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Questions and Answers
What is the primary characteristic of bronchiectasis?
Which of the following conditions is NOT a predisposing factor for bronchiectasis?
What dysfunction is most commonly associated with primary ciliary dyskinesia?
Which imaging method is most effective for visualizing bronchiectasis?
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What is a common consequence of impaired ciliary function in conditions like cystic fibrosis?
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In bronchiectasis, what causes permanent dilation of the bronchi and bronchioles?
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What condition is associated with situs inversus in addition to bronchiectasis?
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Which of the following is a common feature observed in patients with cystic fibrosis?
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Which type of chronic bronchitis is characterized by a productive cough without airflow obstruction?
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What is the primary cause of chronic bronchitis?
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What does a Reid index greater than 0.5 indicate?
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Which histological feature is NOT associated with bronchiectasis?
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Which histological change is typical of chronic bronchitis?
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What is the classic biochemical abnormality observed in cystic fibrosis?
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Which symptom is least likely associated with asthma?
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Which factor is least likely to contribute to the pathogenesis of chronic bronchitis?
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Which of the following complications is NOT typically associated with cystic fibrosis?
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In chronic asthmatic bronchitis, what occurs alongside hyperreactive airways?
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What primary defect is responsible for the symptoms of cystic fibrosis?
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Which mechanism contributes to the recurrent pulmonary infections seen in cystic fibrosis?
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What does chronic inflammation in chronic bronchitis primarily lead to?
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In the context of bronchiectasis, which pathogen is most commonly associated with chronic infections?
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How does cystic fibrosis primarily affect the pancreas?
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What role does the CFTR protein play in cystic fibrosis pathogenesis?
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Study Notes
Papanicolaou Stain
- Reddish core with a fuzzy margin
- Cells attached secondarily
- Arrow points at a macrophage
Charcot-Leyden Crystals
- Eosinophils
Bronchiectasis
- Permanent dilation of bronchi and bronchioles
- Destruction of smooth muscle and supporting elastic tissue
- Results from or is associated with chronic necrotizing infections
- Not a primary disorder
Conditions Predisposing to Bronchiectasis
-
Congenital or hereditary conditions:
- Cystic fibrosis
- 1° ciliary dyskinesia (immotile cilia syndrome/Kartagener syndrome)
- Immunodeficiency states associated with recurrent bacterial infections (i.e., immunoglobulin deficiencies)
-
Postinfectious conditions:
- Necrotizing bacterial pneumonias (especially with Staph aureus, Klebsiella, or TB in endemic regions)
- Bronchial obstruction due to tumor, foreign bodies
- Other conditions: Rheumatoid arthritis, SLE, IBD, chronic GVHD
Pathogenesis of Bronchiectasis
-
Cases due to nonspecific obstruction and infection:
- Both are necessary for full-blown lesions
- Either can be the initiating factor
-
Normal clearing mechanisms are impaired:
- Most readily apparent in cystic fibrosis
- Thick viscid mucus obstructs airways and predisposes to infections
- Most readily apparent in cystic fibrosis
-
Primary ciliary dyskinesia (immotile cilia syndrome):
- Autosomal recessive/variable penetrance
- Defects in ciliary motor proteins (dynein arm mutations)
- Kartagener syndrome: situs inversus, bronchiectasis, sinusitis
- Bronchiectasis
- Sterility in males: sperm dysmotility
- Poorly functioning cilia: Retention of secretions --> recurrent infections --> bronchiectasis
Immotile Cilia Syndrome
- Normal dynein arms
- Immotile cilia syndrome
Bronchiectasis: CT Scan and Gross Specimen
- Dilated airways on the CT scan in longitudinal section (arrowhead) and cross section (arrow)
- Cut surface of lung with distended peripheral bronchi filled with mucopurulent secretions (white arrow) in cystic fibrosis
Bronchiectasis: Gross
- Bronchiectasis tends to be localized with disease processes
- Widespread bronchiectasis is typical in patients with cystic fibrosis
Bronchiectasis: Histology
- Desquamation of surface epithelium with ulceration
- Destruction of cartilage and smooth muscle
- Dilatation of airways
- Chronic inflammation and fibrosis of wall
- Intraluminal purulent material
Cystic Fibrosis Pathogenesis
- Disorder of epithelial ion transport affecting fluid secretion in exocrine glands and epithelial linings of the respiratory tract, GI tract, and reproductive tract
- Abnormally viscid mucous secretions --> airway and the pancreatic duct blockages
- Leading to recurrent and chronic pulmonary infections and pancreatic insufficiency
- Most common (incidence 1:2500 live births) lethal genetic disease in whites (carrier freq 1:20)
- Classic biochemical abnormality: high sodium chloride level in sweat (sweat chloride test)
- Primary defect is reduced production (or abnormal function) of an epithelial chloride channel protein encoded by the CF transmembrane conductance regulator (CFTR) gene
- In sweat duct, result is increased sodium and chloride in sweat
- In epithelium of respiratory and GI tract, decreased chloride secretion with increased sodium and water reabsorption in airways results in dehydrated mucus layer, defective mucociliary action, and mucous plugging
- Cystic fibrosis affects several organs:
-
Pancreas: Mild to most severe
- Exocrine gland atrophy and fibrosis
- Impaired fat absorption
- Vitamin A deficiency
- Small intestine: Mucus plugs --> meconium ileus
-
Pulmonary: Thick viscid mucous of submucosal glands --> sinusitis, mucous plugging of bronchioles --> bronchiectasis --> abscesses
- Staph aureus, Hemophilus influenzae, and Pseudomonas aeruginosa are the most common organisms with Burkholderia cepacia (formerly Pseudomonas cepacia) increasing
-
Pancreas: Mild to most severe
Chronic Bronchitis Types
- Simple chronic bronchitis: Productive cough but no physiologic evidence of airflow obstruction
- Chronic asthmatic bronchitis: Hyperreactive airways with intermittent bronchospasm and wheezing
-
Obstructive chronic bronchitis: Develop chronic airflow obstruction
- Usually have associated emphysema
Pathogenesis of Chronic Bronchitis
- Hallmark is hypersecretion of mucus beginning in large airways
- Most important cause is cigarette smoking, followed by other environmental pollutants
-
Histologic findings:
-
Mucus gland hyperplasia: Increased size of glands
- Reid index = ratio of thickness of mucous gland layer to the thickness of the wall between the epithelium and the cartilage (NL = 0.4)
- Goblet cell metaplasia: Often seen
- Inflammatory infiltration of bronchiolar walls: Lymphocytes, macrophages, neutrophils, no eosinophils
- Fibrosis of the bronchiolar wall: Older patients
- May see squamous metaplasia or dysplasia: Especially with smoking
-
Mucus gland hyperplasia: Increased size of glands
Evolution of Chronic Bronchitis and Emphysema
- Both chronic bronchitis and emphysema can result from different pathways
- Either one can predominate
Chronic Bronchitis: Histopathology
- Chronic inflammation
Reid Index
- Ratio of thickness of submucosal mucus gland layer to thickness of bronchial wall between epithelium and cartilage
- Normally = 0.4 or less
- Increased (>0.5) in chronic bronchitis proportionally to the duration and severity of disease
Asthma
- A chronic disorder of the airways
- Usually an immunologic reaction marked by:
- Episodic bronchoconstriction due to increased airway sensitivity to varied stimuli
- Inflammation of bronchial walls
- Increased mucus secretion
- Presents with recurrent episodes of wheezing, breathlessness, chest tightness, and cough, especially at night and/or in the early morning
- Symptoms are usually associated with:
- Exposure to allergens (pollen, dust mites, animal dander, molds)
- Viral infections (respiratory syncytial virus, rhinovirus)
- Irritants (cigarette smoke, air pollution, cold air, exercise)
- Drugs (aspirin, NSAIDs)
Asthma - Diagnosis
- Clinical history and examination: History of recurrent wheezing and shortness of breath
- Spirometry: To assess airflow obstruction
- Peak expiratory flow rate: Measures the maximum speed of air that can be forcibly exhaled
- Methacholine challenge test: Used to assess airway hyperresponsiveness
- Allergy testing: Skin prick test, blood tests
- Chest x-ray: To rule out other lung diseases
- Blood tests: To measure eosinophil count and other inflammatory markers
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Description
Test your knowledge on respiratory pathology, focusing on Papanicolaou stain, Bronchiectasis, and related conditions. Explore details about the characteristics, predisposing factors, and pathogenesis of common respiratory diseases.
