RESPIRATORY DISEASES: Introduction

3 General Classes of Respiratory Disorders

1. Obstructive Ventilatory Diseases (OVD)
- BA, COPD, Bronchiolitis, Bronchiectasis, CF
2. Restrictive Ventilatory Diseases (RVD)
- PAINT
- Pleura: Pleural Effusion
- Alveoli: CHF, PTB
- Interstitium: ILD
- Neuromuscular: GBS, ALS
- Thoracic Cage: Kyphoscoliosis
3. Abnormalities of the Vascular System
- PE, PHPN, Pulmonary Veno-Occlusive disease
Signs and symptoms: Cough, dyspnea and wheezing
- Cough: acute (<2weeks), chronic (>8weeks)
Diagnostic Evaluation:
1. PFT
2. ABG
Imaging
BRONCHIAL ASTHMA
General Considerations Physiology Pathology Diagnosis Treatment
- Atopy: major risk factor AHR – airway TH2: IL-4, IL-5, IL-9, IL-13 Spirometry A. Bronchodilators
- Viral infection: RSV, hyperreactivity, - Decreased FEV1/FVC 1. B – agonists
rhinovirus, Corona virus physiologic abnormality > Eosinophil linked to (<70%) with reversibility - adenylyl cyclase 
of asthma AHR (>12% and 200 ml increase cAMP
> MC trigger of an
increase in FEV1 after - tolerance: down
acute severe - decreased FEV1/FVC > Increased CD4 + T-cells SABA or 2-4 weeks trial regulation of B -receptors
exacerbation - decreased PEF of OCS)
- Dermatophagoides sp. - increased airway > IL 13 induces mucus 2. Antocholinergics
> MC trigger of resistance hypersecretion AHR measured by - prevent
exacerbation - increased residual methacholine challenge bronchoconstriction and
- Thunderstorm asthma: volume (hyperinflation) > Nitric oxide (NO): test (PC20) mucus secretion
related to eosinophilic - SE: dry mouth, urinary
disrupts pollen grains
- Autopsy: thickening and inflammation FeNO (Fraction of retention and glaucoma
- β-adrenergic blockers edema of airway wall exhaled Nitric oxide):
- Exercise- induced with mucous plug measures airway 3. Theophylline
asthma (EIA): increased inflammation - PDE inhibition
osmolality in airway
lining fluid and triggers B. Controller
mast cell mediator 1. ICS
- reduce AHR
release
- local SE: dysphoria and
- Intrinsic asthma oral candidiasis -> use
> Adult-onset spacers
> Normal IgE
> Aspirin sensitive 2. Systemic steroids
> with nasal polyp - acute attacks
> (-) skin test
3. Omalizumab
- anti – IgE
COPD
General Considerations Physiology Pathology Diagnosis Treatment
- Risk Factors: cigarette Airflow limitation: major Histone Deacetylase 2 Spirometry: A. Pharmacotherapy
smoking and biomass fuel physiologic change in (HDAC2) – inactivation - decreased FEV1/FVC 1. Smoking cessation:
- Genetics: COPD (<70%) influence the natural
> α1AT deficiency Transcriptio of MMP, IL-8 - increased RV, increased history of COPD
Decreased FEV1/FVC and TNF FRC a. Bupropion
- Z alleles: markedly
without reversibility - increased RV/TLC b. NRT
decrease α1AT levels Lung destruction - decreased DLCO: c. Varenicline
- 2 Z-alleles or 1 Z and Scooped out lower part of Emphysema 2. Anticholinergic
1 null allele: MC form descending limb of flow A. Large Airway - Tiotropium,
of severe α1AT volume curve - goblet cell hyperplasia GOLD severity: Ipratropium
deficiency -> cough and mucus > 80: mild 3. B- agonist
- Centriacinar Increased RV, production >50 - <80: moderate - Synergistic with
Increased RV/TLC >30 - <50: severe Ipratropium
emphysema: most
B. Small Airway <30: very severe 4. ICS/OCS
frequently associated V/Q mismatch: major - major site of increased 5. Theophylline
with cigarette smoking cause of hypoxemia in resistance: <2mm - Roflumilast: selective
- Decreased FEV: most COPD diameter PDE4 inhibitor 
closely associated with - goblet cell metaplasia chronic bronchitis
mortality 6. Antibiotics
- Advanced disease: decreased clara cells - Azithromycin
7. Oxygen
Cachexia: increased
decreased surfactant - only treatment that
TNF- α -> weight loss, can decrease mortality
bitemporal wasting and C. Lung parenchyma
loss of subcutaneous - destruction of gas- B. Non-pharmacologic
tissue exchanging air spaces 1. Influenza vaccination >
- Hoover’s sign: pneumonia vaccination
Emphysema 2. Pulmonary
paradoxical inward
- increased CD8+ cells rehabilitation
movement of rib cage 3. Lung volume reduction
with inspiration surgery (LVRS) – upper
lobe emphysema
4. Lung transplant
 Acute exacerbation:
Bronchodilators

Glucocorticoids Antibiotics

Others
- O2
- NIPPV
- MV/IPPV

BRONCHIECTASIS
General Considerations Physiology Pathology Diagnosis Treatment
- Irreversible airway - poor mucociliary clearance and susceptibility to High resolution computed - H. influenza and P.
dilatation infection  microbial colonization  chronic tomography (HRCT): aeruginosa: MC isolated
- Tubular type: MC form inflammation  release proteases, ROS, imaging modality of organisms
proinflammatory cytokines damage large airway choice Diagnostic criteria for
1. Focal bronchiectasis
walls - “tram track” or “signet NTM infection:
- Secondary to ring sign” 1. >/= 2 sputum samples
obstruction (+) on culture
2. Diffuse bronchiectasis 2. >/= 1 BAL fluid (+) on
- Infection culture
3. Biopsy with
- Location by etiology histopathologic features
1. CF - upper lung field of NTM + 1 sputum (+)
2. NTM (MAC) – mid lung culture
3. Allergic 4. Pleural fluid sample (+)
bronchopulmonary on culture
aspergillosis (ABPA) – Treatment: Macrolide +
central airways Rifampin + EMB
-Suppressive antibiotics
as prevention: long-term
macrolide for 6-12 mos.
CYSTIC FIBROSIS
General Considerations Physiology Pathology Diagnosis Treatment
- Autosomal recessive A. Respiratory CFTR (CF Sweat electrolyte test: 1. Recombinant DNAse
- Mutation of CFTR gene - copious hyperviscous Transmembrane mainstay aerosol (Dornase) and
- H. influenza, S. aureus, and adherent secretions conductance regulator) hypertonic saline
obstruct small and - A conduit for Cl- and CFTR mutation analysis nebulization
and Pseudomonas
medium sized airways HCO3- transport across
aeruginosa plasma membrane 2. Ivacaftor: potentiate
B. Pancreas - Provide sufficient depth CFTR channel opening
- Concretions  obstruct of periciliary fluid layer and stimulate ion
pancreatic duct  transport
chronic malabsorption, CFTR deficiency  ciliary
poor growth, fat-soluble collapse  failure to clear
vitamin insufficiency overlying mucus

C. Others
- Meconium ileus
- Infertility
PLEURAL EFFUSION
General Considerations
- Pleural fluid formation
exceeds absorption
- Lymphatic absorption
has 20x more absorptive
capacity than pleural fluid
production
Physiology Pathology Diagnosis Treatment
Transudative Effusion Ultrasound: imaging of A. Transudative Effusion
1. CHF: increased choice - usually treat systemic
hydrostatic pressure - Differentiate whether cause
2. Hepatic hydrothorax: fluid is transudate or
direct movement of exudate B. Exudative Effusion
peritoneal fluid through 1. Parapneumonic
small openings in the 1. Exudate - Indication for CTT:
diaphragm into the -Local factors: a. Gross pus
pleural space a. Pleural fluid b. (+) gram stain/culture
protein/serum protein of pleural fluid
Exudative Effusion >0.5 c. Pleural fluid glucose
1. Parapneumonic b. Pleural fluid LDH/ <60mg/dl
effusion: inflammation serum LDH >0.6 d. pleural pH< 7.20
from infection c. Pleural fluid LDH > 2/3 e. loculated pleural fluid
2. Malignant effusion the normal upper limit
- MC: lung, breast cancer for serum 2. Malignancy
and lymphoma - pleurodesis
- Tumor invasion of the 2. Transudate
pleura and blockade of - Systemic 3. Chylothorax
pleural lymphatic - MC: CHF - CTT plus Ocreotide
3. Mesothelioma
- Asbestos exposure * Specific Considerations:
4. Pulmonary embolism - 1. CHF
- Exudate and transudate - NT-proBNP >1500pg/ml
5. TB is diagnostic of CHF
- Hypersensitivity
reaction to TB protein in 2. TB
the pleural space - adenosine deaminase
>40 IU/L or
interferon γ >140 pg/mL
3. Chylothorax
- MC cause is trauma
 - triglyceride >1.2mmol/L
or 110mg/dL

4. Hemothorax
- if pleural fluic Hct is
>1/2 of that in the
peripheral blood

PNEUMOTHORAX
General Considerations Physiology Pathology Diagnosis Treatment
1. Primary Spontaneous 1. PSP Chest Radiography 1. PSP
Pneumothorax (PSP) - Rupture of apical pleural - Simple aspiration
- No underlying lung blebs
Thoracoscopy with
disease
2. SSP stapling of blebs
- young; marfanoid - Bullae formed by
habitus; smokers primary lung disease 2. SSP
- Immediate CTT
2. Secondary 3. Traumatic
Spontaneous Pneumothorax Thoracoscopy with
Pneumothorax (SSP) - Penetrating or non- stapling
penetrating
- with underlying lung
3. Traumatic
disease (eg: COPD, PTB) 4. Tension Pneumothorax Pneumothorax
- The positive pleural - Supplemental O2 or
3. Taumatic pressure compromises aspiration
Pneumothorax ventilation and is
- MC cause: Iatrogenic transmitted to the CTT
mediastinum  decrease
venous return  4. Tension Pneumothorax
4. Tension Pneumothorax
decrease cardiac output - Immediate CTT or large
- The pressure in the bore needle on the 2nd
pleural space is (+) anterior ICS
throughout the
respiratory cycle
INTERSTITIAL LUNG DISEASES
General Considerations Physiology Pathology Diagnosis Treatment
Progressive exertional Spirometry 2 Major histologic HRCT 1. Glucocorticoids
dyspnea: most common - decreased TLC, FRC and Patterns: - in some cases, this is - mainstay of therapy
presenting complaint RV enough to reach a - suppression of
- decreased FEV1 and 1. Granulomatous diagnosis inflammation
FVC - eg: Sarcoidosis - eg: IPF
Lymphangioleiomyomatosis - increased FEV /FVC or - provide better 2. Lung Transplantation
1
(LAM) and pulmonary normal 2. Inflammation and assessment of extent and - severe cases
involvement in tuberous - decreased DLCO: fibrosis distribution of disease
sclerosis occur exclusively effacement of alveolar - eg: Interstitial - determine the most
in premenopausal women capillary units pneumonia appropriate area for
biopsy
ABG
Idiopathic Pulmonary
- hypoxemia with PFT
Fibrosis (IPF): most respiratory alkalosis - restrictive defect
common form of idiopathic
interstitial pneumonia Lung biopsy
- most effective method
Pleuritis: most common for confirming the
pulmonary manifestation of diagnosis and assessing
the disease activity
SLE

LAM: young women with

“emphysema”, recurrent
pneumothorax and chylous
pleural effusion
PNEUMONIA
General Considerations Physiology Pathology Diagnosis Treatment
I. CAP IL 1 & TNF: Fever Phases: 1. Clinical and radiologic Important antimicrobial
- MC route of infection  1. Edema diagnosis concepts
aspiration from the IL-8 & G-CSF: peripheral - proteinaceous exudate
leukocytosis, neutrophilia and bacteria in the alveoli 2. Etiologic diagnosis A. Typical organisms
oropharynx
and purulent secretions a. sputum GS/CS eg: Strep. Pneumonia, S.
- cough mechanism and 2. Red Hepatization - GS: suitability of sample aureus, H. influenza,
gag reflex offer critical Macrophage and - RBC in the intraalveolar - > 25 neutrophils Moraxella
protection from neutrophils: alveolar exudates - < 10 squamous - B. lactam antibiotics
aspiration capillary leak  - neutrophil influx epithelial cells eg: Cephalosporins
- Streptococcus “crackles” - (+) bacteria b. blood culture
pneumonia: MC etiology c. urinary Ag tests B. Atypical
3. Gray Hepatization - Legionella - Legionella, Chlamydia,
- MRSA may cause
- Neutrophil is the d. PCR: nasopharyngeal Mycoplasma, viruses
necrotizing pneumonia predominant cell swabs - Tx: Macrolides or
- fibrin deposition - viruses, legionella, Quinolones
II. HCAP - (-) bacteria mycoplasma, chlamydia,
- successful containment mycobacteria C. Special organisms
III. VAP/HAP of infection tuberculosis 1. Pseudomonas:
- Endotracheal tube: most Tazobactam +
4. Resolution 3. Biomarker Piperacillin;
important risk factor
- macrophage is the a. Procalcitonin Cephalosporins
dominant cell b. CRP
2. MRSA: Vancomycin or
Linezolid

Prevention:
1. Pneumonia vaccination
- PCV 13 and PPSV 23

2. Influenza vaccination
TYPES OF RESPIRATORY FAILURE

Type I: Acute Hypoxemic Type II: Hypoventilatory Type III: Perioperative Type IV: Shock

Mechanism Qs/ Qt VA Atelectasis Hypoperfusion

CNS drive FRC

N-M coupling Closing volume Cardiogenic

Etiology Air space flooding
Hypovolemic
Septic
Dead space

Pulmonary edema Overdose/ injury Obese/Supine; MI; PHPN

Ascites/ peritonitis;
Pneumonia MG; ALS; Botulism Upper abdominal Dehydration;
incision; Anesthesia Hemorrhage;
Clinical description
Chest Trauma BA/COPD; Pulmonary Tamponade
fibrosis; Kyphoscoliosis Age/smoking;
Lung hemorrhage Fluid overload Endotoxemia;
Bacteremia