pulmonary cystic fibrosis

Cystic fibrosis (CF) results in chronic pulmonary disease in the majority of patients resulting from the production of viscous secretions and impairment of mucociliary clearance due to alterations in airway surface liquid.

Chronic infection results, and the combination of tissue damage by pathogenic organisms and a robust host inflammatory response leads to the development of bronchiectasis and progressive lung disease.

Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway secretions (mucus). CF lung disease pathogenesis reflects the vulnerability of airway surfaces to dehydration and collapse of mucus clearance.

This predisposition is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, resulting in :
 (i) the absence of CFTR-mediated Cl- secretion and regulation of epithelial Na+ channel (ENaC) function;
 (ii) the sole dependence on extracellular ATP to rebalance these ion transport processes through P2 purinoceptor signaling.

Synopsis

 mucus plugging of the tracheobronchial glands

 bronchial lesions

• hyperplasia of bronchial glands
• papillary hyperplasia of the bronchial mucosa
• bronchial obstruction
• bronchial infections
• chronic bronchitis

 bronchiolar lesions

• bronchiolitis
• peribronchiolar fibrosis

 obstructive pneumonia

• pulmonary interstitial inflammation
• pulmonary interstitial finrosis

 organizing pneumonia
 air-trapping
 collapse

 chronic sepsis

• bronchial ulceration
• destruction of the bronchial cartilage
• bronchiectasis
• pus-filled bronchiectatic cavities
• abscesses
• penumonic consolidation

 lymphoid follicular hyperplasia
 air cysts

• bronchiectatic cysts
• pneumatoceles
• interstitial emphysema
• emphysematous bullae

 pneumothora
 attenuated and shortened interalveolar septae

  pulmonary hypertension lesions
 cor pulmonale lesions
 hemoptyses

Complications

 infectious pneumonias

 bronchopulmonary aspergillosis

• Aspergillus bronchial colonisation (endobronchial aspergillosis)
• endobronchial semi-invasive aspergillosis
• allergic bronchopulmonary aspergillosis
• progressive chronic pulmonary aspergillosis (PCPA) (semi-invasive pulmonary aspergillosis)
• invasive pulmonary aspergillosis

See also

 cystic fibrosis

References

 Boucher RC. Cystic fibrosis: a disease of vulnerability to airway surface dehydration. Trends Mol Med. 2007 Jun;13(6):231-40. PMID: 17524805