Respiratory Disease Flashcards

Question

Pirfenidone

Answer 1

MOA: Inhibits TGF beta and fibroblast proliferation. Slows rate of FVC decline S/E - Rash - Nausea - Diarrhoea

Answer 2

GGO Reticular opacity, but diffuse and can have sub-pleural sparing. Traction Bronchiectasis NO honeycombing.

Answer 3

CTD HIV Drugs (amiodorone, MTX, flecainide, nitrofurantoin) Hypersensitivity pneumonitis Idiopathic NSIP

Answer 4

Considered treatable with corticosteroids.

Answer 5

Not a single disease. Need a history of exposure + clinical features. HRCT features: - Centrilobular nodules or GGO - mosaic hyper-attenulation

Answer 6

Mepo (IL 5) 4 weekly injection and Benza (IL 5 R) 8 weekly injections. Major benefit in eosinophilic asthma Indicated in those with moderate to severe asthma and serum eosinophil counts 150-300cells/ml. IL5 is the major cytokine for growth, differentiation, recruitment, activation and survival of eosinophils.

Answer 7

Effective airway clearance is the cornerstone: - Physiotherapy - Inhaled saline and mannitol Macrolides - reduce exacerbation frequency What not to use: - Inhaled antibiotics have no evidence. - Recombinant human deoxyribonuclease - only used in Cystic fibrosis, trials showed harm in non-cystic fibrosis. - Long term corticosteroids or bronchodilators (only if co-comitant COPD or asthma)

Answer 8

CFTR = CF transmembrane conductance regulator del508 causes misfolding and retention of protein in ER. 50% homozygous for del508 - LUMACAFTOR partly rescues CFTR transport and therefore is effective in this group. 40% compound heterozygous for del508 and another mutation 10% have Gly511Asp mutation which results in abnormal gating of a normally inserted protein (Arg117His is similar) - These respond to IVACAFTOR which is a CFTR potentiator.

Answer 9

Bronchoarterial ratio of >1 Lack of airway tapering Airway visible within 1cm of pleura

Answer 10

Airway secretion management: - Inhaled DNase alpha - hypertonic saline - Positive expiratory pressure therapy Anti-inflammatory therapy: - Macrolide antibiotics (ensure no NTM, ECG before starting)

Answer 11

Burkholderia cepacia complex: - Chronic infection associated with faster decline in lung function and short survival - Multi-drug resistant - Lung transplant with B cepacia associated with poor outcome NTM: - Lung transplant with NTM not associated with worse outcome - M Abscessus associated with poor outcome and transplant complications - Only treat NTM infections if associated with evidence of clinical symptoms, nodular infiltrates or cavitating lesions.

Answer 12

Normal Class 1 - no functional CFTR produced . Class 2 - CFTR is misfolded and does not reach cell surface (d508) Class 3 - CFTR reaches the surface but does not open properly. Class 4 - CFTR does not conduct ions properly (R117H) Class 5 - Normal CFTR but created in insufficient quantities. Class 6 - reduced half life of CFTR

Answer 13

CFTR Potentiator - G551D (Gly551Asp) - CFTR does not open correctly (gating defect), Ivacaftor increases Cl transport - Not effective for del508

Answer 14

CFTR Corrector Partially corrects the mis-folding and therefore improved transport to cell surface. Given in combination with IVACAFTOR which improved ion transport once at cell surface. LUMACAFTOR/IVACAFTOR is beneficial for del508 HOMOZYGOTES, but has is not of clinical benefit to del508 heterozygotes.

Answer 15

Age >65 Significantly limited functional status Colonisation with Burkolderia cepacia (cenocepacia) or Mycobacterium abscessus Transplant survival outcomes: - 1 year 88% - 5 year 67% - 10 year 50%

Answer 16

Tezacaftor + Ivacafotr was shown to be effective in both d508 heterozygotes and homozygotes.

Answer 17

Now first line for del508 homozygotes and heterozygotes.

Answer 18

Complex hyper-sensitivity reaction to colonisation with aspergillus fumigtatis Occurs exclusively in those with asthma or CF

Answer 19

1 - must have predisposing condition (asthma or CF) 2 - Must have both of: - elevated igE levels - Positive skin prick test or elevated IgE to Aspergillus fumigatus 3 - at least 2 of the following: - Positive aspergiluus precipitans or elevated igG to A fumigatus - radiology consistent with ABPA - Total eosinophil count of > 0.5

Answer 20

Proximal cylindrical bronchiectasis Mucus pluggins Tree in bud opacity Atelectasis GGO Mosaic attenuation

Answer 21

Doubling of IgE + new radiological infiltrates: Treat with: - Prednisolone 0.5mg/kg for 2 weeks and then wean over 2 months. - Itraconazole for 16 weeks Be aware of -azole CYP3A4 inhibition and Cushings syndrome

Answer 22

Omalizumab Mepolizumab and Benralizumab Dupilumab

Answer 23

Sleepy (43%) - Increased risk of Cardiovascular disease - Best response to PAP therapy Minimally symptomatic (25%) - Some response to PAP Disturbed sleep (33%) - Less response to PAP

Answer 24

Mild OSA - AHI 5-15 Moderate OSA - AHI 15-30 Severe OSA - AHI >30 Obstructive Apnoea = 90% reduction in respiratory nasal flow for 10 seconds WITH respiratory effort (No flow but ongoing respiratory effort = obstructive) Hypopnoea = 30% reduction in nasal flow for 10 seconds with a 4% desat. Central Apnoea = no effort.

Answer 25

PAP therapy Mandibular advancement therapy - only mild/moderate, or if unable to tolerate PAP. Sleep position modification Surgery Weight Loss Smoking cessation. Proven Benefits: - Improved symptoms - Hypertension (reduction of 1.7mmHg 24 hour mean BP) - Reduction in CVD and mortality - Improved insulin resistance - Improved QOL, reduced motor vehicle accidents.

Answer 26

Non-pharmacological - Ensure ferritin is >100microg/L Pharmacological (dopamine agonists) - Pramipexole - Ropinirole - Rotigotine Patch But augmentation is common, worsening RLS with increasing doses of medications. Impulse control disorders - 9 months after therapy. Second line: Pregabalin or Pregabaline

Answer 27

Persistence of pulmonary hypertension secondary to pulmonary emboli despite effective anti-coagulation for 3 months. Important to perform VQ scan and TTE on patients with ongoing dyspnoea secondary to PE (incidence of 3.4% after acute PE) VQ preferred imaging modality for diagnosis, but CTPA is preferred to characterise lesion and assess if suitable for surgery.

Answer 28

First line in Pulmonary endarterectomy (PEA), however 40% of patients will not be suitable. If unable to have PEA, medical therapy: 1 - Riociguat 2 - Bosentan 3 - Sildenafil

Answer 29

BMPR mutation

Answer 30

Combination therapy with: - Ambrisentan (Selective Endothelin A R antagonist) - Tadalafil (PDE5 inhibitor)

Answer 31

Over 50 specific of legionella in Aus, but 2 most common pathogenic are: - longbeachae (commonly found in potting mix) - pneumophila Gram NEGATIVE, strict growth requirements that will not grow in usual media. Intracellular pathogens, will respond well to macrolide, tetracyclines, and quinolones. They are ubiquitous and found in water and wet areas. No human to human transmission. Legionnaires Disease: - Atypical pneumonia - Gastrointestinal symptoms - Neurological Symptoms - Hyponatraemia - elevated LFTs and CK The urinary legionella antigen test is the most rapid and sensitive test available but will only test for L pnuemophila serotype 1 and may only be positive after day 5 of the illness.

Answer 32

Type 1 Narcolepsy: - Loss of the Orexin secreting neurons in the hypothalamas - Orexin is responsible for promoting wakefulness during daytime hours and surpresses REM sleep - thought to be Autoimmune, associated with HLA DQB1*06

Answer 33

Sleep is still refreshing Sleep paralysis Hyponogogic and Hypnopompic hallucinations Cataplexy (sudden loss of muscle tone induced by emotional trigger) - In type 1 Narcolepsy

Answer 34

Sleep Studies: - Early REM transition and disrupted sleep cycle Multiple sleep latency test: - Mean sleep latency of < 8 minutes - 2 or more sleep onset REM periods (SOREMP) Management: - Behavioural - Psychostimulants may be needed

Answer 35

Sensitive but not specific for exudate, positive if one or more of: 1 - Pleural fluid protein to serum protein >0.5 2 - PF LDH to Serum LDH > 0.6 3 - Pleural fluid LDH more than 2/3 ULN for serum LDH (LDH > 200)

Answer 36

Asbestosis Benign asbestos pleural effusion Pleural Plaques - these are benign, and are NOT a premalignant condition, they do not require any follow-up. They may grow over time. Rounded Atelectasis Mesothelioma Non-Small cell lung Ca

Answer 37

1 - T2 high asthma 2 -Pulmonary strongyloidiasis 3 - ABPA 4 - Chronic eosinophilic pneumonia 5 - EGPA Note Hypersensitivity Pneumonitis is a type 3 hypersensitivity reaction that does not cause eosinophilia but is more associated with restrictive deficits and reduced gas transfer.

Answer 38

T1 - IgE mediated, atopy T2 - Cytotoxic/IgG or IgM mediated. Direct binding of antibody to cellular antigen causes destruction of cell via ADCC, NK cell or Macrophage activation. T3 - Immune complex mediated T4 - Delayed T cell mediated

Answer 39

T1 - IgE mediated, atopy T2 - Cytotoxic/IgG or IgM mediated. Direct binding of antibody to cellular antigen causes destruction of cell via ADCC, NK cell or Macrophage activation. T3 - Immune complex mediated T4 - Delayed T cell mediated

Answer 40

Trial of: - Bilateral Hilar Lymphadenopathy - Erythema Nodosum - Arthritis (typically ankles) LS is an acute form of Sarcoidosis, it may also present with malaise and fevers. It has a favourable prognosis with usually complete resolution in 6 months.

Answer 41

Previously called BOOP. Alveolar injury caused by unknown insult --> formation of organised granulation tissue within the alveoli lumen and bronchioles leading to respiratory failure. Presentation: Weeks of fever, malaise, cough. Often failed treatment for presumed bacterial pneumonia. Imaging: Asymmetrical, bilateral patchy peripherally located consolidation and GGO. COP is a diagnosis of exclusion.. BAL to exclude infection, haemorrhage, malignancy. Biopsy often required to make diagnosis. Treatment 1mg/kg Prednisolone weaned over 6-12 months. Good prognosis if treated.

Answer 42

PaC02 = (1.5 x HCO3) + 8 (+/-2) For example if HCO3 is 16 PaC02 should be 24+8 = 32 If HC03 is 10 PaC02 should be 10+5+8 = 23

Answer 43

Increase in HC03 should be = DeltaPaC02 / 10 Normal PaC02 is 40 Normal Bicarb is 24 PaC02 is 60 Increase in HC03 should be 2 Therefore HC03 should be 24+2 = 26

Answer 44

Increase in HC03 should be 4 x delta PCO2/10 PC02 is 60 (chronic) HC03 increase should be 8 HC03 should be 24+ 8 = 32

Answer 45

Decrease in HC03 should be 2 x delta PCO2/10

Answer 46

Decrease in HC03 should be 5 x delta PCO2/10

Answer 47

PC02 = 40 + (0.7 x Delta HCO3)

Answer 48

Delta ratio should be calculated in a metabolic acidosis to determine if mixed. Delta anion gap = change in anion gap / change in HC03 <1 - likely mixed HAGMA/NAGMA 1-2 normal pattern for HAGMA >2 likely mixed chronic metabolic alkalosis or chronic respiratory acidosis (which would increase the HC03).

Answer 49

Primary (young, no lung disease) - If <2cm and asymptomatic - consider discharge - If >2cm or symptomatic, attempt needle aspiration, if able to reduce to <2cm and improve symptoms then consider discharge with OPD in 2-4 weeks. Secondary (Age >50, or lung disease) - If >2cm or breathless then insert size 8-14Fr chest drain and admit - If 1-2cm and not breathless then attempt needle aspiration - If <1cm then admit for high flow oxygen and observation 24 hours NOTE: - Any bilateral or unstable gets chest drain - measure the inter-pleural distance at the level of the hilum, large pneumothorax is defined as >2cm at hilum or > 3cm at apex.