Restrictive Lung Disease Flashcards
What is pulmonary fibrosis?
Excess collagen/scar tissue in the interstitial tissue of the lung
What are the causes of pulmonary fibrosis?
Occupation
- Exposure to pets/birds EAA (extrinsic allergic alveolitis)
Previous radiotherapy
Drugs
- Amiodarone
- Methotrexate
- Nitrofurantoin
- Penicillamine
- Bleomycin
- Cyclophosphamide
Systemic disorders
- Vasculitis
- Wegener’s granulomatosis
- Churg–Strauss syndrome
- Behçet’s syndrome
- Goodpasture’s syndrome
- Sarcoidosis
Connective tissue disorders
- SLE
- Rheumatoid arthritis
- Sjögren’s syndrome
- Systemic sclerosis
- Polymyositis
Neoplasm
- Lymphoma
- Lymphangitic carcinoma
Inherited disorders
- Tuberous sclerosis
- Neurofibromatosis
Others
- Amyloidosis
What drugs can cause pulmonary fibrosis?
Amiodarone
Methotrexate
Nitrofurantoin
Penicillamine
Bleomycin
Cyclophosphamide
What investigations are used in pulmonary fibrosis diagnosis?
Bloods
- FBC
- U&E
- LFT
- Calcium levels
- ACE (angiotensin-converting enzyme) levels
Immunology
- Anti-GBM (glomerular basement membrane)
- ANA (antinuclear antibodies)
- ANCA (antineutrophil cytoplasmic antibodies)
- Rheumatoid factor
- Serum precipitins
Imaging
- CXR
- HRCT
Spirometry
- Restrictive pattern
Bronchoscopy + biopsy
Describe a restrictive pattern in spirometry
decreased FEV1
decreased FVC
normal or increased FEV1/FVC
Describe the presentation of idiopathic pulmonary fibrosis
Clinically slow progression
Cough
Dyspnoea
Clubbing
Fine bi basal end inspiratory crackles
What is the prognosis of idiopathic pulmonary fibrosis?
poor, average life expectnancy 3-4 years
What is the management of idiopathic pulmonary fibrosis?
Pulmonary rehabilitation
Oral Anti-Fibrotic Drugs (OAF)
- Pirfenidone
Lung transplant
What is Sarcoidosis?
Chronic multisystem non-caseating granulomatous inflammatory disorder, most commonly affecting the lung
Describe the presentation of sarcoidosis
Depends on the organs involved
Dyspnoea
Dry cough
Erythema Nodosum
- Raised marks on legs
Red eyes
Redness around scars
Lupus pernio pertains
- Rash, not normally itchy or painful, affecting nose, lips, cheek, ears and digits
Arthralgia
Bilateral Hilar Lymphadenopathy
Systemic
- Fever
- Malaise
- Weight loss
What investigations are used in the diagnosis and monitoring of sarcoidosis?
CXR
>Ca2+ Hypercalcaemia
- Macrophages inside the granulomas cause an increased conversion of vitamin D to its active form
Bronchoscopy and biopsy
- Red
- Granulomas
Eye Examination
- Assess for fibroids in back of eye
>Serum ACE levels
- Poorly specific but can be used to monitor disease activity
What causes sarcoidosis?
Idiopathic
What is the management of sarcoidosis?
No treatment if mild disease
NSAIDS
Steroids
For resistant disease consider
- Methotrexate
- Chloroquine
Name some possible complications of sarcoidosis?
Pneumothorax
Bronchiectasis
Respiratory failure
What CXR signs are seen in Sarcoidosis?
Large hilum area/bilateral hilar lymphadenopathy
Snow storm lungs
What group is sarcoidosis more common in?
Afro-Carribean ethnicity
F>M
What are the indications for steroid use in sarcoidosis?
Parenchymal lung disease
Uveitis
Hypercalcaemia
Neurological or cardiac involvement
What is the prognosis of sarcoidosis?
Sarcoidosis spontaneously resolves within 6 months in around 60% of patients
In a small number of patients it progresses with pulmonary fibrosis and pulmonary hypertension, potentially requiring a lung transplant
Death in sarcoidosis is usually when it affects the heart (causing arrhythmias) or the central nervous system
What is hypersensitivity pneumonitis?
Immunological disease characterised by hypersensitivity to various antigens resulting in a allergic reaction, also known as extrinsic allergic alveolitis (EAA)
Describe the presentation of hypersensitivity pneumonitis?
Symptoms usually regress when causative agent is removed
Dry cough
Pyrexia
Myalgia
Rigors 4-9 hours after antigen exposure
Tachypnoea
Auscultation
- Wheeze
- Crackles
What type of hypersensitivity reaction is involved in hypersensitivity pneumonitis?
Type 3 and 4 hypersensitivity reaction
What are the causes of hypersensitivity pneumonitis?
Chemicals
Bird/animal proteins (Avian protein)
Fungi
Bacteria
Thermactinomyces in mouldy hay
What causes ‘bird lung’?
Avian protein on bird feathers
What causes farmers lung?
Thermactinomyces in mouldy hay
What is the management of hypersensitivity pneumonitis?
Corticosteroids
Avoid antigen
