Severe idiopathic pulmonary fibrosis: what can be done?
Idiopathic pulmonary fibrosis (IPF) remains a challenging disease to manage. Two drugs are now available that can slow disease progression in patients with mild-to-moderate IPF. This means that early diagnosis is mandatory, because there are no proven effective therapies for severe IPF. This lack of...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
European Respiratory Society
2017-09-01
|
| Series: | European Respiratory Review |
| Online Access: | http://err.ersjournals.com/content/26/145/170047.full |
| _version_ | 1818149865553657856 |
|---|---|
| author | Antonella Caminati Roberto Cassandro Olga Torre Sergio Harari |
| author_facet | Antonella Caminati Roberto Cassandro Olga Torre Sergio Harari |
| author_sort | Antonella Caminati |
| collection | DOAJ |
| description | Idiopathic pulmonary fibrosis (IPF) remains a challenging disease to manage. Two drugs are now available that can slow disease progression in patients with mild-to-moderate IPF. This means that early diagnosis is mandatory, because there are no proven effective therapies for severe IPF. This lack of proven therapies may be at least partially due to the fact that severe IPF patients are usually not enrolled in randomised, prospective, multicentre, international trials. Clinical observation experiences and preliminary results of long-term, open-label extensions of clinical trials suggest that both pirfenidone and nintedanib may also slow or decrease progression in patients with severe IPF. However, data are sparse and obtained from a relatively small number of patients. Lung transplantation should be taken into account early and discussed with patients, when indicated. Rehabilitative strategies are important and effective supportive therapies. The needs of patients with severe IPF are similar to those of patients with an advanced neoplastic disease. Palliative care and psychological support play an important role in the relief of symptoms of anxiety and depression. Accordingly, these therapeutic approaches should start early in IPF patients. |
| first_indexed | 2024-12-11T13:13:50Z |
| format | Article |
| id | doaj.art-2e4eb99c091843749c9f7aca08ed138a |
| institution | Directory Open Access Journal |
| issn | 0905-9180 1600-0617 |
| language | English |
| last_indexed | 2024-12-11T13:13:50Z |
| publishDate | 2017-09-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | European Respiratory Review |
| spelling | doaj.art-2e4eb99c091843749c9f7aca08ed138a2022-12-22T01:06:07ZengEuropean Respiratory SocietyEuropean Respiratory Review0905-91801600-06172017-09-012614510.1183/16000617.0047-20170047-2017Severe idiopathic pulmonary fibrosis: what can be done?Antonella Caminati0Roberto Cassandro1Olga Torre2Sergio Harari3 U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, MultiMedica IRCCS, Milan, Italy U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, MultiMedica IRCCS, Milan, Italy U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, MultiMedica IRCCS, Milan, Italy U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, MultiMedica IRCCS, Milan, Italy Idiopathic pulmonary fibrosis (IPF) remains a challenging disease to manage. Two drugs are now available that can slow disease progression in patients with mild-to-moderate IPF. This means that early diagnosis is mandatory, because there are no proven effective therapies for severe IPF. This lack of proven therapies may be at least partially due to the fact that severe IPF patients are usually not enrolled in randomised, prospective, multicentre, international trials. Clinical observation experiences and preliminary results of long-term, open-label extensions of clinical trials suggest that both pirfenidone and nintedanib may also slow or decrease progression in patients with severe IPF. However, data are sparse and obtained from a relatively small number of patients. Lung transplantation should be taken into account early and discussed with patients, when indicated. Rehabilitative strategies are important and effective supportive therapies. The needs of patients with severe IPF are similar to those of patients with an advanced neoplastic disease. Palliative care and psychological support play an important role in the relief of symptoms of anxiety and depression. Accordingly, these therapeutic approaches should start early in IPF patients.http://err.ersjournals.com/content/26/145/170047.full |
| spellingShingle | Antonella Caminati Roberto Cassandro Olga Torre Sergio Harari Severe idiopathic pulmonary fibrosis: what can be done? European Respiratory Review |
| title | Severe idiopathic pulmonary fibrosis: what can be done? |
| title_full | Severe idiopathic pulmonary fibrosis: what can be done? |
| title_fullStr | Severe idiopathic pulmonary fibrosis: what can be done? |
| title_full_unstemmed | Severe idiopathic pulmonary fibrosis: what can be done? |
| title_short | Severe idiopathic pulmonary fibrosis: what can be done? |
| title_sort | severe idiopathic pulmonary fibrosis what can be done |
| url | http://err.ersjournals.com/content/26/145/170047.full |
| work_keys_str_mv | AT antonellacaminati severeidiopathicpulmonaryfibrosiswhatcanbedone AT robertocassandro severeidiopathicpulmonaryfibrosiswhatcanbedone AT olgatorre severeidiopathicpulmonaryfibrosiswhatcanbedone AT sergioharari severeidiopathicpulmonaryfibrosiswhatcanbedone |