Clinical features and management of restrictive lung disease Flashcards
What is the physiological definition of a restrictive lung disease?
Forced vital capacity <80% of the predicted normal
How does a Restrictive lung disease affect the lungs generally
They restrict lung expansion
Leading to decreased lung volume and increased work of breathing
What tissues/organs generally cause a restrictive lung disease?
Lungs
Pleura
Nerves/muscles
Bones (skeletal)
(There are others)
What are the 3 big restrictive lung diseases?
Idiopathic pulmonary fibrosis
Sarcoidosis
hypersensitivity pneumonitis
What is an alternative term for these restrictive lung diseases?
Interstitial lung diseases
What pleural conditions are restrictive?
Pleural effusions
Pneumothorax
Pleural thickening
What skeletal conditions are restrictive?
Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures
What neuromuscular conditions are restrictive?
Amyotrophic lateral sclerosis
What sub-diaphragmatic causes are there for restriction of the lungs?
Obesity
Pregnancy
Histologically, what kind of always happens in a restrictive lung disease?
Thickening of the interstitium
ie thickening of the alveolar walls, and spaces between alveolar wall and capillaries and all that stuff
There are over 200 interstitial lung diseases
What can they all lead to, if not treated?
Pulmonary fibrosis
What is pulmonary fibrosis?
Scarring and thickening of the lung tissue
permanent thickening of the interstitium
What is sarcoidosis?
Multisystem granulomatous disease of unknown cause
Characterised by non caseating granulomas (collections of inflammatory cells) in the lungs, skin or lymph nodes
Who is prone to sarcoidosis?
Adults > 40
Women > men
World wide
How do you initially investigate a patient presenting with possible sarcoidosis
History
Examination
CXR
A patient’s CXR strongly indicates sarcoidosis
How do you further investigate it?
Pulmonary function tests
Bloods Urinalysis ECG TB skin test Eye exam
Bronchoscopy
Biopsy
Describe the bronchoscopy process for investigating sarcoidosis
Bronchoscopy including transbronchial biopsies and endobronchial ultrasound
What surgical biopsies can be used for sarcoidosis if needed?
Mediastinoscopy
Video assisted thoracoscopic lung biopsy (VATS)
What general type of drugs is used to treat sarcoidosis?
Steroids
When would sarcoidosis NOT be treated?
Mild disease, no vital organ involvement, normal lung function, few symptoms
When would NSAIDs be used for treatment of sarcoidosis?
-Moderate level of disease-
Erythema nodosum / arthralgia
When would topical steroids be used for treatment?
-Serious level of disease-
Skin lesions / anterior uveitis / cough
When would systemic steroids be used to treat sarcoidosis?
Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia
What is the prognosis for sarcoidosis?
Pretty decent
<1% caucasians die
10-20% sustain permanent pulmonary or extra-pulmonary complications
What respiratory complications are mainly seen with chronic sarcoidosis?
Progressive respiratory failure
Bronchiectasis
Aspergilloma, haemoptysis, pneumothorax
What is the typical presentation for idiopathic pulmonary fibrosis?
Chronic breathlessness and cough
Typically older (60-70), mainly men
Failed Rx for “LVF” or infection
Clubbed fingers
Crackles
What is the general prognosis for idiopathic pulmonary fibrosis?
Median survival 3 years
What are the pharmacological options for idiopathic pulmonary fibrosis?
OAF - oral anti-fibrotic
Pirfenidone
Nintedanib
(Not sure if the drugs above are OAFs or different)
What surgical option can be used for IPF?
Lung transplant
Aside from drugs or surgery
What care do patients with IPF receive?
Refer to ILD clinic (Interstitial lung disease)
Palliative care
