Bronchiectasis

Background

Trachobronchial anatomy.

A - Alveoli AS - Septum alveolare BR - Bronchus respiratorius BT - Bronchus terminalis D - Mucous gland DA - Ductus alveolaris M - Musculus N - Nervus PA - Branch of Arteria pulm. PV - Branch of Vena pulm.

Bronchiectasis normal vs affected airway.

Bronchiectasis pathogenic cycle.

• Uncommon disease caused by chronic infection and inflammation
• Characterized by dilation and destruction of larger bronchi
  • Mucus build up attracts bacteria/microbes--> bacteria multiply--> additional lung infection/inflammation-->more airway damage--> over time, enlarged/widened airways make it harder to breath and clear mucus
• Commonly caused by cystic fibrosis, immune defects, recurrent pneumonias, or (less commonly), congenital or idiopathic

Clinical Features

An example of clubbing.

• Chronic cough productive of thick, often purulent sputum
• Dyspnea
• Crackles/rhonchi, wheezing
• +/- pleuritic chest pain
• +/- hemoptysis, due to airway neovascularization, can be massive
• If advanced;
  • Hypoxia, clubbing
  • Pulmonary hypertension, right-sided heart failure
• Acute exacerbation results from new/worsened infection, may have fever and constitutional symptoms

Differential Diagnosis

Acute dyspnea

Emergent

• Pulmonary
  • Airway obstruction
  • Anaphylaxis
  • Angioedema
  • Aspiration
  • Asthma
  • Cor pulmonale
  • Inhalation exposure
  • Noncardiogenic pulmonary edema
  • Pneumonia
  • Pneumocystis Pneumonia (PCP)
  • Pulmonary embolism
  • Pulmonary hypertension
  • Tension pneumothorax
  • Idiopathic pulmonary fibrosis acute exacerbation
  • Cystic fibrosis exacerbation
• Cardiac
  • Cardiac tamponade
  • Cardiogenic pulmonary edema (CHF)
  • Myocardial Infarction
  • Pericarditis
  • Myocarditis
• Other Associated with Normal/↑ Respiratory Effort
  • Abdominal distension
  • Anemia
  • CO Poisoning
  • Salicylate toxicity
  • Diabetic ketoacidosis (DKA)
  • Diaphragm injury
  • Electrolyte abnormalities
  • Epiglottitis
  • Flail chest
  • Hypotension
  • Metabolic acidosis
  • Pneumonia
  • Pneumothorax/hemothorax
  • Renal Failure
  • Sepsis
  • Toxic ingestion
• Other Associated with ↓ Respiratory Effort
  • Guillain-Barre syndrome
  • Multiple sclerosis
  • Myasthenia Gravis
  • Lambert-Eaton Syndrome
  • Organophosphate toxicity
  • Stroke (Main)
  • Tick paralysis

Non-Emergent

• ALS
• Ascites
• Uncorrected ASD
• Congenital heart disease
• COPD exacerbation
• Fever
• Hyperventilation
• Interstitial lung disease
• Neoplasm
• Obesity
• Panic attack
• Pleural effusion
• Polymyositis
• Porphyria
• Pregnancy
• Rib fracture
• Spontaneous pneumothorax
• Thyroid Disease
• URI

Evaluation

Chest X-ray of hyperinflated lung with bronchiectasis at the right upper lobe.

Bronchiectasis on CT.

• Evaluate for alternative causes of symptoms
• CXR
  • Thickening of airway walls and/or airway dilation
  • Linear perihilar densities, indistinctness of central pulmonary arteries
  • "Tram-track sign"; thickened, dilated airways perpendicular to x-ray beam
  • Elongated, tubular opacities due to mucus plugs
• CT chest
  • Sensitive and specific
  • Airway dilation, signet ring sign (thickened, dilated airway adjacent to smaller artery
  • Lack of normal bronchial tapering-->visible medium bronchi extending out closer to pleura
  • "Tram-track" sign
• Infectious workup
  • Consider sputum for TB
• Diagnosis of cause generally outside ED scope but may include workup for cystic fibrosis, immunodeficiency, ciliary dysmotility

Management

• Bronchodilators
• Supplemental O2 PRN
• Chest PT
• Mucolytics (e.g. NAC mucomyst, hypertonic saline nebs)
• Antibiotics
  • Some patients may be on chronic macrolides or other antibiotics
  • If no CF and no prior culture data, treat against H. influenzae, M. catarrhalis, S. aureus, and S. pneumoniae (e.g. amoxicillin-clavulanate, azithromycin, TMP-SMX)
  • If CF or more severe exacerbation, broaden antibiotics, knowing that many CF patients develop highly resistant polymicrobial infections and likely require multiple antibiotics (e.g. tobramycin, aztreonam, Zosyn, cefepime
  • Consider inhaled antibiotics to reduce sputum bacterial load
• Treat massive hemoptysis

Disposition

• Patients with CF and new hypoxia/significant symptoms are commonly admitted