CLIN PATH I: EXAM #2 (PULM DZ LEC)

Chronic obstructive pulmonary disease (COPD) is a _________________ disease

preventable and treatable

Obstructive Lung Dz: Fundamental issue is __________________ due to DEC airway caliber

resistance to airflow

Obstructive Lung Disease = INC RESISTANCE results from processes within the lumen: Seen with obstructing secretions associated with ____________________

asthma & chronic bronchitis

Obstructive Lung Disease = INC RESISTANCE results from processes in the airway wall: thickening & narrowing can result from inflammation seen with _________________________________________

asthma & chronic bronchitis or bronchial smooth muscle contraction in asthma

Obstructive Lung Disease = INC RESISTANCE results from processes in structures that support the airway:

_______________ is the classic example as lung elastic tissue is destroyed

Emphysema

Obstruction can be reversible

(__________)

Asthma

Obstruction can be irreversible (____________________)

Chronic bronchitis & Emphysema

__________: progressive, largely irreversible airflow obstruction due to (1) loss of elastic recoil and (2) increased airway resistance.

COPD

COPD: ____________________ most important risk.

Cigarette smoking/exposure

_________________: only genetic disease linked to COPD in younger patients <40 y/o.

Alpha-1 Antitrypsin deficiency

_____________: reversible, often intermittent, obstructive disease of the small airways.

(Wheezing, dyspnea, and cough, especially at night. May have chest tightness and fatigue.)

Asthma

Asthma: M/C __________ pulmonary disease

chronic

Asthma: M/C in children (______)

M > F

_________: High risk populations

•African Americans

•Inner-city dwellers

•Premature/low birth wt babies

Asthma

Asthma Triggers: _______________

- Anxiety

- Stress

- Exercise

- Cold Air

- Dry Air

- Hyperventilation

- Viral Infections

Intrinsic (Non-Allergic)

Asthma Triggers: _______________

- Animal Dander

- Pollen

- Mold

- Dust Mites

- Cockroaches

- Associated with increased IgE

Extrinsic (Allergic)

___________: airway hyperreactivity, bronchoconstriction, and inflammation

Asthma

Asthma Triggers:

________________:

• Histamine

• Methacholine

• Adenosine triphosphate

Physiologic or pharmacologic mediators

Asthma Triggers:

________________:

• Penicillin's/Anhydrides

• Animal dander/dust mites

Allergens (induce airway inflammation & reactivity in sensitized individuals)

Asthma Triggers:

________________:

• Exercise

• Air pollutants

• Viral respiratory infections

• Propranolol

• Aspirin, NSAIDs

Exogenous physiochemical agents that produce airway hyperactivity

________ is the strongest risk factor for Asthma.

Atopy

Asthma be fatal with severe & complete airway lumen obstruction by mucous plugs (__________________)

Status asthmaticus

Atopic Triad: _____________ (common manifestations of Atopy)

Asthma + Allergic Rhinitis + Atopic Dermatitis (Eczema)

Fundamental abnormality in asthma is ____________________________

increased reactivity of airways to stimuli

____________: Increased IgE binds to mast cells, initiating an inflammatory response, including increased Leukotrienes.

(airway hyperreactivity, bronchoconstriction, and inflammation)

Asthma

Pathology of Asthma: _____________________________

Wall inflammed and thickened, tightened smooth muscles, air trapped in alveoli

Pathology of Asthma:

Airway inflammation & smooth muscle hyperresponsiveness --> narrowing of airways leading to what?

Airflow resistance

What exacerbates obstruction in Asthma?

Mucous hypersecretion & bronchoconstrictor stimuli

Ashtma: Stimulation of _____________ --> cough & reflex bronchoconstriction

bronchial irritant receptors

_____________ is an ominous sign during a severe asthma attack indicating progressive airway obstruction, muscle fatigue, & falling alveolar ventilation

Mild hypercapnia

Asthma = __________

A. HIGH V/Q

B. LOW V/Q

LOW V/Q

Clinical Manifestations of Asthma

Dyspnea and Chest tightness

• Greater muscle effort stimulates _______________

spindle stretch receptors

Clinical Manifestations of Asthma

Dyspnea and Chest tightness

• Hyperinflation --> ________________

thoracic distension

Clinical Manifestations of Asthma

Dyspnea and Chest tightness

• Lung compliance falls, and work of breathing increases --> ________________

chest tightness

Clinical Manifestations of Asthma

Dyspnea and Chest tightness

• As obstruction worsens, increased VQ mismatch --> ____________

hypoxemia

Clinical Manifestations of Asthma

Dyspnea and Chest tightness

• Rising arterial CO2 tension --> ___________________ --> progressive dyspnea

stimulated respiratory drive

Clinical Manifestations of Asthma

Wheezing:

• Smooth muscle contraction, with mucus secretion and retention --> _________________ --> wheezing

caliber reduction and prolong turbulent airflow

Clinical Manifestations of Asthma

Wheezing:

• Intensity of wheezing does not correlate with ___________

severity

Clinical Manifestations of Asthma

• Combo of airway narrowing, mucus hypersecretion, and neural afferent hyperresponsiveness --> __________

Cough

Clinical Manifestations of Asthma

May be absent in mild disease, but universal in acute exacerbations --> _________________________

Tachypnea and Tachycardia

Clinical Manifestations of Asthma

INC lung hyperinflation augments venous return and INC R EDV causes septum to move to left, compromising LV filling and output --> _____________________

Pulsus Paradoxus

Clinical Manifestations of Asthma

Low VQ ratios --> ____________

Hypoxemia

Clinical Manifestations of Asthma

• Severe attacks --> respiratory muscles fatigue --> ________________________

alveolar hypoventilation, hypercapnia and respiratory acidosis

Clinical Manifestations of Asthma

________________________:

• During active asthma attacks, expiratory flow is reduced

• Give bronchodilator = improve

Obstructive defects in PFT

Clinical Manifestations of Asthma

Hypercapnia and respiratory acidosis

• ______________ --> ventilation is normal or increased, and art PCO2 is either normal or decreased

Mild to mod asthma

Clinical Manifestations of Asthma

___________________:

• (1) a 12% or greater increase in the FEV1 in response to an inhaled bronchodilator or

(2) a 20% or greater decrease in FEV1 in response to a provoking factor

Bronchial hyperresponsiveness

Clinical Manifestations of Asthma

Bronchial hyperresponsiveness:

• (1) a _____________________ in response to an inhaled bronchodilator

12% or greater increase in the FEV1

Clinical Manifestations of Asthma

Bronchial hyperresponsiveness:

(2) a ________________ in response to a provoking factor

20% or greater decrease in FEV1

CC: Productive cough for at least 3 months a year for 2 consecutive years. M/C etiology is smoking.

A. Chronic Bronchitis

B. Emphysema

Chronic Bronchitis

Permanent enlargement of the terminal airspaces (distal to the terminal bronchioles) with no obvious fibrosis. M/C etiology is smoking. Air pollution, hazardous dust.

A. Chronic Bronchitis

B. Emphysema

Emphysema

_________________________:

• Loss of elastic tissue results in a loss of appropriate recoil tension

• No mucus production or cough

• Loss of alveolar surface area and cap bed --> progressive hypoxia and dyspnea

Emphysema

_____________: "pink puffers"

- Cachetic, pursed lip breathing - noncyanotic

Emphysema

______________: "Blue Bloaters"

- obese & cyanotic

Chronic Bronchitis

Chronic Bronchitis: Due to chronic inflammation, the normal ciliated pseudostratified columnar epithelium is replaced with patch squamous metaplasia --> _______________________

decreased mucociliary clearance

_____________:

- Rales (crackles)

- Rhonchi

- Wheezing +/- change in location with cough

- +/- signs of cor pulmonale (peripheral edema, cyanosis)

Chronic Bronchitis

_______________:

- Respiratory Acidosis

- Polycythemia (INC Hematocrit/RBC d/t hypoxia --> erythropoiesis!)

Chronic Bronchitis

_________________:

- INC lung volumes

- “tram track lines” d/t bronchial thickening

- Enlarged cardiac size

Chronic Bronchitis

_________________:

- Dyspnea is M/C Sx

- Accessory muscle use, tachypnea, prolonged expiration.

Emphysema

_________________: Condition marked by irreversible enlargement of airway spaces distal to terminal bronchioles (acinus) accompanied by destruction of their walls w/o obvious fibrosis

Emphysema

_______________: Chronic inflammation, decreased protective enzymes (eg, Aloha-1 Antitrypsin) & increased damaging enzymes (eg, elastase release from macrophages & neutrophils) cause alveolar capillary destruction + alveolar wall destruction.

Emphysema

____________________: Matched V/Q defects & decreased gas exchange surface area. Loss of elastic recoil & airway collapse makes expiration an active process & increased compliance leads to airway obstruction (increased air trapping).

Emphysema

Emphysema: ___________________ involvement is M/C associated with smoking.

Centrilobar (proximal acinar)

Emphysema: ___________________: global destruction of the acinus with diffuse airspace distension

• Associated with α1-antitrypsin deficiency

Panacinar (diffuse)

Dyspnea (hallmark of ______________), chronic cough (with or without sputum production).

PE: Hyperinflation: decreased breath sounds, increased anteroposterior diameter ("barrel chest"), hyperresonance to percussion, wheezing. Cachetic and non-cyanotic = "pink puffers."

Emphysema

Dx Emphysema with a _______________: gold standard.

Airway obstruction: decreased FEV1, decreased FEV1/FVC < 70% predicted, decreased FVC.

Pulmonary function test

Dx Emphysema with a Pulmonary function test = gold standard.

Airway obstruction: _________________________ < 70% predicted, decreased FVC.

decreased FEV1, decreased FEV1/FVC

______________ CXR:

• Hyperinflation

• Flattened diaphragms

• Increase AP diameter

• Decreased vascular markings; Bullae

Emphysema

M/C feature in ___________________ --> infiltration of the lung by inflammatory cells and fluid, leading to scarring, fibrosis, and capillary obliteration

Idiopathic pulmonary fibrosis

________________ – anatomic space bounded by:

• Basement membranes of epithelium & endothelium

Contains:

• Fibroblasts (mesenchymal cells)

• Collagen, elastin, proteoglycans (cellular matrix molecules)

• Some leukocytes (mast cells & lymphocytes)

Lung parenchyma

___________________ typically presents in men at 5th decade (>40 y/o)

Idiopathic Pulmonary Fibrosis

Clinical Manifestations of __________________:

• Progressive dyspnea with dry and persistent cough

• Digital cyanosis, clubbing, and Pulmonary HTN occur in late stages

Idiopathic Pulmonary Fibrosis

Etiology of _____________________:

• Major risk factors --> smoking and environment exposure to organic and inorganic dust ASK ABOUT OCCUPATION!!!!!

Idiopathic Pulmonary Fibrosis

_________________ Imaging Studies:

CXR: Basal predominant reticular opacities ("honeycombing.")

Chest CT: preferred imaging modality - reticular honeycombing, focal ground-glass opacification, traction Bronchiectasis or Bronchilectasis.

Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis (Pulmonary Function Test):

- ________________ - normal or increased FEV1/FVC, normal or decreased FVC, decreased lung volumes (eg, VC, RV, FRC, TLC), decreased DLCO.

Restrictive pattern

In Idiopathic Pulmonary Fibrosis, _______________ is common --> results from patchy fibrosis causing regional VQ mismatch

Hypoxemia

In Idiopathic Pulmonary Fibrosis, ________________ is a grave sign, implying an inability to maintain adequate alveolar ventilation as a result of excess work of breathing

Hypercapnia

What Dz is caused by an infection with Mycobacterium tuberculosis?

Tuberculosis

What shape is Mycobacterium tuberculosis?

Rod shaped acid fast bacilli

How is TB transmitted?

Inhalation of airborne droplets

Pathophysiology of _____________________: after inhalation, Mtb goes to the alveoli, gets incorporated into macrophages and can disseminate from there.

Tuberculosis

TB - Phagocytosis by macrophages (innate immunity) results in either:

• Successful control of the infection --> ________________

OR

• Progression to active disease (primary progressive tuberculosis)

latent tuberculosis

TB - Phagocytosis by macrophages (innate immunity) results in either:

• Successful control of the infection --> latent tuberculosis

OR

• Progression to active disease (_________________)

primary progressive tuberculosis

Risk Factors for TB:

- Close contact w/someone infected with TB, immigrants from highly endemic regions, crowded conditions (eg, prisons, shelters), healthcare workers, immunosuppression (eg, DM).

- _________________: 7-10% yearly with chance of reactivation of latent TB infection.

TB & HIV Infection

TB: In persons with intact immune systems, the next defensive step is formation of ________________ around the organisms.

granulomas

TB: By 2 or 3 weeks, the necrotic environment resembles soft cheese, often referred to __________________.

caseous necrosis.

TB: Lesions undergo fibrosis and calcification, successfully controlling the infection so that the bacilli are contained in the dormant, healed lesions (________________).

Ghon complexes

TB: Lesions in persons with less effective immune systems progress to _________________________.

primary progressive tuberculosis

TB: In immunocompromised pts, the semiliquid necrotic material can then drain into a bronchus or nearby blood vessel --> air-filled cavity at the original site (______________).

cavitation

TB can spread to blood vessels --> __________________

extrapulmonary tuberculosis

________________ can spread to other organs and cause extrapulmonary tuberculosis:

• Blood stream - disseminated or miliary (seed like deposits throughout lung) tuberculosis

• Cervical Lymph Nodes (Scrofula)

• Pleura

• Bones/joints (Pott’s disease)

• Meninges (CNS)

M. tuberculosis

CC: Dyspnea, fatigue, CP, weakness, cyanosis, edema. Exertional syncope if severe.

PE: Signs of RT sided HF - INC JVP, peripheral edema, ascites. Pulmonary regurg, RV heave, systolic ejection click.

Pulmonary HTN (Cor Pulmonale)

Pulmonary HTN M/C cause of death is ______________________

decompensated right heart failure

Pathophysiology of _______________: INC pulmonary vascular resistance leads to RV Hypertrophy, INC RV pressure and eventually RT-sided HF.

Pulmonary HTN (Cor Pulmonale)

_______________: Elevated mean pulmonary arterial pressure > 25mmHg (at rest) and >30 mmHg (w/exercise) with a pulmonary vascular resistance > 3 Wood units.

Pulmonary HTN (Cor Pulmonale)

________________: M/C in middle aged or young women (21-40). BMPR2 gene defect. BMPR2 gene normally inhibits pulmonary vessel smooth muscle growth and vasocontriction.

Primary Pulmonary HTN

Risk Factors of _________________________:

- Collagen Vascular Dz

- Congenital Heart Dz

- Portal HTN

- HIV Infection

- Drugs/toxins

- Pregnancy

Pulmonary Arterial HTN (PAH)

"Plexiform lesion" = _______________

Pulmonary HTN (Cor Pulmonale)

Vascular Injury d/t ___________________:

- INC Endothelin-1/Thromboxane Production

- DEC NO synthase/Prostacyclin Production

Pulmonary HTN (Cor Pulmonale)

A hallmark of PAH is _________________, complex vascular formations originating from remodeled pulmonary arteries.

plexiform lesions (PLs)

Pathophysiology of _____________________:

1. Pulmonary vascular fibrosis/thrombosis

2. Remodeling = DEC compliance "STIFF"

3. INC RV afterload: PUSH against stiff pulm artery

4. INC pulm artery pressure --> DEC CO

5. Compensation: tachy --> INC CO ---> DEC Preload = DEC SV

Pulmonary HTN (Cor Pulmonale)