L3- Pulmonary Pathology II Flashcards by Connor Patrick

ALI = (1- include alternate name) is characterized by (slow/rapid) onset of (3) and (4) in the absence of (5).

1- acute lung injury // noncardiogenic pulmonary edema
2- abrupt / rapid onset
3- significant hypoxemia
4- bilateral pulmonary infiltrates
5- cardiac failure

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(1) is the histological manifestation of ALI/(2)/(3). (1) is the root cause of (4).

1- DAD, diffuse alveolar damage
2/3- (acute lung injury), noncardiogenic pulmonary edema, shock lung syndrome
4- ARDS (acute respiratory distress syndrome)

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DAD is the main cause of (1) during one of the following settings: (2). (1) is characterized by (3).

(diffuse alveolar damage)
1- ARDS, acute respiratory distress syndrome
2- sepsis, severe trauma, diffuse pulmonary infection (etc)
3- progressive respiratory insufficiency

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list the ‘direct’ causes of pulmonary insult in ARDS (hint- 6)

pneumonia
aspiration
emboli
inhalation injury
drowning
oxygen toxicity

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list the ‘indirect’ causes of pulmonary insult in ARDS (hint- 7)

sepsis
trauma with shock
acute pancreatitis
severe burns
blood transfusion (or its products)
uremia
drugs

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list the risk factors for ARDS

Non-heritable: smoking and alcohol

Heritable: several genes, includes variants that map genes linked to inflammation and coagulation

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list the steps of ARDS/ALI pathogenesis

1) endothelial activation
2) adhesion and extravasation of neutrophils
3) accumulation of intra-alveolar fluid and formation of hyaline membranes

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In ARDS/ALI, endothelial activation occurs in response to….

(1st of 3 steps of pathogenesis)

directly by circulation inflammatory factors
secondary to pneumocyte injury
*mediated by alveolar macrophages

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In ARDS/ALI, after endothelial activation, (1) is increased on the endothelium in order to (2) and migrate them into (3). Once (2) enters into (3), (4) will occur.

(2nd of 3 steps of pathogenesis)
1- adhesion molecules
2- bind neutrophils
3- interstium and alveoli
4- degranulate and release inflammatory mediators

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In ARDS/ALI, inflammatory molecules from neutrophils leads to (1) within the alveoli. (1) will lead to the loss of (2) and the deficiency of (3). As a result (4) and (5) accumulate within the alveoli in order to form (6).

(3rd of 3 step of pathogenesis)
1- leaky capillaries
2- diffusion across alveolar membrane
3- surfactant (type II pneumocyte damage)
4- protein rich fluid
5- cellular debris
6- hyaline membrane

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list the chemical mediators involved in the pathogenesis of ARDS/ALI

CKs
oxidants
GFs
TNF
IL-1, IL-6, IL-10
TGF-β

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In the resolution of ARDS/ALI, (1) enter the intra-alveolar space in order to remove debris and (2) leading to (3). (4) will proliferated to replace pneumocytes. Endothelium will restore as a result of (5).

1- macrophages
2- release fibrogenic factors
3- fibrosis of alveolar wall
4- bronchiolar stem cells
5- proliferation of uninjured capillary endothelium

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list the phases exhibited histologically for ARDS

(DDD- diffuse alveolar damage)

1) acute / exudative phase, 0-4 days
2) organizing / proliferative phase, 4 days - 3 wks

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Acute phase, aka (1), occurs for (2) duration after onset of ARDS. It is characterized by a (3) gross appearance of the lungs, (4) of the interstitium and intra-alveolar spaces, and (5) of the alveolar epithelium in order to create (6), the main characteristic feature.

1- exudative phase
2- (days 0-4)
3- heavy, firm lungs
4- edema / hemorrhage
5- necrosis and sloughing of cells
6- Hyaline Membranes

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Organizing phase, aka (1), occurs for (2) duration after onset of ARDS. It starts with the proliferation of (3) and the organizing of (4) in order to establish (5) as the end result.

1- proliferative phase
2- (day 4 - week 3)
3- type II pneumocytes
4- fibrin exudates into Fibrosis
5- alveolar septal thickening

(Note- there is resorption of the Hyaline Membranes created in the acute phase)

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list the sign and symptom progression of ARDS/DAD

rapid onset, w/in wks
severe dyspnea –> tachypnea –> hypoxemia (refractory to O2 therapy)
cyanosis
respiratory failure

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how is ARDS/DAD investigated

1) diffuse bilateral infiltrates on radiograph (i.e. CXR)

2) V/Q mismatch (ventilation perfusion mismatch)

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ARDS/DAD has a (1) mortality rate, where (2) are factors that indicate a poor prognosis. If there is recovery, normal function may take up to (3- time) to return. It is treat with (4).

1- 40% (although decreasing)
2- advanced age, bacteremia/sepsis, progression to multi-system organ failure
3- 6-12 mos
4- supportive therapy

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Obstructive lung diseases are considered (1) disorders and affect the (2) parts of the lung. (3) is increased and is exhibited by (4) on spirometry.

1- airway disorders
2- trachea to terminal bronchioles
3- airflow resistance (limits expiratory rates on forced expiration)
4- reduced FEV1/FVC ratio

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Restrictive lung diseases are considered (1) disorders and affect the (2) parts of the lung. (3) are decreased and is exhibited by (4) on spirometry.

1- parenchymal disorder
2- respiratory bronchioles to alveoli / alveolar ducts
3- TLC, O2 diffusing capacity, other lung volumes, lung compliance
4- inc FEV1/FVC ratio (or normal)

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Obstructive lung disease spirometry, indicate inc, dec, or no change to the following:
TLC, FEV1, FVC, FEV1/FVC, FRC, RV

TLC- inc
FEV1- large dec
FVC- dec
FEV1/FVC- dec, <0.7 
FRC- inc
RV- inc

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Restrictive lung disease spirometry, indicate inc, dec, or no change to the following:
TLC, FEV1, FVC, FEV1/FVC, FRC, RV

TLC- dec
FEV1- dec
FVC- large dec
FEV1/FVC- inc/normal. >0.8
FRC- dec
RV- dec

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list the common obstructive lung diseases (hint- 4)

emphysema
chronic bronchitis
asthma
bronchiectasis

list the common restrictive lung diseases (hint- 4)

Acute Restrictive Disease: ARDS
Chronic Restrictive Disease: interstitial fibrosis, pneumoconiosis, granulomatous
chest wall deformities
neuromuscular

COPD affects (men/women) more

affects men and women equally

compare COPD to Asthma in terms of types of airflow obstruction

COPD (+ emphysema, bronchiectasis): loss of elastic recoil --> expiratory obstruction secondary to airway collapse // Irreversible Asthema: anatomic airway narrowing // Reversible

list the 3 reasons why chronic bronchitis and emphysema are grouped together in COPD

1) smoking is common trigger 2) overlapping features: many Pts present with features of both conditions 3) management of disease is similar

Emphysema is characterized by (1) occurring in the (2) part of the lung. It is accompanied by (3), but (4) is importantly absent.

1- abnormal permanent airspace enlargement 2- distal to terminal bronchioles 3- destruction of airspace walls 4- fibrosis

list the 4 types of Emphysema

- Centrilobular / Centriacinar - Panlobular / Pananinar - Para-septal / Distal Acinar - Irregular / Paracicatricial

In centrilobular, aka (1), emphysema, the (2) parts of the respiratory tract is affected and the (3) part of the lung is most affected. (4) is the common cause. (5) is a unique feature seen in individual lobules.

1- centriacinar 2- respiratory bronchiole (spares distal alveoli) 3- upper lungs 4- smoking 5- diseased and normal airspaces in same lobule

the most common emphysema is (1); (2) is the other common emphysema seen in practice

1- centrilobular / centriacinar 2- panlobular / panacinar

In panlobular, aka (1), emphysema, the (2) parts of the respiratory tract is affected and the (3) part of the lung is most affected. (4) is the common cause.

1- panacinar 2- respiratory bronchiole to distal alveoli affected 3- lower lung 4- AAT-1 deficiency

describe the 2 rare or less common emphysemas

Para-septal / Distal acinar: - next to atelectasis, along septa, margins of lobes, subpleural - more common in upper lobes - bullae may form - may lead to pneumothorax Irregular / Paracicatrical: surrounding scar, asymptomatic

Emphysema pathogenesis is based on (1). The (2) component of one is released from (3) after its infiltration, and functions to (4). The (5) factor plays a protective role in the lung. Therefore an increase in (2) or decrease in (5) can lead to (6).

1- imbalance of protease-antiprotease mechanism 2- protease 3- neutrophils (+ other leukocytes) 4- destroy lung tissue 5- antiprotease (ex. AAT, inhibits neutrophilic proteasses) 6- destruction of alveolar parenchyma

what are the 3 genetic factors that play a role in emphysema

A1AT deficiency: antiprotease deficiency, accompanied by abnormal protein accumulating in liver => chronic liver disease TGF-β gene polymorphism: dec signaling => inadequate elastin repair MMP gene polymorphisms: inc in emphysema

A1AT is a (1) type protein. Its abnormal form from (2) genotype found on chromosome (3), can have abnormal folding, leading to accumulation in (4) causing (5).

``` 1- antiprotease (in lung) 2- Pi-ZZ (Pi-MM is normal) 3- chr.14 4- liver 5- chronic liver disease ```

list the 3 major histological features of emphysema

- large alveolar spaces - alveolar septa destruction w/o fibrosis (septa is free flowing in air spaces) - elastin destruction in small airways => lung collapse during expiration, a functional obstruction

describe the symptoms or patient presentation for emphysema

- dyspnea, insidious/progressive onset - productive cough, variable - weight loss, hyperventilation and excessive puffing/panting => pink puffers

describe the signs seen on patient exam for emphysema

- barrel chest - prolonged expiration + pursed lips - sitting forward, hunched trying to squeeze air out of lungs

describe the spirometry results for emphysema

- inc lung volumes: TLC, RV - dec FVC, FEV1 (more so) => FEV1:FVC dec - dec DLCO (diffusing capacity for the lungs) due to loss of SA in lung for diffusion

describe the results of CXR and blood gases seen in emphysema

CXR: inc air trapping, flat domes of diaphragm Blood Gases: normal until later presentation (pink puffers), showing hypoxia, hypercapnia, respiratory acidosis

The main end progression of emphysema is (1), and (2) is the main complication seen.

1- irreversible lung damage | 2- Cor Pulmonale

describe how to diagnose chronic bronchitis

``` (clinical Dx) persistent productive cough: ->3 consecutive mos in a yr ->2 consecutive yrs -no other identifiable cause ```

Chronic bronchitis is mostly caused by......

inhaled irritants: smoking or urban dweller inhalation of SO2, NO2

describe the steps of pathogenesis for chronic bronchitis in response to inhaled irritants

1) submucosal gland hypertrophy => mucus hypersecretion 2) goblet cell metaplasia in bronchioles 3) smooth muscle hyperplasia + peribronchiolar fibrosis => small airway obstruction distally 4) inflammation: Tc cells, macrophages, neutrophils

describe the gross appearance of the lung in chronic bronchitis

- hyperemia and edema of mucous membranes | - excessive mucinous or mucopurulent secretions

describe the histological appearance of the lung in chronic bronchitis

- inc Reid index = thick mucus gland layer - goblet cell metaplasia - smooth cell hyperplasia - peribronchiolar fibrosis - irritant => squamous metaplasia

describe Reid index and its relationship to chronic bronchitis

Reid Index- ratio of thickness of mucous gland layer to thickness of wall between epithelium and cartilage -chronic bronchitis, >40%

describe the symptoms or patient presentation for chronic bronchitis

- persistent cough (see criteria in other slide) - progression to dypsnea upon exertion - cyanosis (blue bloaters)

describe the signs seen on patient exam for chronic bronchitis

- hypoxia, hypercapnea, cyanosis - persistent hypercapnea --> desensitizes PCO2 sensitive centers --> switch to respiration drive based on PO2 --> caution needed with O2 therapy

what are the 2 main complications that stem from chronic bronchitis

- secondary infections | - pulmonary HTN --> Cor Pulmonale

compare i) age of onset and ii) CXR results between emphysema and chronic bronchitis

i) age of onset Emphy: 50-75 y/o CB: 40-45 y/o ii) CXR Emphy: hyperinflation, small heart CB: prominent vessels, large heart

compare i) airway resistance and ii) elastic recoil between emphysema and chronic bronchitis

i) airway resistance: - Emphy: normal or slight inc - CB: inc ii) elastic recoil: i) Emphy: low ii) CB: normal

compare presentation of i) dyspnea, ii) cough, and iii) general appearance between emphysema and chronic bronchitis

Emphysema: i) severe and early dyspnea ii) late, scanty sputum in cough iii) pink puffer CB: i) mild and late dyspnea ii) early, copious sputum in cough iii) blue bloater

compare presentation of i) infections, ii) respiratory failure, and iii) cor pulmonale between emphysema and chronic bronchitis

Emphysema: i) occasional ii) terminal iii) rare, terminal CB: i) common ii) repeated (cyclic) iii) common