Restrictive Lung Disease Flashcards
Other words for restrictive lung disease
Interstitial, diffuse lung disease
What is the lung interstitium
The connective tissue space around airways and vessels and between basement membranes of alveolar walls. In normal they’re in contact
Signs of restrictive lung disease
Reduced lung compliance, low FEV1 and FVC but normal ratio, reduced gas transfer (Tco and Kco), ventilation perfusion imbalance
How is diffuse lung disease found
Abnormal CT or CXR, dyspnoea, respiratory failure, heart failure
Diffuse alveolar damage associations
Major trauma, chemical injury, circulatory shock, drugs, infection, radiation, autoimmune
How many ribs can you see in a normal CXR
Posterior 10 ribs (less with restrictive, more with obstructive)
Exudative stage of DADS
Edema and inflammation and dense eosinophilic hyaline membranes
Proliferative stage of DADs
Interstitial inflammation and fibrosis
Histological features of DADs
Protein rich oedema, fibrin, hyaline membranes, denuded basement membranes, epithelium and fibroblast proliferation, scarring
Sarcoidosis
A multisystem granulomatous disorder of unknown aetiology
Histopathology of sarcoidosis
Epithelial and giant cell granulomas, associated fibrosis, little lymphoid infiltrate
Who does sarcoidosis affect
Young adults, more females than men, more African Americans in USA, in temperate climates
Organs involved in sarcoidosis
Lymph nodes, lung, spleen, liver, skin eyes skeletal muscle, bone marrow, salivary glands
How does sarcoidosis present in young adults
Painful joints, swollen fat under skin, bilateral lymphadenopathy
Antigens in hypersensitivity pneumonitis
Thermophilic actinomycetes, bird/animal proteins eg faeces, fungi, chemicals
Acute Hypersensitivity pneumonitis presentation
Fever, dry cough, myalgia, chills, crackles, tachypnoea, wheeze, precipitating antibody
Chronic hypersensitivity pneumonitis presentation
Insidious, malaise, SOB, cough, crackles and some wheeze
Histopathology of hypersensitivity pneumonitis
Type 3 and 4 immune complex mediated hypersensitivity reactions, soft Centri-acinar epitheloid granulomas, Upper zone
Causes of usual interstitial pneumonitis
Connective tissue diseases, drug reaction, post infection, industrial exposure. But most are idiopathic
Histopathology of UIP
Patchy interstitial chronic inflammation, type 2 pnuemocyte hyperplasia (surfactant), smooth muscle and vascular proliferation. Evidence of both old and new injury
UIP in clinic
Idiopathic Pulmonary fibrosis
IPF signs
Elderly and m>f, dyspnoea, cough, basal crackles, cyanosis, clubbing. Most dead within 5 years. CXR shows basal posterior fibrosis and honeycombing, reduced gas transfer
Physiological definition of restriction
Forced vital capacity <80% of normal
Parts of the body that can cause restrictive disease
Lungs, pleura, nerve, muscle, bone
Pleural causes of restriction
Pleural effusion, pnuemothorax, pleural thickening
Skeletal causes of restriction
Kyphoscoliosis Ankylosing spondylitis Thoracoplasty Rib fractures Amyotrophic lateral sclerosis
Sub diaphragmatic causes of restriction
Obesity
Pregnancy
What is the interstitium
Space between alveolar epithelium and capillary endothelium, usually thin
What are interstitial lung diseases
Diseases causing thickening of the interstitium and which can result in pulmonary fibrosis
(sarcoidosis, idiopathic pulmonary fibrosis, hypersensitivity pneumonitis)
What is sarcoidosis
A multisystem granulomatous disease of unknown cause. Non caseating granuloma
Symptoms of sarcoidosis
Erythema nodosum, swollen eye (anterior uveitis), skin lesions, cough
Investigations for sarcoidosis
History and exam and CXR
Then pulmonary function tests, bloods, urinalysis, ECG, TB skin test, eye exam
Then bronchoscopy including biopsies and EBUS
CXR signs of sarcoidosis
Nodal adenopathy, snow storm look,
Surgical biopsies for sarcoidosis
Mediastinoscopy and VATS
How should mild sarcoidosis be treated (no vital organ involvement, normal lung function,few symptoms)
No treatment
How should sarcoidosis with erythema nodosum or arthralgia (joint pain) be treated
NSAIDS
How should sarcoidosis with skin lesions/anterior uveitis/ cough be treated
Topical steroids
How should sarcoidosis with cardiac/neurological /eye disease not responding / Hypercalcaemia be treated
Systemic steroids
Respiratory complications of sarcoidosis
Progressive respiratory failure
Bronchiectasis
Aspergilloma, haemoptysis, pnuemothorax
Typical presentation of idiopathic pulmonary fibrosis
Chronic breathlessness and cough, 60-70 years old, failed infection/left ventricular failure treatment / clubbed fingers and crackles
Idiopathic Pulmonary fibrosis CXR
Fluffy base
Median survival for IPF
3 years
Treatment for IPF
Lung transplant.
Possibly oral anti fibrotics eg pirfenidone, nintedanib, palliative care
Causes of hypersensitivity pneumonitis
Hay, birds etc
