALF concentration and forced vital capacity (FVC) (p = 0,004) and negative correlation between IL-4Rα concentration and BALF lymphocytes (p = 0,04). The BALF concentration of IL-4Rα was significantly higher in acute exacerbation patients (p = 0,0032) and in patients progressing despite corticosteroid treatment (p = 0,04). We observed a positive correlation between MMP-7 BALF concentration and the BALF lymphocytes (p = 0.05), negative correlation between the PAR-2 BALF concentration and DLco (p = 0.04) and a negative correlation between the BALF TNF-α concentration and DLco (p = 0.03). CONCLUSIONS: Specific subgroup of EAA patients with more severe functional impact, distinct BALF cell profile and higher IL-4Rα BALF concentration can be differentiated. Correlations between the BALF concentrations of PAR-2, MMP-7 and TNF-α with clinical parameters may reflect the role of inflammation in the pathogenesis of EAA.

• Klíčová slova
• Bronchoalveolar lavage, Extrinsic allergic alveolitis, Interleukin 4 receptor, Matrix metalloproteinase 7, Prognosis, Proteinase associated receptor 2,
• Publikační typ
• časopisecké články MeSH