Chronic Respiratory

Question 1

Conditions causing fibrosis in upper zones:

Answer 1

C - coal worker's pneumoconiosis
H - histiocytosis/hypersensitivity pneumonitis (extrinsic allergic alveolitis)
A - ankylosing spondylitis
R - radiation
T - TB
S - silicosis/sarcoidosis

Question 2

Conditions causing fibrosis in lower zones:

Answer 2

• IPF - restrictive (reduced gas transfer factor)
• connective tissue disorders
• drug induced: amiodarone, bleomycin, methotrexate

Question 3

Cystic fibrosis

Answer 3

• autosomal recessive
• increased viscosity of secretions (lungs and pancreas)
• CFTR conductance regulator gene - cAMP regulated chloride channel
• 80% delta F508 chromosome 7 long arm

Question 4

Most common organisms affecting cystic fibrosis:

Answer 4

• pseudomonas aeruginosa
• staph aureus
• burkholderia cepacia
• aspergillus

Question 5

Features of cystic fibrosis:

Answer 5

• neonatal: meconium ileus, prolonged jaundice
• recurrent chest infections
• malabsorption: steatorrhoea
• short stature, DM, delayed puberty, rectal prolapse (bulky stools), nasal polyps, male infertility, female subfertility

Question 6

Diagnosis of cystic fibrosis:

Answer 6

sweat test: hyperchloraemic, >60mEq/L

Question 7

Reasons for false positive sweat test in CF:

Answer 7

• malnutrition
• adrenal insufficiency
• glycogen storage disease
• nephrogenic diabetes insipidus
• hypothyroidism, hypoparathyroidism
• G6PD
• ectodermal dysplasia

Question 8

Reasons for false negative sweat test in CF:

Answer 8

• skin oedema

- hypoalbuminaemia/hypoproteinuria secondary to pancreatic excocrine insufficiency

Question 9

Management of CF:

Answer 9

• twice daily chest physiotherapy and postural drainage
• high calorie and fat intake
• vitamin supplements
• pancreatic enzyme supplements with meals
• lung transplant (contra burkholderia cepacia)
• lumacaftor/ivacaftor - homozygous deltaF508 mutation

Question 10

alpha 1 AT def

Answer 10

• young, non smokers, panacinar emphysema (lower lobes)
• chromosome 14, autosomal recessive and co-dominant
• liver: cirrhosis and HCC adults, cholestasis in children
• obstructive spirometry
• management: no smoking, bronchodilators, physiotherapy, IV A1AT concentrate, lung volume reduction, lung transplant

Question 11

Atelectasis

Answer 11

• common postoperative complication - basal alveolar collapse
• airway obstructed by bronchial secretions
• dyspnoea, hypoxaemia, 72 hrs postop
• manage: upright position, breathing exercises

Question 12

Bronchiectasis causes:

Answer 12

• permanent dilation of airways secondary to chronic infection or inflammation
• post infective: TB, measles, pertussis, pneumonia
• CF
• bronchial obstruction e.g. cancer/foreign body
• immune def - selective IgA, hypogammaglobulinaemia
• ABPA
• ciliary dyskinetic: Kartagener’s, Young’s
• yellow nail syndrome

Question 13

Most common organism affecting bronchiectasis:

Answer 13

haemophilus influenzae
(pseudomonas aeruginosa, klebsiella, strep pneumonia)

Question 14

Common imaging findings in bronchiectasis:

Answer 14

• tram track and signet ring signs

- parallel line shadows

Question 15

Management of bronchiectasis:

Answer 15

• physical training
• postural drainage
• antibiotics for exacerbations and long term
• bronchodilators
• immunisation
• surgery if localised

Question 16

Causes of bilateral hilar lymphadenopathy:

Answer 16

• most common: sarcoidosis and TB
• lymphoma/malignancy
• pneumoconiosis e.g. berylliosis
• funghi e.g. histoplasmosis, coccidioidomycosis

Question 17

Cardiac causes of clubbing:

Answer 17

• cyanotic congenital heart disease
• bacterial endocarditis
• atrial myxoma

Question 18

Respiratory causes of clubbing:

Answer 18

• lung cancer
• pyrogenic conditions: CF, bronchiectasis, abscess, empyema
• TB
• asbestosis, mesothelioma
• fibrosing alveolitis

Question 19

Other causes of clubbing:

Answer 19

• Crohn’s
• cirrhosis, PBC
• Grave’s
• Whipple’s

Question 20

Features of eosinophilic granulomatosis with polyangiitis:

Answer 20

• asthma
• blood eosinophilia
• paranasal sinusitis
• mononeuritis multiplex
• pANCA

Question 21

Features of granulomatosis with polyangiitis:

Answer 21

• affects upper and lower RT and kidneys
• epistaxis, sinusitis, nasal crusting
• dyspnoea, haemoptysis
• rapidly progressive GN
• saddle-shaped nose
• vasculitic rash, eye involvement, cranial nerve lesion

Question 22

Features of microscopic polyangiitis:

Answer 22

• renal impairment: increased creatinine, haematuria, proteinuria
• fever
• lethargy, myalgia, weight loss
• palpable purpuric rash
• cough, dyspnoea, haemoptysis
• mononeuritis multiplex

Question 23

Pneumoconiosis presentation:

Answer 23

simple pneumo:

• most common
• often asymptomatic
• increased risk of COPD
• may lead to progressive massive fibrosis

progressive massive fibrosis:

• round fibrotic masses
• upper lobes
• breathlessness on exertion and cough
• black sputum
• obstructive/restrictive mixed

Question 24

Management of pneumoconiosis:

Answer 24

• avoid exposure including smoking

- manage bronchitis

Question 25

Staging of pneumoconiosis:

Answer 25

- I: some opacities but lung markings visible - II: large number opacities but markings visible - III: no markings

Question 26

Causes of obstructive sleep apnoea:

Answer 26

- obesity - macroglossia: acromegaly, hypothyroid, amyloidosis - large tonsils - Marfan's

Question 27

Consequences of OSA:

Answer 27

- daytime somnolence - compensated respiratory acidosis - hypertension

Question 28

Management of OSA:

Answer 28

- weight loss - CPAP - intra oral devices

Question 29

Sarcoidosis incl features:

Answer 29

- multisystem disorder - non-casaeting granulomas - young adults and african descent - acute: erythema nodosum, bilateral hilarity lymphadenopathy, swinging fever, polyarthralgia - insidious: dyspnoea, non-productive cough, malaise, weight loss - skin: lupus pernio - hypercalcaemia - macrophages in granulomas increase conversion vit D to active form

Question 30

Sarcoidosis associated syndromes:

Answer 30

- Lofgren's: bilateral hilar lymphadenopathy, erythema nodosum, fever and polyarthralgia - Mikulicz: enlarged parotid, lacrimal glands due to sarcoidosis, TB or lymphoma - Heerford's: uveoparotid fever - enlarged parotid, fever and uveitis secondary to sarcoidosis

Question 31

Management of sarcoidosis:

Answer 31

indication for steroids: - CXR stage 2 or 3 with symptoms - hypercalcaemia - eye, heart or neuro involvement

Question 32

Silicosis:

Answer 32

- fibrosing lung disease - inhalation of fine particles of crystalline silicon dioxide - risk factor for developing TB - egg shell calcification of hilar lymph nodes - mining, slate works, foundries, potteries

Question 33

How can you investigate A1AD prenatally?

Answer 33

amniocentesis

Question 34

Assessment of LTOT requirement in COPD patients:

Answer 34

- very sever airflow obstruction (FEV1<30%) - cyanosis - polycythaemia - peripheral oedema - raised JVP - O2 sats <=92% on room air - assessment done by ABG on 2 occasions at least 3 weeks apart in stable COPD

Question 35

What criteria in COPD qualifies them for LTOT:

Answer 35

- pO2 <7.3kPa - or pO2 7.3-8kPa with one of: secondary polycythaemia, peripheral oedema or pulmonary hypertension - must not be smoking

Question 36

Asbestos:

Answer 36

- causes variety of disease - pleural plaques: benign, do not undergo malignant change, most common form, after latent period of 20-40years - pleural thickening - asbestosis: latent period 15-30 years, lower lobe fibrosis, SOB and reduced exercise tolerance - mesothelioma: crocidolite asbestos most dangerous form, palliative chemo, poor prognosis - lung cancer

Question 37

What is the investigation of choice in IPF?

Answer 37

high resolution CT

Question 38

What ratio indicates exudate as opposed to transudate?

Answer 38

>0.5

Question 39

What can high levels of ACE indicate?

Answer 39

sarcoidosis