RADIOLOGY CXR

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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
• 
  

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RADIOLOGY - HRCT

• - bronchial dilatation
• - bronchial wall thickening
• - classification (pathology)
• sensitivity (97) gt CXR 3
• chromosomal radiosensitivity
• - plain CXR (x 3 days background)
• - HRCT x 30-40
• - conventional CT x 200
• ? routine baseline
• ? (a)symptomatic monitoring

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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, 490-495
• Chest High Resolution CT in Adults with Primary
  Humoral Immundeficiency. Feydy et al. British
  Journal of Radiology (1996) 69, 1108-1116
• Clinical Utility of High-Resolution Pulmonary
  Computed Tomography in Children with Antibody
  Deficiency. Manson et al. Pediatric Radiology
  (1997) 27, 794-798
• 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,
  589-593
• 3
  
  

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RADIOLOGY

• Kainulainen et al 1999
• CVID x 18, XLA x 4
• CXR HRCT
• Bronchiectasis 3 16

• 3 year follow-up
• ?
• Disease progression (5)
• Serum IgG
• Case No T0 T36
• 1 9.9 10.0
• 2 4.6 6.1
• 8 3.7 5.1
• 10 3.7 4.9
• 21 3.1 5.7

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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 2001
  4

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MANAGEMENT GENERAL ISSUES

• Shared Care (Immunologist/Respiratory Physician)
  optimal 4
• 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
• 
  (Cochrane Database of Systematic Reviews. 3,
  2003)
• Anti-inflammatory agents

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REPLACEMENT THERAPY

• Risk/benefit assessment
  4
• IV/Sc routes optimal 2
• ? pulmonary infections in XLA/CVID (v untreated)
  2
• Optimal dosing/frequency/serum IgG level not
  established
• Tailor route/dose/infusion frequency
  3
• --------------------------------------------------
  -------------
• Maintain IgG gt5g/l
  2
• Paediatric target mid reference range
  4
• IgG gt8g/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

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REPLACEMENT THERAPYHigh dose v low dose
secondary outcome, pulmonary function

• Eijkhout et al 2001 (randomised, double-blind,
  multicentre, crossover, n43)
• 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,
  n12)
• High dose ? ? FVC FEV1
  plt0.01
• Roifman et al 1987 (randomised, crossover, n12)
• Mean FEV1 FVC high dose phase v low dose
  phase plt0.01
• Bernatowska et al 1987 (two-dose, crossover,
  non-randomised, n13)
• High dose ? ?Max. expiratory flow FEV1
  NA
  

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ACUTE INFECTION

• MICROBIOLOGY
• Culture sensitivity routinely in acute setting
  3
• Value unclear in chronic situation - confirm
  original pathogen
• 
  - ? emerging resistance
• 
  - additional pathogens
• ANTIBIOTICS
• Effectiveness established in exacerbations
  (bronchiectasis) 2
• Higher doses for longer periods
  4
• Local treatment protocols
  4

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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
• - ?
  gt 3years treatment
• (The Cochrane Library, Oxford. 2, 2003)
• (Cochrane Database of Systematic Reviews.
  3, 2003)
• Bronchiectasis - limited meta-analysis (6 RCTs)
  
• - marginal
  benefit / cautious support
• (Evans et al. Thorax 2001)

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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 gt 3 exacerbations
  / year 4
• 
  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 4

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ANTIBIOTIC PROPHYLAXIS

• (Heelan et al., ESID 2002)

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SURGERY

• Diagnostic delay gt 2 years ? need for surgical
  procedures
• 
  
  Adequate treatment ?
  lobectomy/pneumonectomy by 95
• (UK PAD Audit 1993-96)
  
  3
• Important treatment option with favourable
  outcomes
• especially in focal bronchiectasis
• (Cohen et al 1994, Mansharamani Koziel
  2003)
  3

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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

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PIN GUIDELINES

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