Obstructive Lung Diseases CIS Flashcards
40 yo male presents with gradual onset of dyspnea, cough, and wheezing over the last 2-3 years.
Family History is remarkable for a father deceased at age 53 with cirrhosis.
Social History: 20 pack year history of tobacco use
Physical Examination: decreased breath sounds to auscultation, lungs are hyperinflated by percussion, peripheral cyanosis and clubbing of the digits
Pulmonary Function Testing
FEV1 1.06 L 36% of predicted
FEV1/FVC 38%
No significant improvement with bronchodilator treatment
Pick the letter that best describes this patient’s disorder
A) Sweat testing confirms diagnosis
B) Purified protein derivative skin testing will confirm diagnosis
C) This disorder is caused by an imbalance in neutrophil elastase
D) Sputum cytology will confirm diagnosis
E) Acid fast sputum stain will confirm diagnosis
C) This disorder is caused by an imbalance in neutrophil elastase
Alpha-1 Antitrypsin Deficiency
Genetic risk factor for COPD
Etiology of 1-2% of COPD cases
Serine protease inhibitor secreted by the liver that protects the lung tissue against the action of neutrophil elastase and serine proteases.
Patients are very susceptible to damage from cigarette smoking.
Should be considered in young patients with COPD
Can lead to LFT abnormalities and cirrhosis
19 year old male presents to the emergency room in acute respiratory distress. He has had a productive cough for several days
Past Medical History: Frequent bouts of sinusitis, hospital admission 2 years ago with pneumonia, and meconium ileus at birth
Physical Examination: Oxygen Saturation 93% on 2L NC, nostril flaring, subcostal retractions, wheezes, rhonchi, and clubbing of the fingers
Pulmonary Function Testing with moderate airway obstruction.
Which statement is true regarding this disorder?
A) B Vitamin Malabsorption is common in this disorder.
B) 95% of males with this disorder are sterile.
C) This disease is characterized by non-caseating granulomas
D) This is an autosomal dominant disorder
E) All siblings will be gene carriers
95% of males with this disorder are sterile.
Cystic Fibrosis
Autosomal recessive disorder caused by mutation of the cystic fibrosis transmembrane conductance regulator protein.
Organs affected include lungs, pancreas, intestines, liver, sweat gland, sinuses, and the vas deferens.
Lung disease leads to death in 90% of patients.
Median survival 37 years
Lung Manifestations in CF
Cough, dyspnea, decreased exercise tolerance, fatigue, and increased sputum production
Steep decline in lung function at adolescence
Daily productive cough
Airway infection with Pseudomonas aeruginosa is the primary pathogen. Staphylococcus aureus, and methicillin resistant Staphylococcus aureus are also common.
Other infections include bronchopulmonary mycoses, and nontuberculous mycobacterial infections
Pancreatic Manifestations of CF
Exocrine pancreatic insufficiency can lead to impaired growth
Signs of malabsorption include bulky, foul smelling stools and flatulence.
Malabsorption of fat soluble vitamins occur.
Liver Manifestations of CF
Hepatomegaly
Splenomegaly
Hematemesis secondary to esophageal or gastric varices from portal hypertension
Sweat Gland in CF
Failure of chloride absorption from the lumen into the ductal lining cell.
Marked elevation of chloride and sodium in sweat
Vas deferens in CF
almost all male are sterile
Other clinical manifestations in CF
Endocrine Pancreas: 1/3 of patients have diabetes by age
30
Electrolyte abnormalities can lead to nausea/vomiting, decreased appetite, circulatory collapse, and seizures
Musculoskeletal: decreased bone density secondary to decreased absorption of vitamin D, glucocorticoid treatment, and decreased exercise.
Kidney: nephrolithiasis
Cystic Fibrosis Diagnosis
Screening with immunoreactive trypsinogen which is a marker of pancreatic injury
Genetic mutation analysis
Diagnosis confirmed with sweat testing
The sweat test measures chloride concentration in sweat that is stimulated by pilocarpine iontophoresis.
5% of diagnosis are made after the age of 18
Pulmonary function testing in CF
obstruction
Chest x-ray in CF
Hyperinflation
Bronchiectasis
Oral Antibiotics in CF
Azithromycin [25-40 kg] 250 mg PO every Mon, Wed, Fri; [> 40 kg] 500 mg PO every Mon, Wed, Fri
Patients ≥ 6 years of age with Pseudomonas aeruginosa meet criteria for use
Trimethoprim/sulfamethoxazole 20 mg/kg/day PO divided every 6-8 hours
Ciprofloxacin 40 mg/kg/day PO divided every 12 hours
IV antibiotics in CF
Aztreonam 200 mg/kg/day IV divided every 6-8 hours
Cefepime 150 mg/kg/day IV divided every 8 hours
Ceftazidime 200 mg/kg/day IV divided every 8 hours
Ciprofloxacin 30 mg/kg/day IV divided every 8-12 hours
Meropenem 120 mg/kg/day IV divided every 8 hours
Piperacillin/tazobactam 400 mg/kg/day divided every 4-6 hours
Tobramycin 7.5-15 mg/kg/day IV divided every 8-24 hours
[Higher dose, extended interval dosing may be more effective and less nephrotoxic]
Vancomycin 60 mg/kg/day divided IV every 6-8 hours
Inhaled antibiotics in CF
Tobramycin 300 mg inhaled twice daily in 28 day cycle
Aztreonam 75 mg inhaled three times daily in 28 day cycle
Other CF Treatments
Pancreatic enzymes, vitamin supplementation, bronchodilators, hypertonic saline inhalation, dornase alfa(rhDNase), ibuprofen, oxygen, bronchodilators, lung transplant
Chest percussion and postural drainage
55 year old male with a history of chronic obstructive pulmonary disease complains of increased shortness of breath, dyspnea with minimal exertion, and decreased exercise tolerance.
Past Medical History : Multiple hospital admissions for COPD exacerbations, intubated last admit
Social History: 40 pack year history of tobacco use
Medications: maximal doses of inhaled steroid, salmeterol, and tiotropium. He frequently has been treated with oral antibiotics and oral steroids.
Which of the following Pulmonary Function Test results are most likely in this patient?
A) Decreased FEV1/FVC
B) Decreased functional residual capacity
C) Decreased total lung capacity
D) Increased FEV1
E) Increased FEV1/FVC
A) Decreased FEV1/FVC
Chronic Obstructive Pulmonary Disease
Progressive, mostly irreversible airflow obstruction
Onset: Middle age or elderly 20-30 years after exposure
4th leading cause of mortality in the United States
Cigarette smoking is the leading cause of COPD
Smokers have a 40ml/year reduction in FEV1 after age 30
Lung growth can be impaired by maternal smoking in pregnancy and second hand smoke in childhood
Other exposures: workplace dusts from mining, cotton mills, and grain handling facilities
Lung Mechanics in COPD
Elastic recoil is the lungs innate ability to deflate following inflation.
Elastic fibers in the lung parenchyma, along with surface tension at the alveolar air-liquid interface are responsible for elastic recoil
Elastic recoil maintains the patency of small airways
Elastic recoil is markedly decreased in COPD
Airway resistance is increased in COPD
The sites of airflow obstruction are distal airways less than 2mm diameter
COPD History
Current or past cigarette use
Dyspnea with slow progression
History of acute bronchitis
History of a chronic cough
Sputum production
Wheezing
Physical Findings COPD
Barrel Chest
Prolonged expiratory phase
Accessory muscle use
Low Diaphragm
Distant Heart Sounds
Diminished Breath Sounds
Rhonchi
Wheezing
Cyanosis
Pedal Edema
Distended Jugular Veins
Hepatic congestion
Cachexia
“Blue Bloaters”
“Pink Puffers”
Pulmonary Function Testing in COPD
I Mild
FEV1/FVC = 80% of predicted
Pulmonary Function Testing in COPD
II Moderate
FEV1/FVC
Pulmonary Function Testing in COPD
III Severe
FEV1/FVC
Pulmonary Function Testing in COPD
IV Very Severe
FEV1/FVC
Chest x-ray in COPD
Hyperinflation Flattened Diaphragms Increased restrosternal space Bullae Can be normal in mild to moderate COPD
Differential Diagnosis COPD
Asthma
Bronchiectasis
Bronchiolitis obliterans
Emphysema
Enlargement of the air spaces distal to the terminal bronchiole with destruction of the alveolar walls
Centriacinar: affects respiratory bronchioles distal to the terminal bronchiole, remainder of the acinus spared. Occurs with smoking
Panacinar: alveolar ducts, adjacent alveoli, coalescence and bullae formation. Common in alpha 1 antitrypsin deficiency. Occurs in smoking
Most severe COPD patients have a combination of centriacinar and panacinar emphysema
Chronic Bronchitis
Enlargement of bronchial mucous glands and increased epithelial goblet cell production leads to cough and increased mucous production.
45 year old female with a history of asthma complains of a daily cough and increased dyspnea. She now wakes up 2 or 3 nights a week with symptoms. Her current medicine is a medium dose inhaled steroid and albuterol inhaler as needed.
Physical Examination: Pulse 80, Respiratory Rate 16 Lung examination is remarkable for bilateral expiratory wheezing
How would you categorize this patient’s asthma?
A) Intermittent asthma
B) Mild persistent asthma
C) Moderate persistent asthma
D) Severe persistent asthma
What is the most appropriate addition to this patient’s treatment?
A) Add a long acting B2-agonist inhaler
B) Add an ipratropium metered-dose inhaler
C) Double the dose of inhaled corticosteroid
D) Start a 10-day course of a macrolide antibiotic
mild persistant asthma
Asthma
Clinical syndrome of unknown etiology with 3 distinct components
1) Recurrent airway obstruction that resolves spontaneously or with treatment.
2) Airway hyperresponsiveness: exaggerated bronchoconstrictor response to stimuli that have little or no effect on nonasthmatic patients.
3) Airway Inflammation
Population Affected by Asthma
8% of Adults
Boys more common than girls before puberty
Women more common than men
Most cases start before age 25, but can occur at any age
15 million outpatient visit per year
2 million hospitalizations per year
Asthma Pathology
Mild Asthma: edema and hyperemia of the mucosa and infiltration of the mucosa with mast cells, eosinophils, and lymphocytes.
Moderate Asthma: chemokines eotaxin, RANTES, macrophage inflammatory protein 1 alpha, and interleukin 8 lead to inflammation and smooth muscle constriction
Severe: hypertrophy and hyperplasia of airway glands and smooth muscle lead to severe airway thickening
Airway Obstruction
Caused by a combination of
1) constriction of airway smooth muscle 2) thickening of airway epithelium 3) liquids in the airway
Asthma Triggers
Atopy
Occupational
Allergy
Food
Cold Air
Smoking or Smoke in the
environment
Pollution
Climate Changes
Emotion
Medication
Asthma Mediators
Acetylcholine: released from intrapulmonary motor nerves stimulate M3 muscarinic receptors causing airway smooth muscle constriction
Histamine: released from mast cells – minor role
Leukotrienes and Lipoxins: derived by the lipoxygenation of arachidonic acid released from the target cell membrane phospholipids during cellular activation
Nitric Oxide: produced by airway epithelial cells and by inflammatory cells found the asthmatic lung. High levels found during asthma attacks
Asthma History
Dyspnea, cough, wheezing, and anxiety
Exercise induced, aspirin ingestion, extrinsic(allergen induced), or intrinsic(unknown)
Cough, hoarseness, or inability to sleep through the night
Rapid changes in temperature or humidity may lead to an attack
Consider occupation exposures
