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Newer Modes of Treating Interstitial Lung Disease

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Newer Modes of Treating Interstitial Lung Disease


Wim A. Wuyts, Michiel Thomeer, Maurits G. Demedts Curr Opin Pulm Med. 2011;17(5):332336

Disclosures

Abstract and Introduction Treatment Practical Approach Challenges for the Future Conclusion

Abstract and Introduction


Abstract
Purpose of Review This review critically discusses recent advances in the treatment of idiopathic pulmonary fibrosis (IPF). Moreover, it also focuses

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Newer Modes of Treating Interstitial Lung Disease

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on the practical approach of a patient diagnosed with IPF and uncovers challenges for the future. Recent Findings Treatment can be divided into three major parts. Firstly, many new agents have been tested, but only the combination of Nacetylcysteine with corticosteroids, azathioprine and pirfenidone was able to show some significant effects. In the mean time, many second-generation agents are under development. Lung transplantation has made some major progress by introducing an appropriate allocation system. Finally, as part of best supportive care, several studies show that pulmonary rehabilitation might induce some important effects on quality of life. Summary So, it is clear that major progress has been made in the treatment of IPF, but we are convinced that an orchestrated effort will lead to a better understanding and treatment of this devastating condition. MOST POPULAR ARTICLES
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Management of Interstitial Lung Disease in Elderly Patients Pneumonitis and Pulmonary Fibrosis in a Patient Receiving Adjuvant Docetaxel and Cyclophosphamide for Stage 3 Breast Cancer The Current Position of Surgical Lung Biopsy in the Diagnosis of Idiopathic Pulmonary Fibrosis

Introduction
Pulmonary fibrosis is the end stage of many different interstitial lung diseases, including interstitial lung diseases of known cause and idiopathic interstitial pneumonias (Table 1). As interstitial lung diseases cover more than 200 different disease entities, we will focus on the archetype of the fibrotic diseases: idiopathic pulmonary fibrosis (IPF). IPF has an estimated incidence of nine per 100 000 person-years and would affect about 500 000 patients in the USA and Europe.[1] For a long time, the pathogenesis of IPF has been considered as chronic inflammation leading to irreversible formation of fibrosis. In the last American Thoracic Society/European Respiratory Society guidelines, IPF is defined as a specific form of chronic fibrosing interstitial pneumonia limited to the lungs in association with a usual interstitial pneumonia pattern on histology. [2] It is a relentlessly fibrotic disease, consisting of a deposition of extracellular matrix causing architectural distortion of the lungs. IPF is refractory to current pharmacological intervention and mean survival is about 3 years after diagnosis. The present review provides a comprehensive overview of recent developments in the treatment of IPF. Many new antifibrotics of the first generation have been tested with mixed results, but second-generation, more powerful agents are in development. Next to drugs, we will also discuss progress in lung transplantation (LTX) and best supportive care. Moreover, we will provide a practical approach for the physician in selecting appropriate treatment for the individual patient. In the final section, we will focus on the future of treating pulmonary fibrosis on the basis of recently published articles.

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Curr Opin Pulm Med.2011;17(5):332 3362011LippincottWilliams& Wilkins

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