CF, IPF, Sarcoid, OSA

Question 1

What is IPF?

Answer 1

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

Question 2

What are the risk factors for IPF?

Answer 2

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

Question 3

What are the characteristic features of IPF?

Answer 3

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

Question 4

What are the causes of upper zone PF?

Answer 4

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

Question 5

What are the causes of lower zone PF?

Answer 5

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

Question 6

How does IPF present?

Answer 6

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

Question 7

How is PF investigated?

Answer 7

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

Question 8

How is PF managed?

Answer 8

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

Question 9

How does OSA occur?

Answer 9

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

Question 10

Who typically gets OSA?

Answer 10

Obese

Middle-aged men

Question 11

What are the risk factors for OSA?

Answer 11

Marfan's
Acromegaly
Hypothyroid
Amyloidosis
Large tonsils

Question 12

What are the Sx of OSA?

Answer 12

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

Question 13

How is OSA investigated?

Answer 13

Polysomnography= DIAGNOSTIC

Pulse Oximetry

Question 14

What is classed as severe OSA?

Answer 14

> 15 episodes in 1 hour sleep

Question 15

How is OSA managed?

Answer 15

MILD: ↓weight, ↓OH-, stop smoking

MOD-SEVERE: CPAP

Question 16

How does cystic fibrosis affect the lungs?

Answer 16

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

Question 17

How does cystic fibrosis affect the pancreas?

Answer 17

Dehydration of pancreatic secretion

Leads to stagnation in pancreatic ducts

Question 18

What is the cause of CF?

Answer 18

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

Question 19

How does cystic fibrosis affect the biliary tract?

Answer 19

↓ion transport
Means ↓water into lumen
Bile becomes concentrated

Question 20

What are the signs of CF in a neonate?

Answer 20

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

Question 21

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

Answer 21

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

Question 22

How is CF investigated?

Answer 22

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

Question 23

How is CF managed?

Answer 23

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

Question 24

What are the complications of CF?

Answer 24

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

Question 25

What is sarcoidosis?

Answer 25

Multisystem granulomatous disorder characterised by non-caveating granulomas

Question 26

What are the risk factors for sarcoidosis?

Answer 26

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

Question 27

How does acute sarcoidosis present?

Answer 27

Often resolves spontaneously Erythema Nodosum Polyarthralgia

Question 28

What are the clinical features of sarcoidosis?

Answer 28

``` 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 ```

Question 29

How is sarcoidosis investigated?

Answer 29

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

Question 30

How is sarcoidosis managed?

Answer 30

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

Question 31

What are the complications of sarcoidosis?

Answer 31

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

Question 32

What are the different stages of sarcoidosis seen on CXR?

Answer 32

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

Question 33

What is atelectasis?

Answer 33

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