Airway disease Flashcards
1
Q
Paraseptal emphysema
A
Pathology:
- Thin/tall habitus: ?negative pressure at apices from gravity
- Associations: smoker, IVDU, HIV, Connective tissue disorder
- Can cause spontaneous pneumothorax
IMAGING:
- <10mm (if larger –> subpleural bullae)
- Subpleural and peribronchovascular arcades of cystic spaces
- Cystic spaces separated by intact interlobular septa
- Upper lobe predominance
DDx:
- Cystic lung disease
- Centrilobular emphysema
- Panlobular emphysema
- Honeycombing
2
Q
Cystic Fibrosis
A
Pathology:
- Abnormal chloride ion transporter: Thick mucous/secretions –> not cleared –> infected repeatedly –> chornic inflammation –> scarring/fibrosis of airways –> bronchiectasis
- Often colonised with Pseudomonas Aeruginosa
-
Genetics:
- Autosomal recessive,
- Various mutations of CFTR (cystic fibrosis transmembrane conduction regulator) gene on chromosome 7
- Most common(90%) mutation is: p.F508del - deletion of 3 nucleotides resulting in loss of phenylalanine [F] at the 508th position of the protein[p]
Associations:
- Exocrine pancreatic insufficiency
- Pansinusitis
- Biliary cirrhossis
- Bone demineralisation
- Infertility
Epi:
- 3200 per year
- Caucasians >>>>african americans/asians
Clinical: most commonly childhood onset
- Merconium ileus
- Failure to thrive
- Recurrent respiratory infection: p.aeruginosa, S.Aureus, H.Influenzae
- Cough, wheeze, SOB
- Pneumothorax
- Hemoptysis
Prognosis:
- most survive to >40
- Death: PAH, cor-pulmonale, hemoptysis
Rx:
- Bronchodilators
- Promote airway secretion clerance: chest physio, inhaled hypertonic saline/DNAse 1
- Antibiotic: prophylactic + treatment
- Lung transplant
Imaging:
- upper lobe predominant diffuse bronchiectasis + bronchial wall thickening
CT/Radiograph:
- May be normal in early disease
- Air trapping/mosaic attenuation/hyperinflation
- Mucoid impaction: finger in glove centrally, V/Y/tree-in-bud opacities in peripheral bronchioles or finger in glove
-
Bronchiectasis:
- upper lobe predominant
- Air fluid level suggests acute exacerbation
- Bronchial wall thickening
- Atelectasis: subsegmental-lobar
- Consolidation (infection)
- Subpleural bullae/cystic change
- Hilar/mediastinal lymphadneopathy (chronic inflammation)
- Pulmonary artery hypertension
- Pneumothorax
- Fatty degeneration of pancreas
Angiogram: dilated bronchial arteries, may be embolised for control of bleeding
DDx
- ABPA
- Primary ciliary dyskinesia
- Post infectous bronchiectasis (more focal)
Follow up:
-
CXR:
- 2-4 years in stable patients
- Every year if deteriorating PFT
- HRCT: diagnosis of early disease, complications and progression
- MRI: disease progression
3
Q
Infectious Bronchiolitis
A
Pathology:
- Acute bronchiolar injury –> mocosal injury (epitheilia necrosis + wall inflammation) –> neutrophil rich intra-luminal exudates.
-
Eitiology:
- Children: viral/bacterial (mycoplasma)
- Immunocompromised: fungal, CMV
- Bacterial: chlamydia, mycobacteria, S.Aureus, legionella, hemophilis influenzae, pseudomonas
Clinical:
- LRTI/URTI
- Symptom severity children >> adult
Rx:
- Supportive
- Bronchodilators
- Therpay agains organism (if known)
- Steroids
Imaging:
- Centrilobular nodules/GGO
- Tree-in-bud
- Mosaic attenuation/air trapping
- Consolidation: more common in bacterial infection
- Cavitary lesions: TB, MAC, fungus
- Bronchiectasis: MAC
DDx:
- Hypersensitivity pneumonitis
- Aspiration pneumonia
- Respiratory bronchiolitis +/- associated ILD
- Follicular bronchiolitis
- Diffuse Panbronchiolitis
- Constrictive bronchiolitis
4
Q
ASTHMA
A
Pathology:
- Chronic inflammation of small/mid-sized bronchi:
- Wall oedema
- Smooth muscle and glandular remodelling/hypertrophy
- Bronchiolar wall thickening
- Mucous stasis
- Constrictive bronchiolitis
- Cause chronic obstructive disease, typically reversible.
- Common triggers: animal, dust, weather, mold, infection
- Associations: ABPA, Bronchocentric granulomatosis
Radiograph/CT:
- Bronchial wall thickening or peribronchial cuffing (most common)
- Hyperinflation: Transient or fixed
-
Complications:
- Atelectasis: related to mucoid impaction, typically RML
- Pneumothorax & pneumomediastinum
- Pneumopericardium, pneumoretroperitoneum
- Rarely pneumorrhachis & subdural air
-
Bronchiectasis:
- mucoid impactions: finger in glove
- can be associated with ABPA (often cystic/varicose)
-
Bronchiolitis:
- Small centrilobular nodules
- Air trapping
- Mosaic perfusion
- Pneumonia
DDx:
- Vocal cord paralysis
- Tracheal/carinal obstruction
- Constrictive bronchiolitis
- Infiltrative lung disease
5
Q
Chronic Bronchitis
A
Pathology:
- Productive cough on most days for > 3 months in each of 2 consecutive years without other causes
- Aetiology: smoking, air pollution, occupational exposure, genetics
- Genetics: linkage to chromosomes 22/12p
Associations:
- Emphysema
- Lung cancer
- Coronary artery disease
Epi:
- 4% adults >18
- M > F
Clinical:
- Cough, SOB, Wheeze
- Blue bloater
- Cor Pulmonale
- PFT often normal
Radiograph:
- Often normal
- Bronchial wall thickening: tram-tracking, peribronchial cuffing, increased intersitial lung markings/peribronchial thickening
- Hyperinflation
- Other: Cor pulmonale, Saber-sheath trachea
CT:
- Bronchial wall thickening
- Mucous in tracheobronchial tree
- Air trapping/mosaic attenuation
- Cor pulmonale: enlarged right heart/PA
- Associated emphysematous changes
Rx:
- Smoke cessation
- Bronchodilators
- Steroids
- Antibiotics for recurrent infections
- Immunisation: pneumococcus and influenza
DDx:
- Centrilobular emphysema
- Asthma
- Acute Bronchitis
6
Q
Broncholithiasis
A
Pathology:
- Calcified or ossified endobronchial material
- Calcium phosphate (85%) >> calcium carbonate (15%)
- Causes:
- Erosion and extrusion of calcified lymph nodes (long standing necrotising granulomatous lymphadenitis): TB, histioplasmosis
- Silicosis
- Erosions/extrusion of calcified bronchial cartilage
- Endobronchial infection + dystrophic calcification
Associations:
- PAVM
- Broncho-eosphageal fistula
Clinical:
- Cough
- Hemoptysis
- Recurrent LRTI
- Lithoptysis
Rx: bronchoscopy and removal, surgical lobectomy
Imaging:
-
Location:
- Middle lobe > upper lobe.
- Right > Left.
-
Endobronchial/peribronchial calcified nodule
- Distortion/narrowing of adjacent airway
- Complete airway obstruction
- Extraluminal air –> endobronchial erosion
- Solitary >> multiple
- Does not enhance
-
Signs of bronchial obstruction:
- Atelectasis
- Post-obstructive pneumonia
- Bronchiectasis
- Air trapping/mosaic perfusion
- Mucoid impaction
DDx:
- Carcinoid (40% calcify)
- Airway Harmatoma
- Tracheobronchopathia Osteochondroplastica
- Airway Amyloidosis
- Mediastinal fibrosis
7
Q
Centrilobular emphysema
A
Pathology:
- Strongly linked to smoking
- Associations: respiratory bronchiolitis, chornic bronchitis, hyperinflation, secondary pulmonary hyperteion
- 30% of normal lung must be destroyed before pulmonary function deteriorates
Imaging:
- Location: upper lobe predominance
- Centrilobular low attenuation: no discernible wall, surroudning normal lung, SPL border preserved.
- Hyperinflation
- Incidental finding
DDx:
- Panlobular emphysema
- Paraseptal emphysema
- Cystic lung disease
- Constrictive bronchiolitis
- LCH
- Asthma
8
Q
Bronchiectasis
A
Pathology:
- Irreversible dilatation of bronchus/bronchi +/- bronchial wall thickening
-
3 morphological types:
- Cylindrical: tram-tracking, non-tapering, unifrom diameter
- Varicose: string of pearls, alternating dilation/narrowing
- Cystic: bunch of grapes, marked dilatation, rounded
-
Aetiology:
- Idiopathic (40%)
- Congenital: CF, primary Ciliary dyskinesia, Immunocompromosied, bronchial atresia, Mounier-Kuhn syndrome, Williams campbell syndrome
- Infectious: bacterial/viral/fungal.
- ABPA
- Inflammatory: RA, sarcoid, chronic rejection post transplant
- Pulmonary fibrosis: traction bronchiectasis
Imaging questions: (see differentials flash card)
- Morphology + size
- Focal (often post infectious/obstructive) vs diffuse
-
Location (Diffuse):
- Central vs peripheral
- upper/middle/lower lobe predominance
Radiograph:
- Bronchial dilatation
- Bronchial wall thickening: tram-tracking, peribronchial cuffing
- Finger in Glove: mucoid impaction
- Lung: volume loss, cystic change, COPD
HRCT:
- Bronchoarterial ratio >1
- Bronchial wall-thickening
- Signet ring sign
- Finger in glove (mucoid impaction)
- Non-tapering bronchi
- Air-trapping/mosaic attenuation
- Traction bronchiectasis: Bronchiectasis + abnormal lung parenchyma
- *Rx:**
- Smoke cessation
- vaccination
- Postural drainage
- Treat infection
- Surgery if unresponsive to above
9
Q
A
10
Q
Pan lobular emphysema (PLE)
A
Pathology:
- Eitiology:
- Alpha-1-antitrypsin deficiency
- Random
- Congenital bronchial atresia
- Association:
- Chronic bronchitis
- REcurrent infection
- Pulmonary hypertension
Imaging:
- Homogenously distributed
- Lower lobe predominance
- Diffuse regions of low attenuation: Ill-defined
- Difficult to differential between normal lung and PLE
- Bronchiectasis
DDx:
- Centrilobular emphysema
- Paraseptal emphysema
- Cystic lung disease
- Asthma
11
Q
Allergic Bronchopulmonary Aspergillosis
A
Pathology:
- Type 1 hypersensitivty reaction (IgE/IgG) to Asperigillus fumigatus
- Necrotising granulomatous inflammation –> destroys wall of bronchi/bronchioles –> bronchiectasis
- Inspissated mucous plugs containing aspergillus and eosinophils
- does NOT invade mucosa
- Most common cause of eosinophilic lung disease
5 stages:
- Acute disease
- Remission
- Exacerbation/recurrence
- Steroid-dependent asthma
- Fibrosis
Associations:
- 1-15% CF
- 2-32% asthma
Epi: any age, most common 30-40
Clinical:
- cough
- SOB
- Wheeze
- Low grade fever
- Malaise
- Sputum (thick)
- PFT: obstruction, air-trapping, reduced FEV1
Major criteria:
- Asthma/CF
- Immediate skin reactivity with aspergillus Ag
- Serum precipitating Ab to aspergillus fumigatus
- Increased IgE
- Eosinophilia
- Elevated IgE/IgG ab to aspergillus fumigatus
- Central bronchectasis HRCT
- Airspace disease on CXR
Minor criteria:
- sputum aspergillus fumigatus
- Expectoration of brown mucous plugs
- Late skin reactivity with aspergillus-Ag
Prognosis:
- Recurrent infection –> widespread bronchiectasis/fibrosis
- 35% of exacerbations are asymptomatic
Rx:
- Steroids
- Antifungals
- Mab
CT/Radiograph:
- Location: Upper lobe predominance, more central
- Bronchiectasis + wall thickening: often multilobar, cystic/saccular
- Mucous impaction: finger-in-glove, high-attenutaiont
- Lobulated masses
- Centrilobular nodules
- Consolidation/GGO
- Ateletasis (obstruction)
- Air-trapping/mosaic perfusion
DDx:
- Endoluminal lesion: SCC, Small-cell cancer, carcinoid, Mets
- Bronchiectasis: CF, post-infectious, primary ciliary dyskinesia
- Bronchial atresia
- Foreign body
- PAVM
- TB
LINK
12
Q
Constrictive Bronchiolitis
A
Pathology
- Narrowing of membranous and respiratory bronchioles
-
Post infectious:
- Childhood infection
- Swyer-James-Macleod syndrome
- CF
- Lung transplant: chornic rejection, graft vs host, CMV infection
- Hematopoetic stem-cell transplantation: graft vs host
- Connectie tissue disease: RA, SLE, sjogrens, scleroderma
- Inhalational lung disease: NO, Sulfur dioxide, amonia
- idiopathic
- Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
- Other: IBD, paraneoplastic
Clinical:
- Chronic and slowly progressive >>> rapidly progressive
- Rx: dependent on eitiology
Imaging:
- Mosaic attenuation
- Air trapping
- Associated findings: bronchiectasis, bronchial wall thickening, pulmonary nodules
- SJM syndome: focal lung hyperlucency and reduced vascularity
DDx:
