1. RADIOLOGY - CXR Bronchiectasis - vessel ‘crowding’
- loss of vessel markings
- tramline/ring shadows
- cystic lesions/ air-fluid levels
- evidence of TB
Poor:
diagnostic sensitivity
monitoring of progression 3

2. RADIOLOGY - HRCT - bronchial dilatation - bronchial wall thickening
- classification (pathology)
sensitivity (97%) > CXR
chromosomal radiosensitivity
- plain CXR (x 3 days background)
- HRCT: x 30-40
- conventional CT: x 200
? routine baseline
? (a)symptomatic monitoring

3. UNSUSPECTED DISEASE (Clinical v CXR v HRCT)
Bronchiectasis in Hypogammaglobulinaemia - A Computed Tomography assessment. Curtin et al. Clinical Radiology (1991) 44, 82-84
Radiologic Findings of Adult primary Immunodeficiency Disorders. Obregon et al. Chest (1994)106,
Chest High Resolution CT in Adults with Primary Humoral Immundeficiency. Feydy et al. British Journal of Radiology (1996) 69,
Clinical Utility of High-Resolution Pulmonary Computed Tomography in Children with Antibody Deficiency. Manson et al. Pediatric Radiology (1997) 27,
The Value of Computed Tomography in the Diagnosis & Management of Bronchiectasis. Pang et al. Clinical Radiology (1989) 40, 40-44
Review Article: Imaging in Bronchiectasis. Smith et al. British Journal of Radiology (1996) 69, 3

4. RADIOLOGY Kainulainen et al 1999 3 year follow-up 
CVID x 18, XLA x 4
CXR HRCT
Bronchiectasis
3 year follow-up 
Disease progression (5)
Serum IgG
Case No T=0 T=36

5. RADIOLOGY - HRCT RCP Specialty Specific Standards
‘Fit’ patients…….CT scanning should be undertaken in
a minority of patients but usually not more than once a
year or if respiratory function tests or symptoms
deteriorate
JCIA November

6. MANAGEMENT – GENERAL ISSUES
Shared Care (Immunologist/Respiratory Physician) optimal
Bronchodilators (reversible airflow obstruction)
Mucolytics - insufficient evidence to evaluate routine use
(Cochrane Database of Systematic Reviews. 3, 2003)
Physical therapy - insufficient evidence to support or refute usage
Anti-inflammatory agents

8. REPLACEMENT THERAPY
Risk/benefit assessment
IV/Sc routes optimal
 pulmonary infections in XLA/CVID (v untreated)
Optimal dosing/frequency/serum IgG level not established
Tailor route/dose/infusion frequency
Maintain IgG >5g/l
Paediatric target: mid reference range
IgG: >8g/l   infection (v 5g/l, XLA, children) 3
9.4 g/l   infection (v 6.5g/l, XLA/CVID, children/adults) 3
High v standard doses   infections (no. & duration) 2
 days hospitalised
 serum IgG
Insidious disease progression despite ‘adequate’ replacement 3

9. REPLACEMENT THERAPY High dose v low dose: secondary outcome, pulmonary function
Eijkhout et al 2001 (randomised, double-blind, multicentre, crossover, n=43)
High dose (mean trough IgG 9.4 g/l): PEFR 37.3 l/min
Standard dose (mean trough IgG 6.5 g/l): PEFR 11.4 l/min NS
Roifman & Gelfand 1988 (ramdomised, crossover, n=12)
High dose   FVC & FEV p<0.01
Roifman et al 1987 (randomised, crossover, n=12)
Mean FEV1 & FVC high dose phase v low dose phase p<0.01
Bernatowska et al 1987 (two-dose, crossover, non-randomised, n=13)
High dose  Max. expiratory flow & FEV NA

11. ACUTE INFECTION MICROBIOLOGY
Culture & sensitivity routinely in acute setting
Value unclear in chronic situation - confirm original pathogen
- ? emerging resistance
- additional pathogens
ANTIBIOTICS
Effectiveness established in exacerbations (bronchiectasis) 2
Higher doses for longer periods
Local treatment protocols

12. ANTIBIOTIC PROPHYLAXIS
Chronic bronchitis - no place in routine treatment
(Cochrane Database of Systematic Reviews. 3, 2003)
Cystic fibrosis benefits - principally staphylococci
- infancy  3/6 years
- ? older children/adults
- ? > 3years treatment
(The Cochrane Library, Oxford. 2, 2003)
Bronchiectasis - limited meta-analysis (6 RCTs)
- marginal benefit / cautious support
(Evans et al. Thorax 2001)

13. ANTIBIOTIC PROPHYLAXIS
No robust data v placebo
No substantial data v (or additional to) IVIg/SCIg (Silk et al. 1990)
? Single intervention in mild antibody deficiency
- not in more severe phenotypes / tissue damage
Papworth protocol: consider if: > 3 exacerbations / year
radiological / PFT deterioration
? Eradication/clean-up therapy prior to prophylaxis
- no clear evidence of benefit in antibody deficiency + structural lung damage
Development of local protocols for management of infections
(esp. with Primary Care) and initiating prophylaxis

14. ANTIBIOTIC PROPHYLAXIS
(Heelan et al., ESID 2002)

16. SURGERY Diagnostic delay > 2 years:  need for surgical procedures
Adequate treatment:  lobectomy/pneumonectomy by 95%
(UK PAD Audit )
Important treatment option with favourable outcomes
especially in focal bronchiectasis
(Cohen et al 1994, Mansharamani & Koziel 2003)

17. QUESTIONS / ISSUES HRCT in routine screening & monitoring
Radiological changes a primary therapeutic target
- Does HRCT modify our current assumptions about criteria for adequate
treatment of antibody deficiency disorders?
Correct level of Ig treatment
- arbitrary target serum level (evidence) or individualised (clinical + HRCT factors)
- single intervention universally applicable in all patients (probably not)
- higher doses: expense, complications, limited commodity
Roles of: antibiotics
anti-inflammatory agents
bronchodilators
aids to airway clearance
Role of co-factors (e.g. 1AT)
Selective IgA deficiency

19. PIN GUIDELINES Identify need for focused clinical research
Encourage debate and discussion
Reflect uncertainties in the field
Proscriptive as necessary, flexible where possible