L10- Pulmonary Pathology V

Question 1

define pulmonary edema

Answer 1

-accumulation of fluid in alveolar space

=> dec diffusion capacity, hypoxemia, and SOB

Question 2

what are the two types of causes of pulmonary edema

Answer 2

• cardiogenic

- non-cardiogenic

Question 3

In cardiogenic pulmonary edema, there is an increase in (1) that leads to leakage of fluid. (1) is mostly caused by (2).

Answer 3

1- inc hydrostatic pressure

2- L-sided CHF

Question 4

what are the microscopic changes seen in Cardiogenic Pulmonary Edema

Answer 4

• hemosiderin-laden macrophages
• engorged alveolar capillaries
• intra-alveolar transudate

(no hyaline membrane formed in cardiogenic pulmonary edema)

Question 5

list the 5 possible causes of noncardiogenic pulmonary edema

Answer 5

• ADRS (endothelial damage => inc permeability)
• dec oncotic pressure (some form of protein loss)
• high altitude
• pulmonary embolism
• Neurogenic (various traumatic events => inc stress and SNS => blood shift to pulmonary circulation and volume overload)

Question 6

Pulmonary Edema:

(1) and (2) are common Sxs
(3) is seen on CXR

Answer 6

1- SOB
2- productive cough with pink, frothy sputum
3- bilateral lung infiltrates

Question 7

list the many risk factors for PEs

Answer 7

(pulmonary embolism)

• immobility (bed rest, long flight)
• surgery (orthopedic)
• severe trauma (burns, fractures)
• CHF
• oral contraception
• disseminated malignancy (immune CAs)
• Hypercoaguability disorders (factor V leiden, protein C/S, antithrombin III deficiency, etc)

Question 8

The effect of a PE is dependent on (1) and (2).

The consequences of pulmonary arterial occlusion are (3) and (4) [note- explain both].

Answer 8

1- embolus size
2- cardiopulmonary status of Pt

3- hemodynamic compromise: inc pulm. art. press. +/- vasospasm and release of mediators (TX-A2, 5-HT) [+ RHF]

4- respiratory compromise: due to ischemia of pulmonary parenchyma

Question 9

In a PE there is an important rise in pressure in (1).

Hypoxia may appear in a PE, usually secondary to (2), (3), or (4) responses.

Answer 9

1- acute rise in R heart Ps

2- atelectasis: reduced surfactant production in ischemic areas
3- blood shunted to normally hypoventilated areas
4- R –> L shunt thru patent foramen ovale (30% of population)

Question 10

(1) is a PE lodged in the main PA bifurcation. This will result in (2) from either (3) and or (4).

Answer 10

1- saddle embolism (large)
2- sudden death
3- hypoxia
4- acute RHF

Question 11

Post-mortem, how is it determined that a PE is not a thrombus or a post-mortem clot

Answer 11

Embolus- unattached to wall, lines of Zahn are present

Thrombus- attached to vessel wall, lines of Zahn are present

Post-Mortem Clot: not attached to vessel wall, NO lines of Zahn

Question 12

Because the lung parenchyma has a (1) property, it is rare for (2) to occur during a PE, and will mainly occur in (3) patients. (2) is described as (4).

Answer 12

1- dual blood supply (pulm. art., bronchial art.)
2- ischemic necrosis
3- cardiovascular compromised Pts
4- (red infarct) hemorrhagic, wedge shaped, peripheral infarct

Question 13

PE clinical features:

(1) most Pts, 60-80%
(2) is seen in 10-15% of Pts
(3) is the worst manifestation, 5%
(4) <3% will have these chronic complications secondary to recurrent emboli

Answer 13

1- asymptomatic
2- infarction
3- sudden death
4- pulmonary HTN, cor pulmonale

Question 14

In a PE, infarction occurs as a result of (1) and presents as (2)

Answer 14

1- end artery occlusion + cardiovascular compromise

2- dyspnea

Question 15

In a PE, sudden death occurs do to (1) or (2) secondary to an embolus described as (3) or (4)

Answer 15

1- acute cor pulmonale
2- shock

(60% of total pulmonary vasculature is obstructed by…)
3- large embolus (saddle embolus)
4- multiple simultaneous small emboli

Question 16

list the non-thrombotic types of PEs

Answer 16

• air
• fat (via long bone fracture)
• amniotic fluid
• IV drugs
• bone marrow (via aggressive CPR)

Question 17

define pulmonary HTN

Answer 17

Either:

• > 1/4th systemic pressure
• > 25 mmHg at rest

Note- comes in primary (rare) and secondary forms

Question 18

Primary Pulmonary HTN usually presents in (1) type patients [include age/sex]. There is a defect in (2) gene/protein which usually regulates / inhibits (3) which is responsible for (4) function.

Answer 18

1- young women
2- BMPR receptor type 2 (bone morphogenetic protein receptor)
3- TGF-β (BMPR binds ligands in its pathway to inhibit its effects)
4- smooth muscle proliferation

Question 19

list some possible causes of secondary pulmonary HTN

Answer 19

• chronic obstructive or interstitial lung disease
• recurrent PEs
• heart disease (mitral stenosis, L->R shunt)

[OR due to:
dec in vasodilatory agents
inc in vasoconstrictive agents
GF production]

Question 20

Primary Pulmonary HTN usually presents in (1) type patients [include age/sex]. It will present with the following symptoms: (2). (3) are the more serious long-term complications. It is treated through (4).

Answer 20

1- young women
2- syncope, fatigue, exertional dyspnea, chest pain
3- severe respiratory insufficiency, cyanosis, death via RHF (cor pulmonale)
4- vasodilators, antithrombotic agents, lung transplantation

Question 21

Secondary Pulmonary HTN usually presents at (1) age. (2) and (3) are the major complications.

Answer 21

1- any age (reflects underlying pulmonary or cardiovascular disease)
2- respiratory insufficiency
3- RH strain

Question 22

Pulmonary HTN morphological changes:

• (1) main elastic arteries
• (2) medium-sized muscular arteries
• (3) small arteries/arterioles
• (4) heart

Answer 22

1- atherosclerosis / atheromas
2- intimal cell and smooth muscle cell proliferation –> wall thickening
3- thickening, medial hypertrophy, reduplication of IEL and EEL
4- RVH

Question 23

_______ is a key morphological change seen in primary / severe pulmonary hypertension (note- describe)

Answer 23

Plexiform lesions: multiple lumina w/in small artery at branch point from larger artery

-essentially hyper-perfused artery develops multiple capillaries, some of which extend outside the lung

Question 24

list the 3 main diffuse alveolar hemorrhage syndrome

Answer 24

• Goodpasture syndrome
• Idiopathic hemosiderosis
• Wegener’s granulomatosis (polyangiitis with granulomatosis)

Question 25

diffuse alveolar hemorrhage syndromes present with….

Answer 25

(triad)

• hemoptysis
• anemia
• diffuse pulmonary infiltrates

Question 26

Goodpasture syndrome affects the following organs…. (include specific disease in organ)

Answer 26

• kidneys: RPGN type I

- lungs: hemorrhagic interstitial pneumonitis

Question 27

Goodpasture syndrome is caused by (1) leading to (2) in the affected organs

Answer 27

1- Abs against α3 chain in collagen IV (basement membrane collagen)

2- linear deposition of IgG along basement membrane on glomerular basement membrane and alveolar septa

Question 28

Goodpasture syndrome gross morphological changes

Answer 28

• heavy lungs

- red-brown consolidation

Question 29

Goodpasture syndrom microscopic morphological changes:

• intra-alveolar (1) and (2)
• (3) change in alveolar walls
• (4) and (5) changes in later stages

Answer 29

1- intra-alveolar hemorrhage
2- intra-alveolar hemosiderin
3- patchy necrosis of alveolar walls
4- alveolar septal thickening
5- reactive type II pneumocyte hypertrophy

Question 30

Goodpasture syndrome clinical findings

Answer 30

**-hemoptysis, anemia, pulmonary infiltrates

glomerularnephritis Sxs: hematuria, edema, uremia

Question 31

Goodpasture syndrome investigations or Dx requirements

Answer 31

• CXR: focal consolidations

- Immunofluorescents: linear IgG deposits along basement membrane (glomerular BM, alveolar septa)

Question 32

Goodpasture syndrome Tx

Answer 32

• plasmaphoresis
• immunosuppressive therapy
• Renal Transplant in severe cases

Question 33

Wegener’s Granulomatosis, aka (1), is a (2) type disease mainly involving (3) component in its pathogenesis. It will mainly affected (4- age/sex) individuals.

Answer 33

1- polyangiitis with granulomatosis
2- autoimmune disease
3- PR3 / c -ANCA (anti-neutrophil Ab)
4- middle-aged / older men

Question 34

Wegener’s Granulomatosis has the following 3 features (/ affected organs)

Answer 34

• Granulomas of lung and URT
• small-medium vessel Vasculitis
• Glomerulonephritis

Question 35

list the clinical features of Wegener’s Granulomatosis (breakdown by organ and include % chance its involved)

Answer 35

• Lungs/URT: pneumonitis with nodules / cavitary lesions (95%), chronic sinusitis (90%), mucosal ulcerations of nasopharynx (75%)
• Renal: hematuria, proteinuria (nephritis: oliguria, uremia)
• rashes, myalgias, fever, articular involvement, neuritis

Question 36

Wegener’s Granulomatosis histopathology….. (many features)

Answer 36

• **-patchy necrosis (surrounded by healthy tissue)
• neutrophilic micro-abscess
• **-Granulomas: palisaded (fence like pattern), free Giant cells
• **-Vasculitis: necrotizing capillaries
• Necrotizing / Crescentic GN

Question 37

Wegener’s Granulomatosis prognosis and Tx

Answer 37

• 80% mortality in 1 yr if left untreated

- Tx: steroids, cyclophosphamide, anti-TNF, Rituximab

Question 38

Idiopathic pulmonary hemosiderosis is a (common/rare) disorder found more in (adults/children) that is limited to (3) involvement. Its diagnosis requires (4) and its treatment includes (5).

Answer 38

1- rare
2- children
3- lung involvement
4- r/o other disorder
5- steroids, immunosuppression (indicates immune system involvement)

Question 39

compare and contrast organ involvement in:

• Goodpasture’s
• Wegener’s Granulomatosis
• Idiopathic Pulmonary Hemosiderosis

Answer 39

• G: renal, lungs
• WG: renal, lungs, URT, vasculitis
• IPH: lungs only

Question 40

describe histopathology of Idiopathic Pulmonary Hemosiderosis

Answer 40

(similar to Goodpasture’s w/o associated renal disease and no anti-BM Ab)

• intra-alveolar hemorrhage
• intra-alveolar hemosiderin
• patchy necrosis of alveolar walls
• alveolar septal thickening
• reactive type II pneumocyte hypertrophy