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RADIOLOGY - CXR Bronchiectasis - vessel crowding - loss of vessel markings - tramline/ring shadows - cystic lesions/ air-fluid levels - evidence of TB.

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Presentation on theme: "RADIOLOGY - CXR Bronchiectasis - vessel crowding - loss of vessel markings - tramline/ring shadows - cystic lesions/ air-fluid levels - evidence of TB."— Presentation transcript:

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 3 chromosomal radiosensitivity - plain CXR (x 3 days background) - HRCT: x conventional CT: x 200 ? routine baseline ? (a)symptomatic monitoring

3 UNSUSPECTED DISEASE UNSUSPECTED DISEASE (Clinical v CXR v HRCT) Bronchiectasis in Hypogammaglobulinaemia - A Computed Tomography assessment. Curtin et al. Clinical Radiology (1991) 44, 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, Review Article: Imaging in Bronchiectasis. Smith et al. British Journal of Radiology (1996) 69,

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

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 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


8 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 Maintain IgG >5g/l 2 Paediatric target: mid reference range 4 IgG: >8g/l infection (v 5g/l, XLA, children) 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 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 1 p<0.01 Roifman et al 1987 (randomised, crossover, n=12) Mean FEV 1 & 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 1 NA


11 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

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) (Cochrane Database of Systematic Reviews. 3, 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 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

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


16 SURGERY Diagnostic delay > 2 years: need for surgical procedures Adequate treatment: lobectomy/pneumonectomy by 95% (UK PAD Audit ) 3 Important treatment option with favourable outcomes especially in focal bronchiectasis (Cohen et al 1994, Mansharamani & Koziel 2003) 3

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

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