panbronchiolitis%20-%20diffuse
PANBRONCHIOLITIS - DIFFUSE
Diffuse panbronchiolitis is an inflammatory disease affecting the respiratory bronchioles.
It causes progressive suppurative and obstructive respiratory disease.
This is an idiopathic disease which is primarily found in Japan, Korea and China.
Predisposition to the disease may be genetically-related but environmental factors should also be considered.
Failure to treat diffuse panbronchiolitis can lead to development of bronchiectasis, progressive failure and death.

Differential Diagnosis

  • Bronchiectasis
  • Bronchiolitis (infectious)
  • Chronic bronchitis
  • Cystic fibrosis
  • Hypogammaglobulinemia
  • Primary ciliary dyskinesia
  • Allergic bronchopulmonary aspergillosis (ABPA)
  • Obliterative bronchiolitis
  • Sarcoidosis
  • Mycobacterium Avium complex pulmonary infection
  • Churg-Strauss syndrome
  • Wegener’s granulomatosis

Diagnosis

Diagnostic Criteria for DPB by Ministry of Health & Welfare of Japan

  • Diagnosis of diffuse panbronchiolitis (DPB) is definite if the first 3 & at least 2 of the last 3 criteria are present
    • Presence of persistent cough, sputum production & dyspnea on exertion
    • History of chronic paranasal sinusitis
    • Chest X-ray (CXR) shows bilateral diffuse small nodular shadows, or chest computed tomography (CT) scan w/ centrilobular micronodules
    • Presence of coarse crackles, occasional rhonchi, wheezes or squawk on physical exam
    • FEV1/FVC <70% & PaO2 <80 mmHg
    • Cold haemmaglutinin titre ≥64

Physical Examination

  • Crackles, wheezing on chest exam

Laboratory Tests

Pulmonary Functions Tests

  • Spirometry, lung volume determination
    • May show significant obstructive defect w/ or w/o superimposed restriction
    • Significantly limited airways resistant to bronchodilators
    • FEV1/FVC <70%, vital capacity (VC) <80%, residual volume (RV) >150%
    • Hypoxemia may be present in blood gas analysis
  • P. aeruginosa infection, reduced pulmonary diffusing capacity, progression of hypoxemic state & subsequent hypercapnia seen in advanced stages of diffuse panbronchiolitis (DPB)

Imaging

  • Chest X-ray (CXR)
    • May show pulmonary hyperinflation & diffuse bilateral small nodular shadows
    • Findings of bronchiectasis (ring-shaped/tram-line shadows) may be seen in advanced disease
  • High-resolution computerized tomography (HRCT)
    • Bilateral & symmetric centrilobular nodules forming a “tree-in-bud” pattern,
    • Bronchiolectasis
    • Mosaic air trapping on lung periphery
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