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PRIMARY ANTIBODY DEFICIENCY (PAD) & BRONCHIECTASIS (UKPIN / BTS GUIDELINES)

PRIMARY ANTIBODY DEFICIENCY (PAD) & BRONCHIECTASIS (UKPIN / BTS GUIDELINES). BRONCHIECTASIS. A destructive lung disease characterised by: Abnormal & permanent dilatation of medium sized bronchi

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PRIMARY ANTIBODY DEFICIENCY (PAD) & BRONCHIECTASIS (UKPIN / BTS GUIDELINES)

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  1. PRIMARY ANTIBODYDEFICIENCY(PAD)&BRONCHIECTASIS(UKPIN / BTS GUIDELINES)

  2. BRONCHIECTASIS A destructive lung disease characterised by: • Abnormal & permanent dilatation of medium sized bronchi • An associated, persistent and variable inflammatory process producing damage to bronchial elastic and muscular elements

  3. PATHOLOGY Neutrophil proteases (acute infection in a normal or compromised host)  Epithelial injury + Structural protein damage  Damaged, dilated airway  Mucous retention / chronic, recurrent infection  Ongoing inflammation / tissue damage / repair

  4. BRONCHIECTASIS - aetiology • Infection - pertussis, influenza, measles, TB, necrotising peumonia • Bronchial obstruction - mucoid impaction, ABPA • Congenital anatomical lung abnormality • Inherited disorders - ciliary dysfunction - cystic fibrosis - alpha-1 AT deficiency • Undefined (29 - 49%)

  5. BRONCHIECTASIS OF UNDEFINED AETIOLOGY

  6. BRONCHIECTASISPasteur et al. Am J Respir Crit Care Med (2000) 162, 1277-1284

  7. BRONCHIECTASIS in PAD CVID • 53% (Hausser et al 1983) • 44% (Watts et al 1986) • 18% (Hermazewski & Webster 1993) • 20% (UK PAD Audit 1993-96) • 27% (‘chronic lung disease’) (Cunningham Rundles 1999) • 58% (Garcia 2001) • 43% (Busse et al 2002) XLA • 7% (Hermazewski & Webster 1993) • 12% (UK PAD Audit 1993-96) • 20% (Quartier et al 1999)

  8. RESPIRATORY INFECTIONS Figure 2 Types of infection in the 37 patients receiving immunoglobulin replacement treatment. The numbers of each type of infection are listed by each chart section.

  9. DIAGNOSTIC DELAY • Average: diagnosis - 6.3 years treatment - additional 3.9 • Diagnostic delay > 2 years:  risk of bronchiectasis sinusitis iron deficiency (UK PAD Audit 1993-96)3 • Strongest predictor of chronic pulmonary disease in treated patients is established lung disease at time of presentation n= XLAx10, CVIDx12 IMIg x 18, IVIg x 3, FFP x 1 (all + daily antibiotic) (Sweinberg et al 1991)3

  10. UK PAD AUDIT 1993-96 Development of bronchiectasis following diagnosis: <1980 1981-87 >1988 77% 70% 42%

  11. CHRONIC LUNG DISEASE in PAD Damage sustained prior to active treatment and/or Continued inflammation despite treatment

  12. AIMS Define evidence-based guidelines relevant to: • investigation level appropriate to screen for significant antibody deficiency in all patients with bronchiectasis • diagnosis & management of bronchiectasis complicating primary antibody deficiency

  13. Simple Evidence-based Consistent with existing, recognised standards Realistic Explicit Clear & well documented Credible & widely supported Results orientated (outcomes) Valid Reproducible Reliable Involving & representative of key disciplines Clinically applicable Clinically flexible Scheduled for review GUIDELINES

  14. LITERATURE REVIEW Meta-analyses Systematic reviews RCTs Longitudinal studies Case control/cohort studies Case reports/case series Expert opinions DATABASES Ovid Online Collection - Medline, preMedline - CINAHL, EMBASE - Journals@ovid EBM Reviews - Cochrane Systematic Reviews - Cochrane Controlled Trials - Effectiveness Reviews Abstracts - ACP Journal Club Allied & Complementary Medicine Specialty Contacts GUIDELINES

  15. GUIDELINES EVIDENCERECOMMENDATION 1 A 2 B 3 C 4 D (Good Practice Points) SIGN, RCPCH, BTS

  16. DIAGNOSIS PRIMARY ANTIBODY DEFICIENCY • Humoral abnormalities are common in bronchiectasis 3 • Respiratory Physician + Immunologist 4 • Diagnosis of significant antibody deficiency should entail use ofestablished and widely accepted criteria: 4 - Primary Immunodeficiency Diseases. Report of an IUIS Scientific Group Clinical & Experimental Immunology 1999 (118), Suppl 1:1-34 - Diagnostic Criteria for Primary Immunodeficiencies. Clinical Immunology 1999 (93), 190-197 - Practice parameters for the Diagnosis & Management of Immunodeficiency. Annals of Allergy, Asthma & Immunology 1996 (76), 282-294

  17. PFTs - Reversible/irreversible bronchial obstruction - Granulomatous disease etc. • Correlate poorly with Radiology(bronchiectasis)3 - Pulmonary abnormalities in patients with primary hypogammaglobulinaemia Kainulainen et al. Jounal of Allergy & Clinical Immunology (1999) 104, 1031-1036 - Pulmonary manifestations of hypogammaglobulinaemia Dukes et al. Thorax (1978) 33, 603-607 - Radiologic findings of adult primary immunodeficiency disorders: contribution of CT Obregon et al. Chest (1994) 106, 490-495 • Static volumes/flow-volume loops

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