Bronchiectasie Flashcards
General fingings
Bronchiectasis is an irreversible and abnormal dilation in the bronchial tree that is generally caused by cycles of bronchial inflammation in addition to mucous plugging and progressive airway destruction. Although the widespread use of antibiotics has made bronchiectasis rare, conditions such as cystic fibrosis (CF), severe or protracted pneumonia, immunodeficiency, and COPD continue to cause it. The most important clinical finding is a chronic cough with copious mucopurulent sputum. Other symptoms may include dyspnea, rhinosinusitis, and hemoptysis. Physical examination reveals crackles and rhonchi on auscultation, often accompanied by wheezing. High-resolution computer tomography is the best diagnostic test and shows thickened bronchial walls, a signet-ring appearance, and “tram track” lines. Treatment focuses on alleviating symptoms and preventing exacerbations, and includes pulmonary physiotherapy and antibiotics to treat underlying infections. In rare cases, massive hemoptysis may complicate bronchiectasis and necessitate surgery or pulmonary artery embolization.
Etiology
Pulmonary infections are thr most common cause of bronchiectasis in developing countries while in developing countries, most cases are probably caused by tuberculosis, particularly in patients with impaired immune function due to undernutrition or human immunodeficiency virus (HIV) infection.
-Pulmonary infections (i.e., bacterial, viral, fungal), especially severe or chronic infections
Diffuse bronchiectasis
-Disorders of secretion clearance or mucous plugging
-Cystic fibrosis (CF)
-Primary ciliary dyskinesia (PCD)
👓Allergic bronchopulmonary aspergillosis (ABPA)
Bronchial narrowing or other forms of obstruction
-COPD
-Aspiration
-Tumors
-Immunodeficiency (e.g., common variable immunodeficiency Bruton, hypogammaglobulinemia, HIV)
-Chronic inflammatory diseases (e.g., rheumatoid arthritis, Sjögren syndrome, Crohn’s disease)
Focal bronchiactasis
- Tumors
- Untreated pneumonia or obstruction (eg, due to foreign bodies, tumors, postsurgical changes, lymphadenopathy)
Clinica
Tosse cronica produttiva con escreato mucopurulento copioso associato ad emottisi pensare sempre a bronchiectasia.
-💥Chronic productive cough (lasting months to years) with copious mucopurulent sputum ; the following may be heard on auscultation:
1.Crackles and rhonchi
2.👓Wheezing (due to obstruction from secretions,
airway collapsibility, or a concomitant condition)
3.Bronchophony
- Rhinosinusitis
- Dyspnea
- 🧨Hemoptysis: usually mild/self-limiting but severe hemorrhage that requires embolization may occur.
- Nonspecific symptoms (i.e., fatigue, weight loss, pallor due to anemia)
- Clubbing of nails (uncommon)
Exacerbations of bronchiectasis
- Increased production of mucous above baseline
- Low-grade fever
Imaging
NB. Bronchiectasis cannot be ruled out with a chest x-ray!💥
NB Thickening of the airway walls and/or airway dilation!💥
1.Chest x-ray (best initial test)
-Inflammation and fibrosis of bronchial walls lead to the appearance of parallel “tram track” lines. This is a characteristic sign of bronchial wall thickening. (binario)
-Thin-walled cysts (i.e., dilated bronchi forming sacs), possibly with air-fluid levels
-Late-stage bronchiectasis: honeycombing (lesioni cistiche su sfondo fibrotico)
Nella fibrosi cistica le lesioni predominano agli apici polmonari/upper lobes
- High-resolution computer tomography (HRCT): confirmatory test!!
- Dilated bronchi with thickened walls; possible signet-ring appearance and tram track lines
- Cysts, especially at bronchial ends in lower lobes, and honeycombing
3.Bronchoscopy: to visualize tumors, foreign bodies, or other lesions; may also be used in combination with bronchoalveolar lavage (BAL) to obtain specimens for staining and culture
Pulmonary function tests
Attenzione: versamento pleurico e pneumotorace risultano in una alterazione di tipo restrittiva mentre le bronchiectasie determinano una alterazione ostruttiva
Findings consistent with obstructive pulmonary disease (i.e. ↓ FEV1/FVC ratio)
The FEV1 may improve in response to beta-agonist bronchodilators. In more advanced cases, progressive fibrosis may result in decreases in forced vital capacity (FVC), evidence of a restrictive defect on lung volume measurements, and a decreased diffusing capacity for carbon monoxide (DLco).
LAB
- Sputum culture and smear: to determine infectious etiology (i.e. bacteria, mycobacteria, and fungi)
- Blood tests
- CBC with differential: may show anemia of chronic disease and ↑ WBCs, ↑ neutrophils
- HIV testing, genetic testing for CF, immunoglobulin quantitation to determine other possible etiologies
- Serum immunoglobulins (IgG, IgA, IgM) and serum electrophoresis to diagnose common variable immunodeficiency
- Targeted assessment of baseline and specific antibody responses to peptide and polysaccharide antigens (ie, tetanus, capsular polysaccharide of S. pneumoniae and H. influenzae type b) done to assess immune responsiveness
- Two sweat chloride tests and CFTR gene mutation analysis to diagnose cystic fibrosis (including in adults > 40 years without an identifiable cause of bronchiectasis, especially those with upper lobe involvement, malabsorption, or male infertility)
- Rheumatoid factor, antinuclear antibody (ANA), and antineutrophil cytoplasmic antibody testing if an autoimmune disorder is being considered
- Serum IgE and Aspergillus precipitins if patients have eosinophilia, to rule out allergic bronchopulmonary aspergillosis
- Alpha-1 antitrypsin level to evaluate for alpha-1 antitrypsin deficiency if high-resolution CT shows lower lobe emphysema
👓Primary ciliary dyskinesia should be considered if adults with bronchiectasis also have chronic sinus disease or otitis media, particularly if problems have persisted since childhood
Treatment
Bronchiectasis is a permanent anatomical malformation and therefore cannot be cured. However, symptoms and advancement of the disease can be controlled. The treatment of any underlying cause is also very important.
SI CORTICOSTEROIDI
L’emorragia polmonare è indicazione a resezione chirurgica!
✔Conservative
- Bronchopulmonary hygiene and chest physiotherapy: “cupping and clapping” and postural drainage, hydration, directed cough
- Antibiotic therapy of exacerbations
- Smoking cessation
- Vaccinations (i.e. seasonal influenza vaccine, pneumococcal vaccine)
- Bronchodilators, corticosteroids, and nebulized hypertonic saline are not routinely used but may be considered, especially in patients with notable obstructive symptoms.
✔Invasive procedures
-Surgical resection of bronchiectatic lung or lobectomy: indicated in
1.pulmonary hemorrhage
2.inviability of bronchus
3.substantial sputum production in unilateral
bronchiectasis
- 👓Pulmonary artery embolization: indicated in pulmonary hemorrhage
- Lung transplantation should be considered in severe disease.
Complicanze
🧨As the disease progresses, chronic inflammation and hypoxemia cause neovascularization of the bronchial (not the pulmonary) arteries. Bronchial artery walls rupture easily, leading to massive hemoptysis. Other vascular complications include pulmonary hypertension due to vasoconstriction, arteritis, and sometimes shunt from bronchial to pulmonary vessels.
- Recurrent bronchopulmonary infections → obstructive ventilation disorder → respiratory failure and cor pulmonale
- Pulmonary hemorrhage (massive hemoptysis)
- Pleural empyema
- Lung abscess👓
Prevention
-Primary prevention: antibiotic control of bronchial and pulmonary infections in predisposed individuals
Secondary prevention: long-term low-dose macrolide 🧨treatment (e.g., azithromycin) in patients with two or more bronchiectasis exacerbations within one year
(ricorda il trattamento con bactrim nelle UTI ricorrenti nella donna in post menopausa)
Allergic bronchopulmonary aspergillosis
A hypersensitivity reaction to Aspergillus species that occurs most commonly in people with asthma, but sometimes in patients with CF, can cause or contribute to bronchiectasis.
Forma diffusa
Diffuse bronchiectasis occurs when a causative disorder triggers inflammation of small and medium-sized airways, releasing inflammatory mediators from intraluminal neutrophils. The inflammatory mediators destroy elastin, cartilage, and muscle in larger airways, resulting in irreversible bronchodilation. Simultaneously, in the inflamed small and medium-sized airways, macrophages and lymphocytes form infiltrates that thicken mucosal walls. This thickening causes the airway obstruction frequently noted during pulmonary function testing.
With disease progression, inflammation spreads beyond the airways, causing fibrosis (questo spiega immagne a nido d’ape in fase avanzata alla HRCT) of the surrounding lung parenchyma. What inflames the small airways depends on the etiology of bronchiectasis. Common contributors include impaired airway clearance (due to production of thick, viscous mucus in CF, lack of ciliary motility in PCD, or damage to the cilia and/or airways secondary to infection or injury) and impaired host defenses; these factors predispose patients to chronic infection and inflammation. In the case of immune deficiency (particularly CVID), autoimmune inflammation may also contribute.
Forma focale
Focal bronchiectasis usually occurs when a large airway becomes obstructed. The resulting inability to clear secretions leads to a cycle of infection, inflammation, and airway wall damage. The right middle lobe is involved most often because its bronchus is small and angulated and has lymph nodes in close proximity. Lymphadenopathy due to mycobacterial infection sometimes causes bronchial obstruction and focal bronchiectasis.
