2013 NICE 163 临床指南：特发性纤维化诊断管理临床指南
Idiopathic pulmonary fibrosis(NICE clinical guideline 163)
2013-06-12
Idiopathic pulmonary fibrosis is a chronic, progressive fibrotic interstitial lung disease of unknownorigin. It is a difficult disease to diagnose and often requires the collaborative expertise of aconsultant respiratory physician, radiologist and histopathologist to reach a consensus diagnosis.Most people with idiopathic pulmonary fibrosis experience symptoms of breathlessness, whichmay initially be only on exertion. Cough, with or without sputum, is a common symptom. Overtime, these symptoms are associated with a decline in lung function, reduced quality of life andultimately death.The median survival for people with idiopathic pulmonary fibrosis in the UK is approximately3 years from the time of diagnosis. However, about 20% of people with the disease survive formore than 5 years. The rate of disease progression can vary greatly. A person's prognosis isdifficult to estimate at the time of diagnosis and may only become apparent after a period ofcareful follow-up.This guideline contains recommendations on the diagnosis of idiopathic pulmonary fibrosis anddelivery of care to people with idiopathic pulmonary fibrosis, from initial suspicion of the diseaseand referral to a consultant respiratory physician, to best supportive care and disease-modifyingtreatments.The guideline will assume that prescribers will use a drug's summary of product characteristics toinform decisions made with individual patients.This guideline recommends some drugs for indications for which they do not have a UKmarketing authorisation at the date of publication, if there is good evidence to support that use.The prescriber should follow relevant professional guidance, taking full responsibility for thedecision.The patient (or those with authority to give consent on their behalf) should provideinformed consent, which should be documented. See the General Medical Council's Goodpractice in prescribing and managing medicines and devices for further information. Whererecommendations have been made for the use of drugs outside their licensed indications ('offlabeluse'), these drugs are marked with a footnote in the recommendations.
