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
What is sarcoidosis?
Multisystem granulomatous disorder characterised by non-caveating granulomas
What are the risk factors for sarcoidosis?
20-40yo
Female
Afro-Carribbean
HLA-DRB1 & DQB1
How does acute sarcoidosis present?
Often resolves spontaneously
Erythema Nodosum
Polyarthralgia
What are the clinical features of sarcoidosis?
Constitutional: Fever, malaise, night sweats Lymphadenopathy GI: Hepato/splenomegaly Eye: uveitis, conjunctivitis Renal: Renal stones Neuro: Meningitis, Bell's palsy Bone: Cysts in terminal phalanges Heart: Arrhythmia, cardiomyopathy Skin: LUPUS PERNIO RASH
How is sarcoidosis investigated?
1) Bloods: ESR, ↑LFTs,↑Ca2+, ↑Ig, ↑↑ACE
2) 24hr urine: ↑↑Ca2+
3) Tuberculin test: 1/3rd +ve
4) CXR
5) Tissue biopsy = DIAGNOSTIC ()
6) ECG: BBB, arrhythmia due to ↑Ca2+
7) Bone X-ray: Punched out lesions
How is sarcoidosis managed?
ACUTE = Bed rest + NSAIDs
Steroids = Prednisolone PO 4-6w ↓dose over 1yr
IV Methylprednisolone/ImmunoS = Methotrexate/Ciclosporin
What are the complications of sarcoidosis?
Neurosarcoidosis: ∆ CT/MRI
Lofgren’s syndrome: Acute disease w/BHL, erythema nodosum, fever, polyarthralgia
Mikulicz syndrome: Enlargement of parotid/lacrimal
Heerfordt’s syndrome: Parotid enlargement, fever, uveitis
What are the different stages of sarcoidosis seen on CXR?
Stage 1 = BHL
Stage 2 = BHL & peripheral pulmonary infiltrates
Stage 3 = peripheral pulmonary infiltrates ALONE
Stage 4 = Fibrosis, Bulla (honey-comb), Pleural involvement
What is atelectasis?
Common post-op complication
Basal alveoli collapse complete/partial collapse of lung lobe
Leads to resp desaturation
