Lecture 22: Bronchiectasis, Asthma and Vasculitis

Question 1

what is the bronchiestasis?

Answer 1

Abnormal irreversible dilatation of the bronchi caused by destruction of the muscle and elastic tissue

Question 2

bronchiectasis is an obstructive or restrictive disease?

Answer 2

obstructive

Question 3

what is the pathophysiology of bronchiectasis?

Answer 3

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

Question 4

list 2 important causes of upper lobe bronchiectasis

Answer 4

CF and TB

Question 5

what are the causes of lung central zone bronchiectasis?

Answer 5

CF,ABPA, Congenital tracheobronchomegally

Question 6

what are the causes of the lower lobe zone bronchiectasis?

Answer 6

• -childhood infections
• -aspirations
• -immunodeficiency

Question 7

what infections can cause bronchiectasis?

Answer 7

• -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

Question 8

what congenital conditions can cause bronchiectasis?

Answer 8

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

Question 9

what is the ABPA?

Answer 9

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.

Question 10

what is primary ciliary dyskinesia?

Answer 10

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.

Question 11

what is Young’s syndrome?

Answer 11

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.

Question 12

Kartagener syndrome vs Primary ciliary dyskinesia?

Answer 12

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.

Question 13

how immunodeficiency syndromes lead to bronchiectasis?

Answer 13

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

Question 14

what are the causes of bronchial obstruction that can lead to bronchiectasis?

Answer 14

• -Inhaled / aspirated foreign bodies
• -Tumour
• -Mucus plugs in asthma
• -Compressive lymphadenopathy
• -Chronic aspiration

Question 15

which rheumatic conditions increase the risk of bronchiectasis?

Answer 15

• -Sjogrens Syndrome
• -Systemic Lupus Erythematosus
• -Rheumatoid arthritis
• -Also associated with inflammatory bowel disease especially ulcerative colitis

Question 16

which side, left or right is commonly involved in bronchiectasis?

Answer 16

• -Lower lobes; left > right

- -Often bilateral

Question 17

Focal and Diffuse Presentations of bronchiectasis?

Answer 17

• -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

Question 18

inflammation and impaired mucociliary clearance lead to the release of what substance that results in lung destruction?

Answer 18

elastase
trypsin
viscous DNA (from dead cells,  bacteria, and leukocytes)

Question 19

which substances fight destructive proteases to prevent lung damage?

Answer 19

• -elastase inhibitor
• -Alpha-1-antitrypsin
• -Dornase alpha

Question 20

which microbes commonly colonize lungs in bronchiectasis?

Answer 20

• -P.aeruginosa
• -S aureus
• -H. influenzae
• -Burkholderia cepacia

Question 21

bronchiectasis histologically is characterized by…

Answer 21

• -increased mucos exuates
• -cartilage destruction and fibrosis
• -mucous glan hyperplasia
• -inflammatory cell infiltration

Question 22

what are the signs and symptoms of bronchiectasis?

Answer 22

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

Question 23

what are the auscultating findings of bronchiectasis?

Answer 23

• -Crackles and rhonchi
• -Wheezing (due to obstruction from secretions, airway collapsibility, or a concomitant condition)
• -Bronchophony

Question 24

what is the cause of massive hemoptysis in bronchiectasis?

Answer 24

Results from the rupture of dilated bronchial wall vessels (not pulmonary vessels)

Question 25

Bronchiectasis should be suspected in a patient with chronic cough producing large amounts of sputum. T/F

Answer 25

True

Question 26

what are the P/E findings of a patient with bronchiectasis?

Answer 26

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

Question 27

what are the complications of bronchiectasis?

Answer 27

- -Massive hemoptysis - -Respiratory failure - -Brain abscess - -Amyloidosis - -Recurrent bronchopulmonary infections → obstructive ventilation disorder → respiratory failure and cor pulmonale

Question 28

what investigations should be performed in suspected bronchiectasis?

Answer 28

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

Question 29

what are the characteristic imaging findings of bronchiectasis?

Answer 29

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

Question 30

in bronchiectasis, PFT is characterized by...

Answer 30

Pulmonary function tests: findings consistent with obstructive pulmonary disease (i.e. ↓ FEV1/FVC ratio)

Question 31

what is the indication of bronchoscopy in bronchiectasis?

Answer 31

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

Question 32

Pathological Classification of Bronchiectasis:

Answer 32

- Cylindrical - Varicose - Cystic

Question 33

what is the treatment of bronchiectasis?

Answer 33

- -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)

Question 34

what is the Dornase alpha (nebulizedDNAase)

Answer 34

A recombinant form of DNAse that breaks down extracellular DNA in sputum, thereby reducing its viscosity, and increasing mucociliary clearance. Used in cystic fibrosis.

Question 35

what invasive procedures can be performed n bronchiectasis?

Answer 35

- -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.

Question 36

define pleural effusion

Answer 36

- -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

Question 37

Pleural effusions are classified as:

Answer 37

- -TRANSUDATE | - -EXUDATE

Question 38

Transudate versus Exudate

Answer 38

- -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

Question 39

what is the transudate?

Answer 39

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).

Question 40

what is the exudate?

Answer 40

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).

Question 41

what is the pathophysiology of pleural effusion?

Answer 41

- -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

Question 42

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

Answer 42

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

Question 43

what are the clinical features of pleural effusion?

Answer 43

- -Dyspnoea - -Pleuritic chest pain - -Cough, haemoptysis - -Weight loss - -Trauma - -History of other malignancy or cardiac surgery

Question 44

what are the P/E findings of pleural effusion?

Answer 44

- -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

Question 45

tactile fremitus is increased over the pleural effusion. T/F

Answer 45

False | decrased. It is increased in consolidation (pneumonia)

Question 46

what is the management of pleural effusion?

Answer 46

- -CXR - -+/- CT Thorax - -Blood tests - -Thoracocentesis

Question 47

what is the thoracocentesis?

Answer 47

A procedure performed to drain an accumulation of pleural fluid (pleural effusion) by inserting a needle into the pleural cavity. Ultrasonography is used to determine the location and size of the pleural effusion. Indications include drainage for symptom relief (e.g., dyspnea) and fluid analysis for diagnosis of the underlying cause (e.g., infection, malignancy).

Question 48

how thoracocentesis is perofmred?

Answer 48

- -Indicated if effusion persists > 3 days | - -Performed at the bedside; but may require radiological guidance, particularly if small or loculated

Question 49

what is looked for in pleural fluid obtained during thoracocentesis?

Answer 49

--Gross appearance of fluid helpful 1)Bloody, 2)Cloudy 3)Malodorous (Usually thin and yellow) --Ratio of pleural fluid protein to serum protein measured --Glucose, LDH, +/- amylase --Cytology: inflammatory cells, malignant cells --Culture & Sensitivity

Question 50

what are the causes of transudative pleural effusion?

Answer 50

- -CHF - -cirrhosis - -nephrotic syndrome - -hypoalbuminemia - -myxedema/hypothyroidism - -peritoneal dialysis - -glomerulonephritis - -SVC obstruction - -post cardiac surgery - -post MI - -Meigs syndrome -ovarian tumour plus effusion

Question 51

what are the causes of exudative pleural effusion?

Answer 51

- -Infection (parapneumonic effusion, empyema) - -Bronchiectasis - -Lung abscess - -TB - -Neoplasms (primary, metastatic) - -lymphoma - -Granulomatous disorders - -Pulmonary embolism /infarction - -Connective tissue diseases (SLE, RA) - -Acute rheumatic fever

Question 52

the physical appearance of transudate vs exudate?

Answer 52

1) Does not froth or form clots 2) Cloudy or straw-colored fluid (may rarely be hemorrhagic): Whether an effusion is exudative cannot be determined based on color alone. - -Froths when shook and forms clots when left standing

Question 53

Specific gravity of transudate vs exudate?

Answer 53

≤ 1.016 | > 1.016

Question 54

pH of transudate vs exudate?

Answer 54

1) 7.4–7.55 2) 7.3–7.45 - -A pH < 7.30 should raise concern for parapneumonic effusion/empyema or malignant effusion.

Question 55

glucose of transudate vs exudate?

Answer 55

1) ≥ 60 mg/dL 2) < 60 mg/dL - -30–59 mg/dL: suggests malignant effusion, tuberculous pleurisy, empyema, pneumonia, esophageal rupture, or lupus pleuritis - -< 30 mg/dL: suggests rheumatoid pleurisy or empyema

Question 56

Total protein of transudate vs exudate?

Answer 56

≤ 30 g/L | > 30 g/L

Question 57

what is the light's criteria?

Answer 57

A validated measure that determines if pleural fluid is exudative. One of the following has to be present: pleural fluid protein/serum protein ratio greater than 0.5; pleural fluid LDH/serum LDH > 0.6; or pleural fluid LDH > 2/3 the upper limit of serum LDH reference range.

Question 58

define the asthma

Answer 58

- -Episodic, reversible bronchospasm (airflow obstruction) resulting from exaggerated bronchoconstriction in response to various stimuli - -Chronic inflammatory disorder of the airways - -Recurrent episodes of wheezing, breathlessness, chest tightness and coughing - -Heterogeneous disease triggered by a wide variety of inciting agents

Question 59

what is the epidemiology of asthma?

Answer 59

--300 million people worldwide --10-15% of children, 7-10% of adults --Young children commonest; boys>girls --Adolescents least common, higher frequency in adults Childhood asthma has a good prognosis

Question 60

what is the pathophysiology of asthma?

Answer 60

1) Inflammation of the airways 2) The smooth muscle surrounding airway becomes oedematous and tightens airway 3) Reduces the amount of air that can pass through leading to wheeze 4) Airway epithelium becomes oedematous, erythematous and inflamed 5) Inflammatory mediators include: - -Type 2 T helper lymphocytes, IL-4, IL-5, IL-13 - -Eosinophils, IgE, Mast cells, histamine, leukotrienes

Question 61

which inflammatory mediators are involved in the pathophysiology of asthma?

Answer 61

- -Type 2 T helper lymphocytes, IL-4, IL-5, IL-13 | - -Eosinophils, IgE, Mast cells, histamine, leukotrienes

Question 62

what is the role of IL-4?

Answer 62

A cytokine produced by Th2 cells that promotes differentiation of naive CD4+ T cells to produce additional Th2 cells (positive feedback). Also induces class switching of B-cells from IgM-producing to IgG- and IgE-producing subtypes.

Question 63

what is the role of IL-5?

Answer 63

A cytokine secreted by mast cells that stimulates eosinophil proliferation and activation. Also secreted by T cells to promote B cell differentiation and class switching to IgA.

Question 64

what is the role of IL-13?

Answer 64

A cytokine that is primarily produced by Th2 CD4 cells. It activates the alternative macrophage activation pathway and acts on B cells to induce isotype switching to IgE

Question 65

what are the symptoms of asthma?

Answer 65

- -Episodic SOB - -Wheeze - -Chest tightness - -Cough - -+/- sputum production - -Generally worse in morning - -History: elicit triggers

Question 66

Endobronchial obstruction caused by...

Answer 66

- -Bronchospasm - -Mucosal edema - -Hypertrophy of smooth muscle cells - -Increased mucus production

Question 67

allergic vs non-allergic asthma?

Answer 67

1) Allergic asthma: IgE-mediated type 1 hypersensitivity to a specific allergen; characterized by mast cell degranulation and release of histamine after a prior phase of sensitization 2) Nonallergic asthma - -Irritant asthma: irritant enters lung → ↑ release of neutrophils → submucosal edema → airway obstruction - -Aspirin-induced asthma: NSAID inhibition of COX-1 → ↓ PGE2 → ↑ leukotrienes and inflammation → submucosal edema → airway obstruction

Question 68

what are the causative factors of asthma?

Answer 68

1) Allergic asthma (extrinsic asthma) - -Cardinal risk factor: atopy - -Environmental allergens: pollen (seasonal), dust mites, domestic animals (The triggering allergens are proteins present in dander and saliva. Proteins in saliva are transferred to animal hair and feathers through grooming.), mold spores - -Allergic occupational asthma: from exposure to allergens in the workplace (e.g., flour dust): Baker's asthma is one of the most common forms of occupational disease. 2) Nonallergic asthma (intrinsic asthma) - -Viral respiratory tract infections (one of the most common stimuli, especially in children) - -Cold air - -Physical exertion (exercise-induced asthma) - -Gastroesophageal reflux disease (GERD): often exists concurrently with asthma - -Chronic sinusitis or rhinitis - -Medication: aspirin/NSAIDS (aspirin-induced asthma), beta-blockers - -Stress - -Irritant-induced asthma (e.g., from exposure to solvents, ozone, tobacco or wood smoke, cleaning agents)

Question 69

what are the extrinsic factors that cause asthma?

Answer 69

1) Antigen-induced asthma - -Commonest type (30-50%) - -Airborne allergens - pollens, animal hair or fur, insect contamination of house dust mite 2) Occupation Related - -80 different substances identified, - - e.g. animal handlers, woodworkers, fumes from hardening agents (resin ), etc 3) Environmental pollution - -Sulphur dioxide, ozone 4) Drug-induced: - -Aspirin - -Samter’s triad: Aspirin sensitivity, asthma, and nasal polyps - -NSAIDs - -Antibiotics - -Beta-blockers - -ACE inhibitors 5) Viral respiratory tract infections - -Children - Respiratory syncytial virus, influenza, rhinovirus 5) Exercise - in patients who already have asthma - -Heat loss from the epithelium - -Severe exercise and cold air increase risk

Question 70

what drugs can cause asthmatic symptoms and bronchospasm?

Answer 70

- -Aspirin (Samter’s triad: Aspirin sensitivity, asthma, and nasal polyps) - -NSAIDs - -Antibiotics - -Beta-blockers - -ACE inhibitors

Question 71

what is the Samter's triad?

Answer 71

Bronchial asthma and/or chronic rhinosinusitis with polyposis in patients with hypersensitivity to aspirin or other cyclooxygenase-1 inhibitors.

Question 72

what is an aspirin-exacerbated respiratory disease (AERD)?

Answer 72

- -Definition: a chronic condition characterized by Samter's triad; exacerbated by a pseudoallergic sensitivity reaction to aspirin and other NSAIDs (NSAID intolerance) - -Clinical features: Samter's triad (asthma, chronic/recurrent rhinosinusitis, nasal polyps) - -Diagnosis: NSAID/aspirin challenge test - -Treatment: avoid NSAIDs; aspirin desensitization

Question 73

what is the presentation of AERD?

Answer 73

- -Patients present with a history of chronic upper/lower respiratory symptoms and episodes of NSAID-triggered hypersensitivity reaction (bronchospasm) within a few hours of consuming an NSAID. AERD is not an immune-mediated (IgE) allergic response, but a pseudoallergic reaction. - -Patients present with shortness of breath due to bronchoconstriction, malaise, weakness, fatigue, and/or skin flushing/rash after ingestion of aspirin.

Question 74

what is exercise-induced asthma?

Answer 74

Asthma in which bronchoconstriction is triggered by physical exertion. Treated by short-acting inhaled β-agonists shortly before exercise and reduction of potential environmental triggers (cold air, allergens).

Question 75

what is intrinsic asthma (non-allergic) asthma

Answer 75

- -Adults - -No individual or family history of atopic disease - -Skin/inhalation tests negative - -Nasal Polyps - -Drug hypersensitivity to aspirin, - -Penicillin - -Often associated with COPD - -Prognosis less good

Question 76

what are the microscopical features of asthma?

Answer 76

- -Airway infiltration by neutrophils and eosinophils - -Mast cell degranulation - -Basement membrane thickening - -Loss of epithelial integrity - -Occlusion of the bronchial lumen by mucus - -Hyperplasia and hypertrophy of bronchial smooth muscle and goblet cells

Question 77

what is the natural history of asthma?

Answer 77

- -In the majority of patients it is not progressive (contrast with chronic bronchitis, cystic fibrosis, bronchiectasis) - -Some (minority) patients do develop irreversible changes in lung function - -Clinical course is characterized by remissions and exacerbations

Question 78

what is the pulmonary thromboembolism?

Answer 78

Pulmonary embolism is a complication arising from deep vein thrombosis that results in a blood clot blocking the pulmonary artery or its branches

Question 79

what are the sources of PE?

Answer 79

- ->95% arise from thrombi in deep veins of lower legs (popliteal vein or above) - -Thromboemboli do not usually arise from superficial or smaller leg veins

Question 80

Pulmonary thromboembolism can arise from leg superficial veins. T/F

Answer 80

False | Only from deep veins

Question 81

what is the pathophysiology of PE?

Answer 81

thrombus formation → deep vein thrombosis in the legs or pelvis (most commonly iliac vein) → embolization to pulmonary arteries via inferior vena cava → partial or complete obstruction of pulmonary arteries

Question 82

what is the pathophysiologic response of the lung to arterial obstruction

Answer 82

1) Infarction and inflammation of the lungs and pleura) - -Causes pleuritic chest pain and hemoptysis - -Leads to surfactant dysfunction → atelectasis → ↓ PaO2 - -Triggers respiratory drive → hyperventilation and tachypnea → respiratory alkalosis with hypocapnia (↓ PaCO2) 2) Impaired gas exchange - -Mechanical vessel obstruction → ventilation-perfusion mismatch → arterial hypoxemia (↓ PaO2) and elevated A-a gradient (see “Diagnostics” below) 3) Cardiac compromise - -Elevated pulmonary artery pressure (PAP) due to blockage → right ventricular pressure overload → forward failure with decreased cardiac output → hypotension and tachycardia

Question 83

what are the risk factors of PE and DVT?

Answer 83

1) Immobilization: - -Prolonged bed rest - -Surgery 2) Severe trauma 3) CCF 4) Pregnancy 5) OCP 6) Malignancy 7) Hypercoagulable states: - -Factor V Leiden mutation - -Protein C, Protein S deficiency - -Antithrombin III deficiency - -Lupus anticoagulant - -Homocysteinuria 8) Connective tissue disease

Question 84

what is the hypercoagulability state?

Answer 84

An inherited or acquired state in which an individual is predisposed to forming blood clots. This most often manifests as recurrent deep vein thromboses or pulmonary emboli. Etiologies include conditions of inherited thrombophilia (e.g., factor V Leiden mutation), malignancy, pregnancy and the puerperium, various drugs (e.g., oral contraceptives, tamoxifen), and immobilization.

Question 85

what are the causes of circulatory stasis?

Answer 85

- -Afib - -LVD - -immobility or paralysis - -venous insufficiency and varicose veins - -venous obstruction from tumor, obesity or pregnancy

Question 86

what are the causes of the vascular wall injury

Answer 86

- -trauma/surgery - -venipuncture - -chemical irritation - -heart valve disease of replacement - -atherosclerosis - -catheters

Question 87

what are the consequences of the obstructed artery?

Answer 87

1) Depends on the size of the embolism, which in turn dictates the size of the occluded pulmonary artery 2) Consequences: - -Increase in pulmonary artery pressure from blockage of flow and vasospasm caused by neurogenic mechanisms and release of mediators - -Ischaemia of downstream pulmonary parenchyma - -Occlusion of a major vessel causes a sudden increase in pulmonary artery pressure, diminished cardiac output, acute cor pulmonale and possibly sudden death - -Occlusion of a smaller vessel is less catastrophic and may even be clinically silent

Question 88

PE is usually silent (60-80%). T/F

Answer 88

True. - -Small and embolic mass is removed by fibrinolytic activity - -Bronchial circulation sustains the viability of affected lung parenchyma

Question 89

what is the cause of sudden cardiac death in PE?

Answer 89

- -Acute cor pulmonale, cardiovascular collapse when >60% of the vasculature os obstructed - -death due to PEA

Question 90

Obstruction of small to medium pulmonary branches (10-15%) results in...

Answer 90

pulmonary infarction; patients complain of dyspnoea, hemoptysis, pleuritic chest pain

Question 91

Recurrent PE may lead to...

Answer 91

pulmonary hypertension, chronic right sided heart stain (chronic cor pulmonale, pulmonary vascular sclerosis

Question 92

what is the classic presentation of PE?

Answer 92

- -Sudden onset pleuritic chest pain - -SOB - -Hypoxia - -Syncope, seizures - -Decreased level of consciousness - -New-onset atrial fibrillation - -hemoptysis

Question 93

infarction in PE is usually central. T/F

Answer 93

False - -Usually peripheral - -Wedge-shaped with the base at the pleural surface - -Hemorrhage and appear as raised red-blue areas in early stage - -Adjacent pleural covered in a fibrinous exudate; can cause pleuritic chest pain and effusion

Question 94

what investigations should be performed in suspected PE?

Answer 94

1) History and physical examination - -Assess risk - -Examine lower limbs for DVT - -CVS: S3, S4 gallop, Loud P2 2) Bloods - -D-Dimers - -ABG 3) Radiology - -VQ scan - -CT pulmonary angiogram(PE protocol) - -Duplex ultrasonography (DVT) - -ECHO (central PE) 4) ECG - -Tachycardia (>100/min) - -S1Q3T3

Question 95

what are the ECG characteristics of PE?

Answer 95

- -SIQIIITIII -pattern (S wave in lead I, Q wave in lead III and T-wave inversion in lead III. However, this characteristic pattern is rare.) - -New right bundle branch block - -Bradycardia < 50 or tachycardia > 100 bpm - -Right or extreme right axis deviation (30% of cases) - -T negativity in leads V2and V3 (∼ 30%)

Question 96

what is the management of PE?

Answer 96

1) Oxygen 2) Pain relief 3) Thrombolytic therapy 4) Long term anticoagulation 5) Surgical options - -Embolectomy 6) Prevention - -Mobilization - -Anticoagulation - -Vena cava filters - -stockings 7) Investigate for the underlying cause

Question 97

examples of non-thrombotic emboli

Answer 97

- -Air embolism - -Fat embolism - -Amniotic fluid embolism - -Foreign body embolism - -Bone marrow embolism

Question 98

what is the diffuse alveolar hemorrhage?

Answer 98

1) Primary immune-mediated diseases 2) Present as a triad - -Haemoptysis - -Anaemia - -Diffuse pulmonary infiltrates

Question 99

what are the causes of diffuse alveolar hemorrhage?

Answer 99

1) Prototype: Goodpasture syndrome 2) Secondary causes: - -Necrotizing bacterial pneumonia - -Bleeding diathesis - -Passive venous congestion

Question 100

examples of vasculitis affecting lung?

Answer 100

- -Pulmonary angiitis and granulomatosis (GPA; aka Wegener granulomatosis) - -Churg-Strauss syndrome - -Collagen vascular disorders

Question 101

what is Goodpasture syndrome?

Answer 101

- -Rapidly progressive glomerulonephritis plus hemorrhagic interstitial pneumonitis - -Renal and pulmonary lesions are caused by antibodies to antigens common to glomerular and pulmonary basement membranes - -Example of type II cytotoxic antibody-mediated hypersensitivity - -Antibodies can be detected in the serum in >90% of patients (Anti glomerular basement membrane antibodies; also called anti-GBM disease) - -Lungs shows focal necrosis of alveolar walls associated with intra-alveolar hemorrhages, fibrous thickening of septae - -Hemosiderin laden macrophages are seen in the acute setting - -Management: Plasmapheresis and immunosuppressive therapy - -Plasma exchange removes offending antibodies and immunosuppressive drugs inhibit antibody production

Question 102

what is the pathophysiology of Goodpasture syndrome?

Answer 102

- -Auto-antibodies against collagen type IV in the renal and pulmonary capillary basement membrane - -Possibly triggered by viral infections, lymphoma

Question 103

Goodpasture syndrome is a type 2 or 3 HSR?

Answer 103

type 2 | Anti-GBM antibodies

Question 104

what is the Granulomatosis with polyangiitis?

Answer 104

1) Multisystem autoimmune disorder of unknown etiology 2) Hallmark: - -Pauci-immune vasculitis of small to medium-sized vessels - -Necrotising granulomatous inflammation 3) A spectrum of clinical presentations 4) Diagnosis - -Bloods & urinalysis * FBC, U&E, ESR, CRP - -Antineutrophil Cytoplasmic antibody (ANCA) * Cytoplasmic ANCA (c-ANCA) directed against PR3 is most specific for GPA * Some patients with GPA express perinuclear staining ANCA (p-ANCA) specific for myeloperoxidase (MPO) - -Radiology * CXR, CT Thorax (nodular densities, may cavitate), CT sinus - -Biopsy (only in an atypical presentation) * Lungs: necrotizing vasculitis, parenchymal necrotizing granulomatous inflammation * Vessels: acute and chronic inflammation with fibrinoid necrosis of vessel wall 5) Management - -Cyclophosphamide plus glucocorticosteroids - -Steroids plus Rituximab - -Plasma exchange for severe renal disease - -Maintenance therapy in remission

Question 105

what is the hallmark of GPA?

Answer 105

- -Pauci-immune vasculitis of small to medium sized vessels | - -Necrotising granulomatous inflammation

Question 106

GPA is characterized by the presence of p-ANCA or c-ANCA?

Answer 106

``` c-ANCA Cytoplasmic ANCA (c-ANCA) directed against PR3 is most specific for GPA ```

Question 107

what are the characteristic findings on biopsy of GPA?

Answer 107

* Lungs: necrotizing vasculitis, parenchymal necrotizing granulomatous inflammation * Vessels: acute and chronic inflammation with fibrinoid necrosis of vessel wall

Question 108

GPA is treated with immunosuppressive agents. True/False

Answer 108

True - -Cyclophosphamide plus glucocorticosteroids - -Steroids plus Rituximab - -Plasma exchange for severe renal disease - -Maintenance therapy in remission

Question 109

what are the clinical features of GPA?

Answer 109

1) Recurrent RTI’s, fever, night sweats, fatigue, lethargy, weight loss, anorexia 2) Ophthalmic - -Conjunctivitis, episcleritis 3) ENT - -Chronic sinusitis, rhinitis, epistaxis 4) Pulmonary - -Pulmonary infiltrates, cough, hemoptysis, dyspnoea, diffuse alveolar hemorrhage due to alveolar capillaritis 5) Musculoskeletal - -Myalgia, arthralgia 6) Renal - -Crescentic necrotizing glomerulonephritis 7) CNS 8) Cutaneous - -Vasculitic ulcers 9) Cardiac - -Pericardial rub

Question 110

what is the Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss Disease)?

Answer 110

- -Rare systemic necrotizing, granulomatous vasculitis, affects small to medium-sized vessels, associated with severe asthma and blood and tissue eosinophilia - -p-ANCA associated vasculitis (like microscopic polyangiitis)

Question 111

what are the diagnostic criteria of Churg-Strauss?

Answer 111

- -asthma - -eosinophilia>10% - -pulmonary infiltrates - -mono or polyneuropathy - -paranasal sinus abnormality - -extravascular eosinophils on biopsy

Question 112

what is the clinical presentation of Churg0Strauss?

Answer 112

1) Constitutional symptoms - -Malaise, fever, weight loss, myalgia 2) Asthma 3) Paranasal sinusitis 4) Allergic rhinitis - -Recurrent sinusitis - -Polyposis 5) Pulmonary symptoms - -Cough, hemoptysis 6) Arthralgia 7) Skin - -Purpura, bullae, nodules, digital ischemia 8) Cardiac - -Myocarditis, pericarditis, MI 9) GIT - -Eosinophilic gastroenteritis 10) Peripheral neuropathy - -Mononeuritis multiplex

Question 113

what investigations should be performed in suspected CHurg-Strauss?

Answer 113

- -Bloods: ESR, CRP, U&E, FBC (eosinophilia), IgE, rheumatoid factor positive (low titer), p-ANCA + - -Bronchoscopy: Lavage fluid shows raised eosinophils - -Radiology: CXR, CT thorax - -GI investigations (bleeding) - -EMG (nerve damage) - -Biopsy (depend on the site of involvement eg skin biopsy)

Question 114

cyclophosphamide is the first-line treatment of Churg-Strauss. T/F

Answer 114

False | Steroid; less frequently require cyclophosphamide, Rituximab

Question 115

what is the mononeuritis multiplex?

Answer 115

An asymmetrical sensory and/or motor peripheral neuropathy involving damage to ≥ 2 peripheral nerves in separate areas of the body. Common causes include diabetic microangiopathy, medium-vessel vasculitis, certain granulomatous diseases (e.g., sarcoidosis), amyloidosis, and certain infections (e.g., Lyme disease, leprosy, HIV).

Question 116

examples of collagen vascular disorders that can affect the lungs?

Answer 116

SLE, rheumatoid arthritis, scleroderma, dermatomyositis-polymyositis

Question 117

what are the manifestations of collagen disorders affecting the lungs?

Answer 117

- -Interstitial pneumonia -> diffuse interstitial fibrosis - -Pulmonary hypertension - -Pulmonary vasculitis - -Diffuse alveolar hemorrhage - -Pleuritis