Interstitial lung disease Flashcards
What does pulmonary fibrosis mean
Describes a group of diseases which produce interstitial lung damage and ultimately fibrosis and loss of the elasticity of the lungs
Types of lung fibrosis
Replacement fibrosis secondary to lung damage
Focal fibrosis in response to irritants
Diffuse parenchymal lung disease
Causes of replacement lung fibrosis
Infarction
TB
Pneumonia
Irritants which can cause focal lung fibrosis
Coal dust and silica
Which conditions are often associated with pulmonary fibrosis
Connective tissue diseases, including rheumatoid arthritis, SLE, systemic sclerosis and sjogren’s syndrome
Which medications may be associated with lung fibrosis
Amiodarone
Nitrofurantoin
Bleomycin
Which condition often co-exists with idiopathic pulmonary fibrosis
Chronic obstructive pulmonary disease
Risk factors for pulmonary fibrosis
Smoking Certain occupations - exposure to asbestos Environmental factors Chronic viral infections GORD with micro-aspiration
Examples of environmental factors thought to contribute to pulmonary fibrosis
Pigeon breeding and contaminated ventilation systems
Chronic viral infections which are thought to be risk factors for pulmonary fibrosis
Hepatitis C
Epstein-Barr viruses
Presentation of pulmonary fibrosis
Age over 45 years Persistent breathlessness on exertion Persistent dry cough Fine bilateral inspiratory crackles on auscultation of the chest Clubbing Systemically unwell
What signs might you find in an individual with interstitial lung disease on examination
Tachypnoea
Cyanosis
Signs of cor pulmonale and right heart failure in later stages
Diagnosis of idiopathic pulmonary fibrosis
Clinical history and exam
Lung function testing(spirometry and gas transfer)
CXR
High resolution CT scan is gold standard
Secondary investigations of diagnosis of pulmonary fibrosis cannot be made on initial ix
Bronchoalveolar lavage
Trasbronchial biopsy
Surgical lung biopsy
How often should an individual with pulmonary fibrosis be assessed
At diagnosis
6 months
12 months
Repeat the lung function tests at shorter intervals if there is concern
Supportive management of pulmonary fibrosis
Supportive therapy with oxygen for significant resting hypoxaemia
Pulmonary rehabilitation
Regular exercise and weight control
Vaccinations
Smoking cessation
PPI therapy
Medical management of pulmonary fibrosis
Pirefenidone
Nintedanib
When is pirfenidone recommended in management of pulmonary fibrosis
Forced vital capacity between 50% and 80% predicted
Treatment is stooped if there is evidence of disease progression
Surgical management of pulmonary fibrosis
Lung transplant
Considered if a 10% or greater reduction in FVC over six months and a decrease in oxygen stats to below 89% or need for oxygen at rest
Complications of pulmonary fibrosis
Pulmonary hypertension Lung cancer Pulmonary embolism Right ventricular heart failure Coronary heart disease
Which medications can be used in management of cough if it is debilitating
Opioids
Discuss treatment with thalidomide with specialist if cough in interstitial lung disease is intractable
Spirometry findings in idiopathic pulmonary fibrosis
a restrictive picture (FEV1 normal/decreased, FVC decreased, FEV1/FVC increased)
Impaired gas exchange - reduced transfer factor(TLCO)
Causes of fibrosis affecting mainly upper zones
C - Coal worker's pneumoconiosis H - Histiocytosis/ hypersensitivity pneumonitis A - Ankylosing spondylitis R - Radiation T - Tuberculosis S - Silicosis/sarcoidosis
Causes of fibrosis mainly affecting lower zones
idiopathic pulmonary fibrosis
most connective tissue disorders (except ankylosing spondylitis) e.g. SLE
drug-induced: amiodarone, bleomycin, methotrexate
asbestosis
