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Yr 5 Respiratory > CF, IPF, Sarcoid, OSA > Flashcards

CF, IPF, Sarcoid, OSA Flashcards

1
Q

What is IPF?

A

A diffuse parenchymal lung disease with no known cause resulting in chronic, progressive fibrosis of lung interstitium leading to loss of elasticity

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2
Q

What are the risk factors for IPF?

A 
50-70yo
Male
Smoking
Occupational (dust)
Environmental (pigeon breeders)
CT disease (RA, SLE, Sjorgrens)
Recurrent viral infection
GORD
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3
Q

What are the characteristic features of IPF?

A
  • Dyspnoea
  • Diffuse infiltrates on CXR
  • Inflammation +/- fibrosis on biopsy
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4
Q

What are the causes of upper zone PF?

A 
CARTEx:
C: Coal
A: Ankylosing Spondylitis
R: RT
T: TB
Ex: Extrinsic allergic alveolitis
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5
Q

What are the causes of lower zone PF?

A 
CAID:
C: CT disorders
A: Asbestos
I: Idiopathic PF
D: Drugs (Amiodarone, MTX, Cyclophosphamide, Nitrofuran)
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6
Q

How does IPF present?

A 
Dry cough
Dyspnoea on exertion- PROGRESSIVE
Flu like illness
OSA
Cyanosis
Clubbing
Fine end-inspiratory crackles
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7
Q

How is PF investigated?

A

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

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8
Q

How is PF managed?

A

Pulmonary rehab
Supportive care: O2, Opiates, lung transplant
Pirfenidone (DMARD)- anti-fibrotic
Palliative care: LE = 2-4yrs

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9
Q

How does OSA occur?

A

Intermittent closure/collapse of pharyngeal airway
Apnoeic episodes during sleep
Terminated by partial arousal.

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10
Q

Who typically gets OSA?

A

Obese

Middle-aged men

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11
Q

What are the risk factors for OSA?

A 
Marfan's
Acromegaly
Hypothyroid
Amyloidosis
Large tonsils
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12
Q

What are the Sx of OSA?

A 
Loud snoring
Daytime somnolence
Poor sleep quality
Morning headache
↓ libido
↓ cognitive performance
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13
Q

How is OSA investigated?

A

Polysomnography= DIAGNOSTIC

Pulse Oximetry

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14
Q

What is classed as severe OSA?

A

> 15 episodes in 1 hour sleep

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15
Q

How is OSA managed?

A

MILD: ↓weight, ↓OH-, stop smoking

MOD-SEVERE: CPAP

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16
Q

How does cystic fibrosis affect the lungs?

A

↓Cl-/Na+ in lungs
↑mucous thickening = airway dehydration
↓mucociliary clearance = ↑bacterial infection + ↑nucleophilic response = bronchiectasis

17
Q

How does cystic fibrosis affect the pancreas?

A

Dehydration of pancreatic secretion

Leads to stagnation in pancreatic ducts

18
Q

What is the cause of CF?

A

Autosomal recessive, mutation CFTR gene (chr.7) – controls Cl- transp
∆F508 (Caucasian) mutation = ATP-responsive chloride channel

19
Q

How does cystic fibrosis affect the biliary tract?

A

↓ion transport
Means ↓water into lumen
Bile becomes concentrated

20
Q

What are the signs of CF in a neonate?

A 
Failure to pass meconium in 1st 24hrs (Meconium ileus)
FTT (Malabsorption)
Prolonged jaundice
Steatorrhoea
Recurrent chest infections
21
Q

What are the signs of CF in children & young adults?

A 
Bronchiectasis
Recurrent LRTI
Pneumothorax
Diabetes
Gall stones
Nasal polyps
Rectal prolapse
Cyanosis
Clubbing
22
Q

How is CF investigated?

A

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

23
Q

How is CF managed?

A 
Supportive: Chest physio, Vitamin ADEK, high calorie diet
MEDS: 
Bronchodilator = SABA + LABA
Pancreatic enzyme
replacement= Creon
Prophylactic Abx = Fluclox
Annual flu vaccine
24
Q

What are the complications of CF?

A 
Distal intestinal obstruction syndrome
Infertility
Osteoporosis
Arthritis
Vasculitis
Sinusitis
Allergic bronchopulmonary aspergillosis infection
25
Q

What is sarcoidosis?

A

Multisystem granulomatous disorder characterised by non-caveating granulomas

26
Q

What are the risk factors for sarcoidosis?

A

20-40yo
Female
Afro-Carribbean
HLA-DRB1 & DQB1

27
Q

How does acute sarcoidosis present?

A

Often resolves spontaneously
Erythema Nodosum
Polyarthralgia

28
Q

What are the clinical features of sarcoidosis?

A 
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
29
Q

How is sarcoidosis investigated?

A

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

30
Q

How is sarcoidosis managed?

A

ACUTE = Bed rest + NSAIDs
Steroids = Prednisolone PO 4-6w ↓dose over 1yr
IV Methylprednisolone/ImmunoS = Methotrexate/Ciclosporin

31
Q

What are the complications of sarcoidosis?

A

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

32
Q

What are the different stages of sarcoidosis seen on CXR?

A

Stage 1 = BHL
Stage 2 = BHL & peripheral pulmonary infiltrates
Stage 3 = peripheral pulmonary infiltrates ALONE
Stage 4 = Fibrosis, Bulla (honey-comb), Pleural involvement

33
Q

What is atelectasis?

A

Common post-op complication
Basal alveoli collapse complete/partial collapse of lung lobe
Leads to resp desaturation

Yr 5 Respiratory (6 decks)

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