Restrictive lung disease Flashcards
What is diffuse alveolar damage associated eith?
Trauma Circulatory shock Chemical exposure/ toxic inhalation Drugs Infection Idiopathic Radiation Autoimmune disease
Name the histiological features of DAD.
Protein rich oedema Fibrin Hyaline membranes Epithelial proliferation Fibroblast proliferation Denuced basement membrane Scarring in the interstitium and air spaces
Describe how DAD occurs.
Vascular damage causes oedema
Oedema is rich in protein including fibrin
Proteinaceous cosgulation deposits creates hyaline membranes which causes inflammation
The inflammation leads to fibrosis within the interstitium and alveolar walls
What tests are carried out in the diagnosis of sarcoidosis?
Test for increased serum calcium Test for increased serum ACE levels TB skin test to rule out TB Biopsy Urinalysis Bloods
Further investigation:
- bronchoscopy and transthoracic biopsy
- mediastinoscopy
- video assisted thoracic lung biopsy
- endobronchial ultrasound
If a patient presents with lofgrens syndrome which is indicates sarcoidosis what is the treatment?
No treatment is given it is a self limiting condition.
If there is pain then give non steroidal anti inflammatory drugs
If a patient presents with skin lesions or anterior uvietis which indictaes sarcoidosis, what is the treatment?
And if the treatment doesnt work what other treatment can be given?
Topical steroids
Systemic steroids
10-20% of patients with sarcoidosis sustain permenant pulmonary or extra pulmonary complications. Name some of these:
Bronchiectasis Pnuemothorax Haemoptysis Progressive respiratory failure Aspirgilloma
what is a marker of restriction?
vital capacity
what is an abnormal vital capacity?
< 80%
what are the pleural causes of lung restriction?
pleural effusion
pneumothorax
pleural thickening
what are the bone causes of lung restriction?
Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty
Rib fractures
what is a muscle cause of lung restriction?
motor neurone disease
what are sub diaphragmatic causes of lung restriction?
obesity
pregnancy
what are interstitial lung diseases?
diseases which cause thickening of the interstitial and can result in pulmonary fibrosis
what restive lung disease is more common in females (< 40yrs) ?
sarcoidosis
what restrictive lung disease is more common in men > 50 yrs?
usual interstitial pneumonitis
idiopathic pulmonary fibrosis
what region is hypersensitivity pneumonitis likely to occur in the lungs?
upper zones
what is the acute presentation of hypersensitivity pneumonitis?
pyrexia dry cough myalgia chills 4-9hrs after Ag exposure tachypnoea crackles wheeze
what is the chronic presentation of hypersensitivity pneumonitis?
dyspnoea
cough
malaise / low grade illness
crackles wheeze
what is the presentation of lofgrens syndrome for sarcoidosis?
erythema nudism
acute arthralgia
bilateral hilar lymphadenopathy
what are the characteristics of the granulomata found in hypersensitivity pneumonitis?
soft centriacinar epithelioid granulomata
what type of hypersensitivity reaction is hypersensitivity pneumonitis?
type III and type IV
what conditions might usual interstitial pneumonitis be seen in?
connective tissue disorders such as sclerodoma and rheumatoid disease
describe the histopathology in usual interstitial pneumonitis.
patchy interstitial chronic inflammation
type II pneumocyte hyperplasia
smooth muscle and vascular proliferation
proliferating fibroblastic foci
what are the treatments for UIP?
Oral anti fibrotic's such as; - pirfenidone - nintedanib surgical transplantation palliative
in which restrictive lung disease is fibrin rich oedema found?
diffuse alveolar damage
describe the histopathology of hypersensitivity pneumonitis.
soft centriacinar epithelial granulomata
interstitial pneumonitis
foamy histiocytes
bronchiolitis obliterans
which restrictive lung disease is primarily around small airways?
hypersensitivity pnuemonitis.
in which restrictive lung disease is bronchiolitis obliterates found?
hypersensitivity pneumonitis
what are the symptoms of usual interstitial pneumonitis?
cough
dyspnoea
what are the signs of usual interstitial pnuemonitis?
clubbing
cyanosis
bi basal crackles
what is the aetiology of UIP?
mostly idiopathic connective tissue diseases i.e. sclerodoma and rheumatoid disease drug reaction post infection industrial exposure i.e. asbestos
what category of people is UIP most commonly found in?
males > 50yrs
what is found on examination with someone with hypersensitivity pneumonitis?
crackles
