L10- Pulmonary Pathology V Flashcards
define pulmonary edema
-accumulation of fluid in alveolar space
=> dec diffusion capacity, hypoxemia, and SOB
what are the two types of causes of pulmonary edema
- cardiogenic
- non-cardiogenic
In cardiogenic pulmonary edema, there is an increase in (1) that leads to leakage of fluid. (1) is mostly caused by (2).
1- inc hydrostatic pressure
2- L-sided CHF
what are the microscopic changes seen in Cardiogenic Pulmonary Edema
- hemosiderin-laden macrophages
- engorged alveolar capillaries
- intra-alveolar transudate
(no hyaline membrane formed in cardiogenic pulmonary edema)
list the 5 possible causes of noncardiogenic pulmonary edema
- 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)
Pulmonary Edema:
(1) and (2) are common Sxs
(3) is seen on CXR
1- SOB
2- productive cough with pink, frothy sputum
3- bilateral lung infiltrates
list the many risk factors for PEs
(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)
The effect of a PE is dependent on (1) and (2).
The consequences of pulmonary arterial occlusion are (3) and (4) [note- explain both].
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
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.
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)
(1) is a PE lodged in the main PA bifurcation. This will result in (2) from either (3) and or (4).
1- saddle embolism (large)
2- sudden death
3- hypoxia
4- acute RHF
Post-mortem, how is it determined that a PE is not a thrombus or a post-mortem clot
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
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).
1- dual blood supply (pulm. art., bronchial art.)
2- ischemic necrosis
3- cardiovascular compromised Pts
4- (red infarct) hemorrhagic, wedge shaped, peripheral infarct
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
1- asymptomatic
2- infarction
3- sudden death
4- pulmonary HTN, cor pulmonale
In a PE, infarction occurs as a result of (1) and presents as (2)
1- end artery occlusion + cardiovascular compromise
2- dyspnea
In a PE, sudden death occurs do to (1) or (2) secondary to an embolus described as (3) or (4)
1- acute cor pulmonale
2- shock
(60% of total pulmonary vasculature is obstructed by…)
3- large embolus (saddle embolus)
4- multiple simultaneous small emboli
list the non-thrombotic types of PEs
- air
- fat (via long bone fracture)
- amniotic fluid
- IV drugs
- bone marrow (via aggressive CPR)
define pulmonary HTN
Either:
- > 1/4th systemic pressure
- > 25 mmHg at rest
Note- comes in primary (rare) and secondary forms
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.
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
list some possible causes of secondary pulmonary HTN
- 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]
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).
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
Secondary Pulmonary HTN usually presents at (1) age. (2) and (3) are the major complications.
1- any age (reflects underlying pulmonary or cardiovascular disease)
2- respiratory insufficiency
3- RH strain
Pulmonary HTN morphological changes:
- (1) main elastic arteries
- (2) medium-sized muscular arteries
- (3) small arteries/arterioles
- (4) heart
1- atherosclerosis / atheromas
2- intimal cell and smooth muscle cell proliferation –> wall thickening
3- thickening, medial hypertrophy, reduplication of IEL and EEL
4- RVH
_______ is a key morphological change seen in primary / severe pulmonary hypertension (note- describe)
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
list the 3 main diffuse alveolar hemorrhage syndrome
- Goodpasture syndrome
- Idiopathic hemosiderosis
- Wegener’s granulomatosis (polyangiitis with granulomatosis)
diffuse alveolar hemorrhage syndromes present with….
(triad)
- hemoptysis
- anemia
- diffuse pulmonary infiltrates
Goodpasture syndrome affects the following organs…. (include specific disease in organ)
- kidneys: RPGN type I
- lungs: hemorrhagic interstitial pneumonitis
Goodpasture syndrome is caused by (1) leading to (2) in the affected organs
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
Goodpasture syndrome gross morphological changes
- heavy lungs
- red-brown consolidation
Goodpasture syndrom microscopic morphological changes:
- intra-alveolar (1) and (2)
- (3) change in alveolar walls
- (4) and (5) changes in later stages
1- intra-alveolar hemorrhage 2- intra-alveolar hemosiderin 3- patchy necrosis of alveolar walls 4- alveolar septal thickening 5- reactive type II pneumocyte hypertrophy
Goodpasture syndrome clinical findings
**-hemoptysis, anemia, pulmonary infiltrates
glomerularnephritis Sxs: hematuria, edema, uremia
Goodpasture syndrome investigations or Dx requirements
- CXR: focal consolidations
- Immunofluorescents: linear IgG deposits along basement membrane (glomerular BM, alveolar septa)
Goodpasture syndrome Tx
- plasmaphoresis
- immunosuppressive therapy
- Renal Transplant in severe cases
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.
1- polyangiitis with granulomatosis
2- autoimmune disease
3- PR3 / c -ANCA (anti-neutrophil Ab)
4- middle-aged / older men
Wegener’s Granulomatosis has the following 3 features (/ affected organs)
- Granulomas of lung and URT
- small-medium vessel Vasculitis
- Glomerulonephritis
list the clinical features of Wegener’s Granulomatosis (breakdown by organ and include % chance its involved)
- 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
Wegener’s Granulomatosis histopathology….. (many features)
- **-patchy necrosis (surrounded by healthy tissue)
- neutrophilic micro-abscess
- **-Granulomas: palisaded (fence like pattern), free Giant cells
- **-Vasculitis: necrotizing capillaries
- Necrotizing / Crescentic GN
Wegener’s Granulomatosis prognosis and Tx
- 80% mortality in 1 yr if left untreated
- Tx: steroids, cyclophosphamide, anti-TNF, Rituximab
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).
1- rare 2- children 3- lung involvement 4- r/o other disorder 5- steroids, immunosuppression (indicates immune system involvement)
compare and contrast organ involvement in:
- Goodpasture’s
- Wegener’s Granulomatosis
- Idiopathic Pulmonary Hemosiderosis
- G: renal, lungs
- WG: renal, lungs, URT, vasculitis
- IPH: lungs only
describe histopathology of Idiopathic Pulmonary Hemosiderosis
(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
