Lecture 22: Bronchiectasis, Asthma and Vasculitis Flashcards
what is the bronchiestasis?
Abnormal irreversible dilatation of the bronchi caused by destruction of the muscle and elastic tissue
bronchiectasis is an obstructive or restrictive disease?
obstructive
what is the pathophysiology of bronchiectasis?
1) The induction of bronchiectasis requires two factors:
- -An infectious insult
- -Impairment of drainage, airway obstruction
2) Involved bronchi are dilated, inflamed and easily collapsible, resulting in airflow obstruction and impaired clearance of secretions
list 2 important causes of upper lobe bronchiectasis
CF and TB
what are the causes of lung central zone bronchiectasis?
CF,ABPA, Congenital tracheobronchomegally
what are the causes of the lower lobe zone bronchiectasis?
- -childhood infections
- -aspirations
- -immunodeficiency
what infections can cause bronchiectasis?
- -Sequel to bronchopneumonia and bronchiolitis in childhood which has imperfectly resolved
- -Pneumonia complicating measles or whooping cough
- -Allergic Bronchopulmonary Aspergillosis (ABPA)
- -Chronic tuberculous cavities
- -Primary Mycobacterium avium complex infection
what congenital conditions can cause bronchiectasis?
1) Primary ciliary dyskinesia
- -Poorly functioning cilia contribute to retention of secretions and recurrent infections
2) Alpha-1-antitrypsin deficiency
3) Cystic Fibrosis
4) Youngs syndrome
- -Chronic sinopulmonary infections
5) Marfan syndrome
6) Kartageners Syndrome
- -Characterised by a triad of dextrocardia, bronchiectasis and severe sinusitis.
- -Autosomal recessively inherited condition
- -Affects the mobility of the cilia
what is the ABPA?
Chronic exposure to Aspergillus can result in ABPA, presenting with asthmatic symptoms or sinusitis, especially in patients with a history of asthma or cystic fibrosis. It is primarily managed with glucocorticoid therapy.
what is primary ciliary dyskinesia?
A rare, autosomal recessive disorder caused by dysfunctional or absent cilia (e.g., due to mutations in the dynein arm or assembly proteins). Clinical features include recurrent otitis, sinusitis, chronic productive cough, and situs inversus. Fertility is usually reduced due to decreased sperm motility or dysfunctional fallopian tube cilia.
what is Young’s syndrome?
Young syndrome is a condition characterized by male infertility, damaged airways in the lungs (bronchiectasis), and inflammation of the sinuses (sinusitis). … Although the exact cause of Young syndrome has not been identified, it is believed to either be related to childhood exposure to mercury or genetic factors.
Kartagener syndrome vs Primary ciliary dyskinesia?
Kartagener is a subset of primary ciliary dyskinesia characterized by the triad of situs inversus, chronic sinusitis, and bronchiectasis. Additional clinical features of primary ciliary dyskinesia can also be present, such as reduced fertility, chronic ear infections, and conductive hearing loss.
how immunodeficiency syndromes lead to bronchiectasis?
Yes, due to increased rate of respiratory infections
- -Persons with humoral immunodeficiency syndromes involving deficiencies of IgG, IgM and IgA (i.e Hypogammaglobulinaemia)
- -Immunodeficiency due to malignancy eg myeloma, lymphoma
- -Immunoglobulin replacement reduces the frequency of infections and prevents ongoing airway destruction
what are the causes of bronchial obstruction that can lead to bronchiectasis?
- -Inhaled / aspirated foreign bodies
- -Tumour
- -Mucus plugs in asthma
- -Compressive lymphadenopathy
- -Chronic aspiration
which rheumatic conditions increase the risk of bronchiectasis?
- -Sjogrens Syndrome
- -Systemic Lupus Erythematosus
- -Rheumatoid arthritis
- -Also associated with inflammatory bowel disease especially ulcerative colitis
which side, left or right is commonly involved in bronchiectasis?
- -Lower lobes; left > right
- -Often bilateral
Focal and Diffuse Presentations of bronchiectasis?
- -Lower lobes; left > right
- -Often bilateral
- -Bronchi are dilated and contain thick mucopurulent secretions
- -The wall is destroyed with chronic inflammation and lymphoid follicles
- -Bronchial arteries are increased in size
inflammation and impaired mucociliary clearance lead to the release of what substance that results in lung destruction?
elastase trypsin viscous DNA (from dead cells, bacteria, and leukocytes)
which substances fight destructive proteases to prevent lung damage?
- -elastase inhibitor
- -Alpha-1-antitrypsin
- -Dornase alpha
which microbes commonly colonize lungs in bronchiectasis?
- -P.aeruginosa
- -S aureus
- -H. influenzae
- -Burkholderia cepacia
bronchiectasis histologically is characterized by…
- -increased mucos exuates
- -cartilage destruction and fibrosis
- -mucous glan hyperplasia
- -inflammatory cell infiltration
what are the signs and symptoms of bronchiectasis?
Productive cough with purulent sputum
- -Sputum may be mucoid, mucopurulent, thick or viscous
- -Blood streaked sputum or copious hemoptysis may result from erosive airway damage due to acute infection
- -Dyspnoea and wheezing in 75%
- -Pleuritic pain in 50%
- -Recurrent LRTI
what are the auscultating findings of bronchiectasis?
- -Crackles and rhonchi
- -Wheezing (due to obstruction from secretions, airway collapsibility, or a concomitant condition)
- -Bronchophony
what is the cause of massive hemoptysis in bronchiectasis?
Results from the rupture of dilated bronchial wall vessels (not pulmonary vessels)
Bronchiectasis should be suspected in a patient with chronic cough producing large amounts of sputum. T/F
True
what are the P/E findings of a patient with bronchiectasis?
1) Crackles, coarse crepitations, and rhonchi on auscultation
2) Clubbing a common finding in the past, rare now 3%
3) The major confounding disease is COPD
what are the complications of bronchiectasis?
- -Massive hemoptysis
- -Respiratory failure
- -Brain abscess
- -Amyloidosis
- -Recurrent bronchopulmonary infections → obstructive ventilation disorder → respiratory failure and cor pulmonale
what investigations should be performed in suspected bronchiectasis?
1) Bloods
- -FBC, U&E
- -Exclude / confirm aetiologies eg autoantibody screen, sweat test, serum immunoglobulins, IgE, alpha-1-AT levels, etc
2) Microbiology
- -Sputum C&S
- -Blood cultures if clinically indicated
- -Exclude infectious aetiologies (eg TB, aspergillus, etc)
3) Radiology
- -CXR
- -CT Thorax
4) Bronchoscopy
- -Obstruction
- -Culture and cytology of washings
5) PFT’s
what are the characteristic imaging findings of bronchiectasis?
1) CXR
- -Inflammation and fibrosis of bronchial walls lead to the appearance of parallel “tram track” lines
- -Thin-walled cysts (i.e., dilated bronchi forming sacs), possibly with air-fluid levels
- -Late-stage bronchiectasis: honeycombing
2) 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
in bronchiectasis, PFT is characterized by…
Pulmonary function tests: findings consistent with obstructive pulmonary disease (i.e. ↓ FEV1/FVC ratio)
what is the indication of bronchoscopy in bronchiectasis?
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
Pathological Classification of Bronchiectasis:
- Cylindrical
- Varicose
- Cystic
what is the treatment of bronchiectasis?
- -Confirm correct diagnosis
- -Treat infectious exacerbations
- -Reduce risk factors (smoking!!)
- -Vaccinations (including Influenza vaccine and pneumococcal vaccine)
- -Chest physiotherapy
- -Nebulised DNAase, Nebulised antibiotics
- -Maintenance antibiotics
- -Surgery (if focal)
what is the Dornase alpha (nebulizedDNAase)
A recombinant form of DNAse that breaks down extracellular DNA in sputum, thereby reducing its viscosity, and increasing mucociliary clearance. Used in cystic fibrosis.
what invasive procedures can be performed n bronchiectasis?
- -Surgical resection of bronchiectatic lung or lobectomy: indicated in pulmonary hemorrhage, inviability of bronchus, and substantial sputum production in unilateral bronchiectasis
- -Pulmonary artery embolization: indicated in pulmonary hemorrhage
- -Lung transplantation should be considered in severe disease.
define pleural effusion
- -Accumulation of fluid in the pleural space
- -Empyema: accumulation of infected fluid in the pleural space
- -Normally a small amount of thin, pale yellow fluid is present in the pleural space to allow movement of the visceral pleural against the parietal pleura
Pleural effusions are classified as:
- -TRANSUDATE
- -EXUDATE
Transudate versus Exudate
- -Transudates have low specific gravity, low protein and few cells
- -Causes include heart failure, fluid overload, nephrotic syndrome, Peritoneal dialysis
- -Exudates have high specific gravity, high protein and lots of cells
- -Causes include infection, malignancy, pulmonary emboli
what is the transudate?
A type of fluid that is low in proteins and cells and typically due to the permeation of fluid from intact vessels into a cavity. Causes include increased capillary hydrostatic pressure (e.g., pleural effusion in congestive heart failure) and/or decreased capillary oncotic pressure (e.g., edema due to lack of albumin).
what is the exudate?
A protein-rich fluid that occurs as a result of increased vascular permeability from inflammation. Analysis of the fluid reveals a cellular (cloudy) fluid with high protein and low glucose concentration. Can accumulate in cavities (e.g., pleural space, pericardial space).
what is the pathophysiology of pleural effusion?
- -Pleural fluid will accumulate when the rate of pleural fluid formation is greater than the rate of pleural fluid removal by the lymphatics
- -A transudative effusion occurs when alterations in the systemic factors that influence pleural fluid movement result in a pleural effusion. Examples are elevated visceral pleural capillary pressure with left heart failure, elevated parietal pleural capillary pressure with right heart failure, decreased serum oncotic pressure with nephrotic syndrome, hepatic cirrhosis
- -An exudative effusion occurs when the pleural surfaces are altered. Inflammation of the pleura, leading to increased protein in the pleural space is the most common cause of the exudative effusion
A transudative effusion occurs when the pleural surfaces are altered. Inflammation of the pleura, leading to increased protein in the pleural space is the most common cause of the exudative effusion. T/F
False
A transudative effusion occurs when alterations in the systemic factors that influence pleural fluid movement result in a pleural effusion. Examples are elevated visceral pleural capillary pressure with left heart failure, elevated parietal pleural capillary pressure with right heart failure, decreased serum oncotic pressure with nephrotic syndrome, hepatic cirrhosis
what are the clinical features of pleural effusion?
- -Dyspnoea
- -Pleuritic chest pain
- -Cough, haemoptysis
- -Weight loss
- -Trauma
- -History of other malignancy or cardiac surgery
what are the P/E findings of pleural effusion?
- -Decreased breath sounds over side of effusion
- -Bronchial breath sounds immediately above effusion
- -Dull to percussion
- -Absent / reduced tactile fremitus
- -Other findings (depending on underlying cause)
- Ascites, JVP, peripheral oedema, friction rub, unilateral leg swelling etc
tactile fremitus is increased over the pleural effusion. T/F
False
decrased. It is increased in consolidation (pneumonia)
what is the management of pleural effusion?
- -CXR
- -+/- CT Thorax
- -Blood tests
- -Thoracocentesis
