CF + Bronchiectasis Flashcards
What are the CT features of bronchiectasis?
1 of:
- bronch:art >1
- lack of tapering
- airways visible <1cm from costal pleura, or touching mediastinal pleura
Ass. signs:
- bronchial wall thickening
- tree-in-bud
- mucus plug
- mosaicism/air-trapping
What are causes of bronchiectasis? (most common at top)
Idiopathic (40-50%)
Post-infection
Immunodeficiency (CVID)
ABPA
Aspiration/GORD
Congenital (Youngs)
RA/other CTD
CF
IBD
A1AT
Inhalation of toxic gas
Yellow nail
Primary cilia dyskinesia
Mounier-Kuhn
What is Youngs syndrome?
Bronchiectasis + sinusitis + azoospermia
Normal cilia function
Pt has recurrent chest infections, sinusitis and ear infections since birth. What is most likely diagnosis?
Primary ciliary dyskinesia
–> Screen with nasal NO then do nasal biopsy then genetics
–> Finger clubbing unlikely
–> Sperm immotile rather than azoospermia
What is Mounier-Kuhn syndrome?
Bronchiectasis + dilated trachea
What investigations should you carry out in patient with bronchiectasis?
CXR, spirometry, sputum (inc AFB), HRCT
Immunoglobulins
Total IgE
Aspergillus IgE (or skin prick) + IgG
Consider Pneumococcal and tetanus Ag
+/-
RF, Serum electrophoresis, A1AT, bronchoscopy, videofluoroscopy (aspiration), pH study (reflux), sweat tests, genetics (PCD/CF), 24hr sputum collection, echo
What is the bronchiectasis severity score?
Bronchiectasis severity score –> predicts mortality and hospitalisation
Mild = 0-4
Mod = 5-8
Sev = 9+ (out of 26)
Inc: age, bmi, FEV, rad sev, pseud/colonised bacteria, mrc score, hosp ad 2 years/exac in last year
FACED score = simple
- F: FEV1
- A: age
- C: colonised
- E: extensiveness (how many lobes affected on radiology)
- D: dyspnoea (MRC score)
What are the most common bacteria to colonise in bronchiectasis?
H.influenza > pseud > staph aureus
H.inf = gram negative rod (amox)
Pseud = gram negative rod (cipro)
Staph = gram positive cocci (fluclox)
How do you treat pseudomonas in bronchiectasis?
First presentation: 2 weeks cipro (high dose 750mg)
Second: IV abx + 3 months nebs
Colonised:
- 1st line: inhaled colistin
- 2nd line: inhaled gent
- 3rd line: azithromycin or erythromycin
Nb. need clear NTM culture prior to starting long-term macrolide
How should bronchiectasis be managed?
STEP 1
Treat underlying cause
Airways clearance +/- PR
Flu & pneumococcal vaccine
Prompt Abx treatment
Self-management plan
STEP 2 (3+ exac)
physio assessment +/- muco-active treatment (hypertonic saline, carbocysteine)
STEP 3 (3+ exacerbations/year)
Inhaled abx or long term macrolide
STEP 4
Both inhaled abx and macrolide
STEP 5
Regular IV abx (every 2-3 months)
Nb. Macrolide = azithromycin. Can use doxycycline if macrolide not tolerated or contraindicated
How should nasal symptoms in bronchiectasis be managed?
Nasal steroids and irrigation
If no improvement, then ENT
Who with bronchiectasis should be managed in secondary care?
Chronic pseud, NTM or MRSA
Declining lung function
Recurrent exac (≥3/year)
Long term abx
Ass with RA, CVID, IBD, PCD, ABPA
Advanced disease
Considering lung tx
What abx should be used for bronchiectasis?
Against specific pathogen if known e.g. colisitin for pseud
Otherwise, long time azithromycin or erythromcyin with inhaled gent as second line. 3rd line doxycycline.
Nb. need clear NTM culture prior to starting long-term macrolide
Nb. Acute strep pneumo- amox or 2nd line doxy
When should PR be offered in patients with bronchiectasis?
MRC ≥2
When should lung surgery be offered in bronchiectasis?
Localised disease with symptoms not controlled by medical management
When should lung tx be considered in bronchiectasis?
<65yrs
FEV1<30 or rapid progression
What CT findings do you tend to see in NTM?
Middle lobe/lingula bronchiectasis
Tree-in-bud
What is the effect of azithromycin in pts with bronchiectasis?
Decreases frequency of infective exacerbations and increases QOL
How do you treat ABPA?
0.5mg/kg pred 2 weeks then taper
Consider itraconazole as steroid sparing
What are the different classes of mutation in CF?
1 - no functional protein
2 - trafficking deficit (F508del)
3 - defective regulation (G551d)
4 - decreased channel conductance
5 - decreased synthesis
6 - decreased stability
What is type 2 CF mutation?
Trafficking deficit (F508del)
1 - no functional protein
2 - trafficking deficit (F508del)
3 - defective regulation (G551d)
4 - decreased channel conductance
5 - decreased synthesis
6 - decreased stability
What is a normal sweat test?
<30
30-60 = indeterminate
>60 = abnormal
How is CF diagnosed?
Symptoms + positive sweat test or genetics
No symptoms (e.g. neonatal screening) + positive sweat test + genetics
What are most common micro in CF?
H.influenza then staph aureus & pseud
Nb. pseud in adults
How do you eradicate pseud in CF?
2 weeks Oral of IV then inhaled
What is treatment for CF?
Airways clearance
Mucolytic - neb DNAse (dornase alfa) +/- hypertonic saline (if inadequate response to DNase) –> only mannitol if can’t use others and FEV1 decline >2% annually
Inhaled abx if chronic colonisation
Prophylactic azithromycin (if decreasing lung funciton or freq exacerbations)
MODULATORS
Potentiator (opens channel) = ivacaftor
Corrector (trafficking and folding) = lumacaftor/tezacaftor
Next gen corrector = elexacaftor
What is ivacaftor and who is eligible?
Potentiator (opens channel)
>4months + group 3 mutation (e.g. G551d, R117H
Others:
Correctors (traffic and fold)
- Lumacaftor (>2yrs + homo F508del)
- Tezacaftor (>6yrs + homo or hetero with another named mutation)
Nexg gen corrector
- Elexacaftor (>12yrs)
–> give + iva + teza for homo F508del or hetero + another minimal function gene mutation
Nb. Kaftrio = ivacaftor + tezacaftor + elexacaftor
What kind of bacteria is Burkholderia?
Gram -ve bacillus
Pt with CF has abdominal pain and palpable mass in RLQ. What is likely diagnosis and how is it managed?
Distal intestinal obstruction syndrome
Manage: oral/iv fluids
Gastrograffin (diatrizoate)
2nd line = macrogols
How should liver be monitored in CF?
Annual LFTs
–> USS if abnormal + consider Ursodeoxycholic acid treatment (stop if LFTs return to normal of USS normal)
What is first line treatment for Moraxella Catarrhalis is bronchiectasis?
Co-Amoxiclav
2nd line: clari, doxy, cipro
