Predictors of response to pirfenidone treatment in patients with idiopathic pulmonary fibrosis (IPF)
| Autor: | Graziella Turato, Chiara Rigobello, Simonetta Baraldo, Elisabetta Balestro, Marina Saetta, Manuela Campisi, Alessia Fraccaro, Paolo Spagnolo, Davide Biondini, Sofia Pavanello, Manuel G. Cosio, Erica Bazzan |
|---|---|
| Rok vydání: | 2016 |
| Předmět: |
Pathology
medicine.medical_specialty business.industry Disease progression Clinical course Pirfenidone Disease behaviour medicine.disease Gastroenterology Peripheral blood 03 medical and health sciences FEV1/FVC ratio Idiopathic pulmonary fibrosis 0302 clinical medicine 030228 respiratory system Internal medicine Medicine In patient 030212 general & internal medicine business medicine.drug |
| Zdroj: | 1.5 Diffuse Parenchymal Lung Disease. |
| DOI: | 10.1183/13993003.congress-2016.oa4965 |
| Popis: | The clinical course of IPF is heterogeneous and unpredictable, however a rapid decline in FVC and telomere shortening appear to be related to worse prognosis. It has been shown that treatment with Pirfenidone can modify disease progression in the majority of patients. Aims: To investigate whether a different pre-treatment disease progression (FVC decline 10% pred/year) is related to leukocyte telomere length (LTL) and can influence the response to Pirfenidone. Methods: We studied the response to Pirfenidone treatment in 26 patients with IPF who had been followed for 1 year prior the beginning of treatment. According to the decline of % predicted FVC before treatment they were classified as slow (≤10%) or rapid (>10%) progressors. Real-time PCR was used to measure LT length in genomic DNA from peripheral blood leukocytes in patients and in 41 healthy controls. Results: After 1 year on treatment the FVC % predicted decline in rapid progressors (n=9) changed from 19± 2% (absolute value 684±84ml) pre-treatment to 4±2% (162±72ml) (p Conclusion: One year of Pirfenidone treatment significantly decreases the rate of FVC decline in patients with pre-treatment rapid progression, but not in slow progressors. Telomere length does not appear to influence disease behaviour. |
| Databáze: | OpenAIRE |
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