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Faculty of Medical Sciences

Ziekteprogressie bij patiënten met idiopathische longfibrose

Ploeg, E.A. van der (2017) Ziekteprogressie bij patiënten met idiopathische longfibrose. thesis, Medicine.

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Abstract

Background Idiopathic pulmonary fibrosis is an invalidating and incurable disease. It is characterized by restriction of lung volume. Two antifibrotic therapies are now available, nintedanib and pirfenidon. To provide patient specific care, knowledge of disease progression is needed. This study researches patient characteristics, for example comorbidities, BMI and use of antifibrotic therapy as possible predictors for disease progression 1 year after presentation. Also the effectiveness of antifibrotic therapy in this cohort is evaluated. Also presence of clubbing and its relation to percentage predicted FVC and DLCO at presentation was researched. Methods Seventy-seven patients were included in this single-center cohort study conducted in the Martini hospital in Groningen. Baseline data and longitudinal spirometry data were collected as well as spirometry data when patients were on antifibrotic therapy. Primary endpoint was absolute decline in percentage predicted FVC and DLCO as a surrogate marker for disease progression. Secondary endpoint was an absolute decline of at least 10 percentage points of FVC or death within the first year after presentation. Average percentage predicted FVC and DLCO was compared between patients with and without clubbing at presentation. Results No patient characteristics could be identified that predicted decline in percentage predicted FVC or DLCO 1 year after presentation. Current or former smoking was a risk factor for mortality or decline in percentage predicted FVC of at least 10 percentage points 1 year after presentation (OR 5.7, CI 1.004–31.9, p=0.049). Comorbidities or use of antifibrotic therapy were no risk factors for reaching this secondary endpoint. Use of antifibrotic therapy reduced decline of percentage predicted FVC after one year of therapy, -12.9±13.1 percent versus -2.5±10.9 percent (CI-18.4- -2.3, p=0.01). However, no significant difference was found in disease progression between patients with use of antifibrotics and patients who did not use antifibrotics (CI -9.7 – 6.1, p=0.65). presence of clubbing was not related to a difference in percentage predicted FVC or DLCO at presentation. Conclusions Patient characteristics, including clubbing, could not predict disease progression after one year. Current or former smoking is a risk factor for mortality and fast progression. Antifibrotic therapy is effective in reducing decline in percentage predicted FVC, however future research is necessary to verify these results and do further research on predictors for disease progression to promote patient care.

Item Type: Thesis (Thesis)
Supervisor name: Naam begeleider: and Kramer, Dr. H. and Longarts and Martini Ziekenhuis and Afdeling: Longgeneeskunde, Martini Ziekenhuis te Groningen
Faculty: Medical Sciences
Date Deposited: 25 Jun 2020 10:56
Last Modified: 25 Jun 2020 10:56
URI: https://umcg.studenttheses.ub.rug.nl/id/eprint/1637

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