PANCE Blueprint Pulmonary (10%)

Patient will present as → a 25-year-old cystic fibrosis patient complaining of chronic, frequent coughing productive of yellow and green sputum. She recently recovered from a Pseudomonas spp. pneumonia requiring hospitalization. On physical examination, you notice foul breath, purulent sputum, and hemoptysis along with a CXR demonstrating dilated and thickened airways with “plate-like” atelectasis (scarring)

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Bronchiectasis is described as the permanent dilation or destruction of the bronchial walls

• The most widely accepted model describes a "vicious cycle" of inflammation => airway destruction => abnormal mucus clearance, and infection or colonization by bacteria
• Bronchiectasis is best considered the common endpoint of various disorders that cause chronic airway inflammation
• The dilation and destruction of larger bronchi is caused by chronic infection and inflammation. Common causes of this inflammation are cystic fibrosis, immune defects, recurrent infections, and foreign body aspirations. Some cases are idiopathic
• The most common cause is cystic fibrosis
• Presents as chronic cough, purulent sputum, fever, weakness, weight loss, dyspnea in some patients, and hemoptysis
  • Hemoptysis is generally mild, originates from bronchial arteries, and is seen in 50% to 70% of all cases
• The main causative agents of bronchiectasis are adenovirus and influenza
• Bacterial causes: There is little common consensus but Streptococcus pneumoniae and Pseudomonas aeruginosa are the most commonly isolated bacteria
  • Colonization with P. aeruginosa tends to indicate severe disease and predicts worse outcomes

CXR – linear ("tram track") lung markings, atelectasis, dilated and thickened airways “Plate-like” atelectasis (scarring)

• Gold standard diagnosis is – CT of the chest

CXR demonstrating typical "plate-like" atelectasis associated with bronchiectasis

Aggressive antibiotics are the mainstay of treatment

• Chest physiotherapy, nutritional support, inhalable indomethacin (shown to decrease bronchial hypersecretion by inhibiting neutrophil recruitment), bronchodilators, supplemental oxygen, immunoglobulin administration for immunoglobulin deficiency, replacement treatment for patients with alpha-1-anti-trypsin deficiency, and recombinant DNAase to reduce sputum viscosity in patients with cystic fibrosis (CF)
• Eventually lung transplant