This document discusses several pulmonary diseases including asthma, chronic obstructive pulmonary disease (COPD), bronchiectasis, allergic bronchopulmonary aspergillosis (ABPA), and cystic fibrosis. It covers the causes, presentations, diagnostic tests, and treatment approaches for each condition. Pulmonary function tests and imaging studies are important for diagnosis, while treatment involves managing symptoms, treating infections, and addressing the underlying disease in a stepwise manner depending on severity.

1. Pulmonology

5. Asthma
 Asthma, or reactive airway disease,
 is an abnormal bronchoconstriction of the airways.
 Asthma is a reversible obstructive lung disease

6. Causes of acute exacerbations of
symptoms
 Allergens such as pollen, dust mites, and cat dander
 Infection and cold air
 Emotional stress or exercise
 Aspirin, NSAIDs, beta blockers, histamine, any nebulized medication,
 tobacco smoke
 Gastroesophageal reflux disease (GERD)

7. Presentation
 wheezing with the acute onset of shortness of breath,
 cough, and chest tightness .
 Increased sputum production

8.  Symptoms worse at night
 Nasal polyps and sensitivity to aspirin
 Eczema or atopic dermatitis on physical examination
 Increased length of expiratory phase of respiration
 Increased use of accessory respiratory muscles (e.g., intercostals)

9. Diagnostic Tests
 The best initial test in an acute exacerbation:
 peak expiratory flow (PEF) or arterial blood gas (ABG).
 Chest x-ray is most often normal in asthma,
 Chest x-ray is used to:
 Exclude pneumonia as a cause of exacerbation
 Exclude other diseases

10.  The most accurate diagnostic test is
 pulmonary function testing (PFTs).
 Spirometry will show a decrease in the ratio of forced expiratory volume in
1 second (FEV1) to forced vital capacity (FVC).
 The FEV1 decreases more than the FVC.

13. Pulmonary Function Testing in Asthma
 Decreased FEV1 and decreased FVC with a decreased ratio of FEV1/FVC
 Increase in FEV1 of more than 12% and 200 mL with the use of albuterol
 Decrease in FEV1 of more than 20% with the use of methacholine or
histamine
 Increase in the diffusion capacity of the lung for carbon monoxide (DLCO)

14. Additional testing options include:
 CBC may show an increased eosinophil count.
 Skin testing is used to identify specific allergens that provoke
bronchoconstriction.
 Increased IgE levels suggest an allergic etiology.
 IgE levels may also help nguide therapy such as the use of the
 anti-IgE medication omalizumab.

15. Treatment
 Asthma is managed in a stepwise fashion
 Step 1. Always start the treatment of asthma with an inhaled short-acting
beta agonist (SABA) as needed
 Albuterol
 Pirbuterol
 Levalbuterol

16. Step 2. Add a long-term control agent
to a SABA.
 Low-dose inhaled corticosteroids (ICS) are the best initial long-term
control agent.
 Example of ICS are:
 Beclomethasone, budesonide, flunisolide, fluticasone, mometasone,
triamcinolone

17. Step 3:
 Add a long-acting beta agonist (LABA) to a SABA and ICS, or increase the
dose of the ICS.
 LABA medications are salmeterol or formoterol.

18. Step 4.
 Increase the dose of the ICS to maximum in addition to the LABA and SABA.
 Add tiotropium, an antimuscarinic agent.

19. Step 5
 Omalizumab may be added to the SABA, LABA, and ICS in those who
have an increased IgE level.

20. Step 6.
 Oral corticosteroids such as prednisone are added when all the other
therapies are not sufficient to control symptoms.

21. Alternate long-term control agents include:
 Cromolyn and nedocromil to inhibit mast cell mediator release and
eosinophil recruitment
 Theophylline
 Leukotriene modifiers: montelukast, zafirleukast, or zileuton (best with
atopic patients)

22. Adverse Effects of Systemic Corticosteroids
 Osteoporosis
 Cataracts
 Adrenal suppression and fat redistribution
 Hyperlipidemia, hyperglycemia, acne, and hirsutism (particularly in
women)
 Thinning of skin, striae, and easy bruising

24. The severity of an asthma exacerbation is
quantified by:
 Decreased peak expiratory flow (PEF)
 ABG with an increased A-a gradient

25. Treatment
 Oxygen
 Albuterol
 Steroids
 Ipratropium

26. Chronic Obstructive Pulmonary Disease
 COPD is the presence of shortness of breath from lung destruction
decreasing the elastic recoil of the lungs.
 Most of the ability to exhale is from elastin fibers in the lungs
passively allowing exhalation.
 This is lost in COPD,
 resulting in a decrease in FEV1 and FVC with an increase in the
total lung capacity (TLC).
 COPD is not always associated with reactive airway disease such as
asthma, although both are obstructive diseases.

27. Causes
 Tobacco smoking leads to almost all COPD.
 Tobacco destroys elastin fibers.
 If the case describes a patient who is young and a nonsmoker
 alpha-1 antitrypsin deficiency as the most likely cause.

28. Presentation
 Shortness of breath worsened by exertion
 Intermittent exacerbations with increased cough, sputum, and
shortness of breath often brought on by infection
 “Barrel chest” from increased air trapping
 Muscle wasting and cachexia

29. Diagnostic Tests
 The best initial test is chest x-ray:
 Increased anterior-posterior (AP) diameter
 Air trapping and flattened diaphragms

31. The most accurate diagnostic test is PFT:
 Decreased FEV1, decreased FVC, decreased FEV1/FVC ratio (under 70%)
 Increased TLC because of an increase in residual volume
 Decreased DLCO (emphysema, not chronic bronchitis)
 Incomplete improvement with albuterol
 Little or no worsening with methacholine

32.  Reversibility with Inhaled Bronchodilators
 Patients with COPD have a broad range of response to inhaled
bronchodilators such as albuterol.

33.  Plethysmography will show an increase in residual volume.
 Arterial blood gas (ABG): Acute exacerbations of COPD are associated
with increased pCO2 and hypoxia.
 Respiratory acidosis may be present if there is insufficient metabolic
compensation and the bicarbonate level will be elevated to compensate.
 In between exacerbation, not all those with COPD will retain CO2.

34.  CBC: increase in hematocrit from chronic hypoxia
 EKG:
 Right atrial hypertrophy and right ventricular hypertrophy
 Atrial fibrillation or multifocal atrial tachycardia (MAT)
 Echocardiography:
 Right atrial and right ventricular hypertrophy
 Pulmonary hypertension

35. Treatment
 Improves Mortality and Delays Progression of Disease
 Smoking cessation
 Oxygen therapy for those with pO2 ≤55 or saturation ≤88%;
 Mortality Benefit is directly proportional to the number of hours that the
oxygen is used.
 Influenza and pneumococcal vaccinations

36. definitely Improves Symptoms
 Short-acting beta agonists (e.g., albuterol)
 Anticholinergic agents: tiotropium, ipratropium, aclidinium,
umeclidinium
 Steroids
 Long-acting beta agonists (e.g., salmeterol, formoterol, olodaterol)
 Pulmonary rehabilitation

37. Possibly Improves Symptoms
 Theophylline
 Lung volume reduction surgery

38. Treatment of Acute Exacerbations of Chronic
Bronchitis
 Similar to the treatment of acute asthma exacerbations.
 Antibiotics are generally used in acute exacerbations of chronic bronchitis
 (AECB) because infection is by far the most commonly identified cause.

39.  coverage should be provided against Streptococcus pneumoniae, H. influenzae,
and Moraxella catarrhalis.
 Macrolides: azithromycin, clarithromycin
 Cephalosporins: cefuroxime, cefixime, cefaclor, ceftibuten
 Amoxicillin/clavulanic acid
 Quinolones: levofloxacin, moxifloxacin, gemifloxacin

40. Second-Line Agents
 Doxycycline
 Trimethoprim/sulfamethoxazole

41. Criteria for Oxygen Use in COPD
 pO2 below 55 mm Hg or oxygen saturation below 88%
 OR
 If there are signs of right-sided heart disease/failure or an
elevated
 Hematocrit in pt with:
 pO2 less than 60 mm Hg or oxygen saturation below 90%

42. Bronchiectasis
 Bronchiectasis is an uncommon disease from
 chronic dilation of the large bronchi.
 This is a permanent anatomic abnormality that cannot be reversed or
cured.
 Bronchiectasis is uncommon because of better control of infections of the
lung which lead to the weakening of the bronchial walls.

43. Etiology
 The single most common cause of bronchiectasis is cystic fibrosis,
 . Other causes are:
 Infections: tuberculosis, pneumonia, abscess
 Panhypogammaglobulinemia and immune deficiency
 Foreign body or tumors
 Allergic bronchopulmonary aspergillosis (ABPA)
 Collagen-vascular disease such as rheumatoid arthritis

44. Presentation
 Recurrent episodes of very high volume purulent sputum production
 Hemoptysis can occur.
 Dyspnea and wheezing are present in 75% of cases.
 Weight loss
 Anemia of chronic disease
 Crackles on lung exam
 Clubbing is uncommon

45. Diagnostic Tests
 The best initial test is a chest x-ray that shows dilated, thickened
bronchi, sometimes with “tram-tracks,” which is the thickening of the
bronchi.
 The most accurate test is a high-resolution CT scan.

47. Treatment
 Chest physiotherapy (“cupping and clapping”) and postural drainage are
essential for dislodging plugged-up bronchi.
 Treat each episode of infection as it arises.
 inhaled antibiotics
 Rotate antibiotics, 1 weekly each month.
 Surgical resection of focal lesions may be indicated.

48. Allergic Bronchopulmonary Aspergillosis (ABPA)
 ABPA is hypersensitivity of the lungs to fungal antigens that
colonize the bronchial tree.
 ABPA occurs almost exclusively in patients with asthma and a history of
atopic disorders.

49. Presentation
 Look for an asthmatic patient with recurrent episodes of
brown-flecked sputum
 and transient infiltrates on chest x-ray.
 Cough, wheezing, hemoptysis, and sometimes
bronchiectasis occur.

50. Diagnostic Tests
 Peripheral eosinophilia
 Skin test reactivity to aspergillus antigens
 Precipitating antibodies to aspergillus on blood test
 Elevated serum IgE levels
 Pulmonary infiltrates on chest x-ray or CT

51. Treatment
 Oral steroids (prednisone) for severe cases
 Itraconazole orally for recurrent episodes

52. Cystic Fibrosis
 Cystic fibrosis (CF) is an autosomal recessive disorder caused by a
mutation
 in the genes that code for chloride transport.
 This is known as the cystic fibrosis transmembrane conductance
regulator (CFTR).

53.  Mutations in the CFTR gene damage
 chloride and water transport across the apical surface of epithelial cells in
exocrine glands throughout the body.
 This leads to abnormally thick mucus in the lungs, as well as damage
the pancreas, liver, sinuses, intestines, and genitourinary tract
 Damaged mucus clearance decreases the ability to get rid of inhaled
bacteria.

54. Presentation
 Over one-third of CF patients are adults.
 young adult with chronic lung disease (cough, sputum, hemoptysis,
bronchiectasis, wheezing, and dyspnea) and recurrent episodes of
infection.
 Sinus pain and polyps are common.

55. Gastrointestinal Involvement
 Meconium ileus in infants with abdominal distention
 Pancreatic insufficiency (in 90%) with steatorrhea and vitamin A, D, E, and
K malabsorption
 Recurrent pancreatitis
 Distal intestinal obstruction
 Biliary cirrhosis

56. Genitourinary Involvement
 Men are often infertile; 95% have azoospermia, with the vas deferens missing
in 20%.
 Women are infertile.

57. Diagnostic Tests
 The most accurate test is an increased sweat chloride test.
 sweat above 60 meq/L on repeated testing establishes the diagnosis.

58. Additional Diagnostic Tests
 Chest x-ray and CT: There is no single abnormality on imaging of the chest
to confirm a diagnosis of CF. Findings include:
 Bronchiectasis
 Pneumothorax
 Scarring
 Atelectasis
 Hyperinflation
 Arterial blood gas may show hypoxemia and, in advanced disease, a
respiratory acidosis.

59.  PFTs show mixed obstructive and restrictive patterns; decrease in FVC and
total lung capacity; and decreased diffusing capacity for carbon
monoxide.
 Sputum Culture:
 Nontypable Haemophilus influenzae
 Pseudomonas aeruginosa•
 Staphylococcus aureus
 Burkholderia cepacia

60. Treatment
 Antibiotics are routine.
 Inhaled recombinant human deoxyribonuclease (rhDNase).
 This breaks down the massive amounts of DNA in respiratory mucus that
clogs up the airways.

61.  Inhaled bronchodilators such as albuterol
 Pneumococcal and influenza vaccinations
 Lung transplantation is used only in advanced disease not responsive to
the therapy previously listed.
 Ivacaftor increases the activity of CFTR in the 5% of patients who have
aspecific mutation.