CF, IPF, Sarcoid, OSA Flashcards
What is IPF?
A diffuse parenchymal lung disease with no known cause resulting in chronic, progressive fibrosis of lung interstitium leading to loss of elasticity
What are the risk factors for IPF?
50-70yo Male Smoking Occupational (dust) Environmental (pigeon breeders) CT disease (RA, SLE, Sjorgrens) Recurrent viral infection GORD
What are the characteristic features of IPF?
- Dyspnoea
- Diffuse infiltrates on CXR
- Inflammation +/- fibrosis on biopsy
What are the causes of upper zone PF?
CARTEx: C: Coal A: Ankylosing Spondylitis R: RT T: TB Ex: Extrinsic allergic alveolitis
What are the causes of lower zone PF?
CAID: C: CT disorders A: Asbestos I: Idiopathic PF D: Drugs (Amiodarone, MTX, Cyclophosphamide, Nitrofuran)
How does IPF present?
Dry cough Dyspnoea on exertion- PROGRESSIVE Flu like illness OSA Cyanosis Clubbing Fine end-inspiratory crackles
How is PF investigated?
1) Bloods: FBC, CRP, ABG, AutoAb
2) CXR: ↓Lung vol, honeycombing, ground glass
3) HRCT: DIAGNOSTIC for interstitial disease
4) Spirometry: RESTRICTIVE, ↓↓FVC, ↓Transfer factor
5) Bronchoalveolar lavage: Alveolitis diagnosis
6) Lung biopsy
How is PF managed?
Pulmonary rehab
Supportive care: O2, Opiates, lung transplant
Pirfenidone (DMARD)- anti-fibrotic
Palliative care: LE = 2-4yrs
How does OSA occur?
Intermittent closure/collapse of pharyngeal airway
Apnoeic episodes during sleep
Terminated by partial arousal.
Who typically gets OSA?
Obese
Middle-aged men
What are the risk factors for OSA?
Marfan's Acromegaly Hypothyroid Amyloidosis Large tonsils
What are the Sx of OSA?
Loud snoring Daytime somnolence Poor sleep quality Morning headache ↓ libido ↓ cognitive performance
How is OSA investigated?
Polysomnography= DIAGNOSTIC
Pulse Oximetry
What is classed as severe OSA?
> 15 episodes in 1 hour sleep
How is OSA managed?
MILD: ↓weight, ↓OH-, stop smoking
MOD-SEVERE: CPAP
How does cystic fibrosis affect the lungs?
↓Cl-/Na+ in lungs
↑mucous thickening = airway dehydration
↓mucociliary clearance = ↑bacterial infection + ↑nucleophilic response = bronchiectasis
How does cystic fibrosis affect the pancreas?
Dehydration of pancreatic secretion
Leads to stagnation in pancreatic ducts
What is the cause of CF?
Autosomal recessive, mutation CFTR gene (chr.7) – controls Cl- transp
∆F508 (Caucasian) mutation = ATP-responsive chloride channel
How does cystic fibrosis affect the biliary tract?
↓ion transport
Means ↓water into lumen
Bile becomes concentrated
What are the signs of CF in a neonate?
Failure to pass meconium in 1st 24hrs (Meconium ileus) FTT (Malabsorption) Prolonged jaundice Steatorrhoea Recurrent chest infections
What are the signs of CF in children & young adults?
Bronchiectasis Recurrent LRTI Pneumothorax Diabetes Gall stones Nasal polyps Rectal prolapse Cyanosis Clubbing
How is CF investigated?
Screening: Guthrie heel prick test at 5d old
SWEAT TEST = GOLD STANDARD
Genetics: FISH for ∆F508
CXR/CT
Bloods: FBC, U&E, LFT, Clotting, Vitamin ADEK
Spirometry: OBSTRUCTIVE
How is CF managed?
Supportive: Chest physio, Vitamin ADEK, high calorie diet MEDS: Bronchodilator = SABA + LABA Pancreatic enzyme replacement= Creon Prophylactic Abx = Fluclox Annual flu vaccine
What are the complications of CF?
Distal intestinal obstruction syndrome Infertility Osteoporosis Arthritis Vasculitis Sinusitis Allergic bronchopulmonary aspergillosis infection
