Pulmonary fibrosis describes a group of diseases which produce interstitial lung damage and ultimately fibrosis and loss of the elasticity of the lungs. It is a chronic condition characterised by shortness of breath, diffuse infiltrates on CXR and inflammation and/or fibrosis on biopsy. Pulmonary fibrosis may be secondary to a wide range of diseases or may be idiopathic with no known underlying cause. There are three types of lung fibrosis:
- Replacement fibrosis secondary to lung damage - eg, infarction, tuberculosis and pneumonia.
- Focal fibrosis in response to irritants - eg, coal dust and silica.
- Diffuse parenchymal lung disease (DPLD), which occurs in idiopathic pulmonary fibrosis (IPF) and extrinsic allergic alveolitis.
- The aetiology and pathogenesis of IPF is unknown. The condition is part of a spectrum of conditions known as interstitial lung diseases.
- Pulmonary fibrosis can occur in association with a number of connective tissue diseases, including rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and Sjögren's syndrome.
- Pulmonary fibrosis can also result from certain occupational exposures, including asbestos, coal dust and silica. Some medications may also cause lung fibrosis, including amiodarone, nitrofurantoin and bleomycin.
- Pulmonary fibrosis may also occur following inhalation of irritant substances, such as with hypersensitivity pneumonitis caused by exposure to birds or moulds.
IPF is the most common interstitial lung disease, with an estimated incidence in the UK of around 7.44 per 100,000 population.
IPF is rare in people younger than 45 years and in the UK the median age of presentation is 70 years. The prevalence is around 15 to 25 cases per 100,000 people and increases with age. Most people with IPF smoke or have a history of smoking. IPF often co-exists with chronic obstructive pulmonary disease.
IPF may show familial clusters (<5% of all cases) but the genetic reason for this is not yet fully understood, as it does not occur in a predictable fashion.
- Smoking - risk factor in familial and sporadic cases, especially if there is a smoking history of >20 pack years.
- The condition is common in certain occupations - for example, in people who work with silica, asbestos, heavy metals or mouldy foliage.
- Environmental factors include pigeon breeding and contaminated ventilation systems.
- Chronic viral infections have been investigated as potential risk factors, with a focus on hepatitis C and Epstein-Barr viruses.
- Gastro-oesophageal reflux disease with micro-aspiration.
The diagnosis of pulmonary fibrosis should be considered in any patient presenting with breathlessness, especially if there is a pre-existing medical history or environmental exposure possibly associated with pulmonary fibrosis (see above).
- Age over 45 years.
- Persistent breathlessness on exertion.
- Persistent dry cough.
- Bilateral inspiratory crackles on auscultation of the chest.
- Clubbing of the fingers
- Normal spirometry or impaired spirometry usually with a restrictive pattern but sometimes with an obstructive pattern.
Patients may be systemically unwell and may have a flu-like illness, fatigue or weight loss at presentation. Extrapulmonary features may include arthralgia, muscle pains and skin rashes. Obstructive sleep apnoea may be a common presenting feature.
Other signs on examination may include tachypnoea, cyanosis and also signs of cor pulmonale and right heart failure in the later stages.
- Initial assessment should include a detailed history, carrying out a clinical examination and performing blood tests (eg, FBC, ESR, CRP, autoantibodies) to assess environmental and occupational exposure, connective tissue diseases and with drug history.
- Lung function testing (spirometry and gas transfer).
- Performing CT scans of the thorax (including high-resolution images - HRCT). HRCT reveals pattern of usual interstitial pneumonia (subpleural basal predominance, reticular pattern, honeycombing, and absence of inconsistent features - eg, micronodules or cysts.
- If a confident diagnosis cannot be made on the basis of clinical features, lung function and radiological findings, bronchoalveolar lavage or transbronchial biopsy and/or surgical lung biopsy should be considered.
Due to the nonspecific nature of the presenting symptoms and signs, there are many other diagnoses which must be considered, ranging from very common disorders such as heart failure through to much rarer diseases. Other diagnoses to be considered include:
- Heart failure.
- Chronic obstructive pulmonary disease.
- Pulmonary embolism.
- Lung cancer
- Extrinsic allergic alveolitis.
IPF may be found in association with several autoimmune disorders such as:
- Thyroid disease
- Systemic sclerosis
- Rheumatoid arthritis
- Autoimmune liver disease
- Systemic lupus erythematosis
Management should be under a multidisciplinary team and include the management of any underlying cause associated with pulmonary fibrosis and an assessment for pulmonary rehabilitation.
Assessments for pulmonary rehabilitation should be made at the time of diagnosis and repeated at 6-month or 12-month intervals. Pulmonary rehabilitation including exercise and educational components should be tailored to the needs of each individual patient.
- Supportive therapy with oxygen is recommended for those with significant resting hypoxaemia.
- Physiotherapy may be helpful.
- Regular exercise and weight control should be encouraged.
- Vaccinate against influenza and pneumococcus.
- Encourage the patient to stop smoking if he or she continues to do so.
- Proton pump inhibitor therapy should be considered in all and has been associated with stabilisation in lung function.
- In end-stage disease, opiates may help excessive cough.
Pulmonary rehabilitation seems to be safe for people with pulmonary fibrosis and improvements in functional exercise capacity, dyspnoea and quality of life immediately following pulmonary rehabilitation have been shown. There is little evidence regarding longer-term effects of pulmonary rehabilitation.
It has been acknowledged for some time that the effectiveness of medical therapies has been disappointing. Medication should be initiated under specialist supervision. The management will include treatment of any underlying cause, oxygen therapy and other supportive treatments for symptoms.
- Pirfenidone is recommended by the National Institute for Health and Care Excellence (NICE) as an option for treating IPF but only if the person has a forced vital capacity (FVC) between 50% and 80% predicted.
- Nintedanib, an intracellular tyrosine kinase inhibitor with antifibrotic and anti-inflammatory properties, is newly licensed for the treatment of IPF and has been approved by NICE. It has been shown in clinical trials to slow the rate of decline of pulmonary function tests similar to pirfenidone.
Neither pirfenidone nor nintedanib is a cure for IPF and disease severity for most patients continues to progress despite treatment.
Lung transplant may be required for patients who fail to respond to medical therapy. Lung transplantation offers survival benefits in carefully selected patients. 23% of lung transplants worldwide are performed on patients with IPF.
Patient selection is important and it is recommended to refer patients with IPF for consideration of transplant when they demonstrate a 10% or greater reduction in FVC over six months and a decrease in oxygen saturations to below 89% or the need for oxygen at rest.
Other treatments which may need to be considered
- Acute exacerbations - corticosteroids are recommended.
- Pulmonary hypertension - treatment is not recommended in the majority but clinicians need to review each case individually.
These may include:
- Pulmonary hypertension.
- Lung cancer.
- Pulmonary embolism.
- Right ventricular heart failure.
- Coronary heart disease.
The median survival for people with IPF in the UK is approximately 2.5 years from the time of diagnosis.
However, the rate of disease progression varies and about 20% of people with the disease survive for more than five years.The prognosis for other causes of pulmonary fibrosis will depend on the cause and comorbidity.
Further reading and references
Nintedanib for treating idiopathic pulmonary fibrosis; NICE Technology Appraisal Guidance (January 2016)
Wallis A, Spinks K; The diagnosis and management of interstitial lung diseases. BMJ. 2015 May 7350:h2072. doi: 10.1136/bmj.h2072.
Idiopathic pulmonary fibrosis; NICE Quality Standard, January 2015
Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management; European Respiratory Society and others (2011)
Idiopathic pulmonary fibrosis: the diagnosis and management of suspected idiopathic pulmonary fibrosis; NICE Clinical Guideline (June 2013)
Prasad R, Gupta N, Singh A, et al; Diagnosis of idiopathic pulmonary fibrosis: Current issues. Intractable Rare Dis Res. 2015 May4(2):65-9. doi: 10.5582/irdr.2015.01009.
Meyer KC; Diagnosis and management of interstitial lung disease. Transl Respir Med. 2014 Feb 132:4. doi: 10.1186/2213-0802-2-4. eCollection 2014.
Dowman L, Hill CJ, Holland AE; Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014 Oct 610:CD006322. doi: 10.1002/14651858.CD006322.pub3.
Pirfenidone for treating idiopathic pulmonary fibrosis; NICE Technology Appraisal Guidance, April 2013
Tzouvelekis A, Bonella F, Spagnolo P; Update on therapeutic management of idiopathic pulmonary fibrosis. Ther Clin Risk Manag. 2015 Mar 311:359-70. doi: 10.2147/TCRM.S69716. eCollection 2015.
Kistler KD, Nalysnyk L, Rotella P, et al; Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature. BMC Pulm Med. 2014 Aug 1614:139. doi: 10.1186/1471-2466-14-139.
Costabel U; The changing treatment landscape in idiopathic pulmonary fibrosis. Eur Respir Rev. 2015 Mar24(135):65-8. doi: 10.1183/09059180.00011414.
Hyldgaard C, Hilberg O, Bendstrup E; How does comorbidity influence survival in idiopathic pulmonary fibrosis? Respir Med. 2014 Apr108(4):647-53. doi: 10.1016/j.rmed.2014.01.008. Epub 2014 Feb 2.
Has anyone been diagnosed with pleuroparnchymal fibroelastosis (PPFE). Pulmonologist and radiologist think I might have it. Not much written on it - fairly new to the medical community, I believe....CherylH
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