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Pulmonology- through fibrotic dz Flashcards

1
Q

What are the two types of pneumocytes, their functions

A

Type I- epithelium

Type II- make surfactant, lung stem cells

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2
Q

What are the functions of Macrophages in the lugns

A

Digest Antigens and clear surfactant

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3
Q

What cells make surfactant?
How is it released?
What happens to extra surfactant?

A

Surfactant is made by type II pneumocytes, stored in lamellar bodies which look like onions on EM.
The tubular myelin flattens out and gets absorbed onto the surface.
Excess surfactant is either reabsorbed and recycled by the type II pneumocytes or cleared by macrophages

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4
Q

How is pulmonary vasculature different than systemic vasculature

A

Pulmonary vasculature vasoconstricts in response to decreased oxygen to shunt blood to better oxygenated BV. Systemic BV do the opposite.

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5
Q

What are the 4 congenital anomalies

A

Pulmonary hypoplasia
Bronchogenic Cyst
Tracheo-esophogeal fistula
Bronchopulmonary sequestration

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6
Q

Pulmonary Hypoplasia- Si/Sx of presentation

Etiology

A

Presentation- neonate with respiratory distress

2 etiologies - Potters sequence and Diaphragmatic hernia

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7
Q

Potters Sequence

A

Renal Agenesis or lower urinary tract blockage –> dec amniotic fluid vol –> many issues but causes BILATERAL pulmonary Hypoplasia

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8
Q

Diaphragmatic Hernia

A

If diaphragm doesnt form correctly a loop of bowel may be in the thorax and inhibit the ability of the lungs to form.
Occurs more commonly on the left because the liver is on the right.
UNILATERAL
Would see bowel on a chest xray

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9
Q

Bronchogenic Cyst

A

Cystic space lined by bronchial epithelium so it fills with muccus.
Space occupying lesion leading to respiratory insufficiency
can potentially become infected

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10
Q

Tracheo-esophogeal fistula
Most common type
Presentation
dx

A

Anomalous connection between trachea and esophagus
most commonly it is atresia of the esophagus with proximal and distal connections to the trachea
Presents during the first feeding with coughing/ spitting and predisposes the pt to lipoid pneumonia
dx w/ barium x ray

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11
Q

Bronchopulmonary sequestration

A

segment of lung that is not connected to the main bronchus.
Has a different airway connection and different vasculature connection (comes off of thoracic aorta)
This is a space occupying lesion that is fatal if hemorrhages and is predisposed to infection

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12
Q

Atelectasis- definition

A

Collapse of alveoli

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13
Q

Atelectasis- complications

A

hypoxemia due to the poor ventilation

Risk of infection

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14
Q

4 types of atelectasis

A

Resorptive
Compression
Contraction
Neonatal

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15
Q

Resoprtive Atelactasis causes

A

bronchial obstruction or tumor that isolates part of the lung leading to all of the air in those alveoli to be absorbed
Tumor or muccus

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16
Q

Compression atelectasis- casues

A

External pressure on lungs that cause the lung to collapse ie tumor, pneumothorax, pleural effusion

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17
Q

Contraction type atelactasis- scar

A

scarring of lung from

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18
Q

Neonatal atelactasis- 2 causes

A

Premature babies can have atelectasis because surfactant production doesn’t start till 28 weeks and isn’t sufficient till 34 weeks. If a baby is going to be born prematurely can give the baby gluccocorticoids to stimulate surfactant production
Maternal Diabetes- insulin inhibits surfactant production.

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19
Q

Pulmonary edema- MCC

A

Left heart failure but can also be caused by infection, toxin, drowning

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20
Q

Pulmonary edema- pathogenesis

A

hemodynamic causes ie dec hydrostatic pressure ie LHF or dec oncotic pressure
Microvaacular injury- vessels become leaky ie drugs, toxins, infections

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21
Q

Pulmonary edema progression

A

Interstitial edema- lugns become stiff and pt has SOB
Alveolar edema- fluid enters alveolar airspace causes blocked ventilation and hypoxemia. As a side note this is a good medium for pneumonia- pneumonia secondary to edema
Chronic congestion- small hemorrhages allow blood to leak into the alv leading to the eventual formation of Heart Failure cells

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22
Q

What is a Heart failure cell

A

Hemosiderin laden macrophage from digesting RBCs

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23
Q

Pulmonary edema- Gross and microscopic appearance

A

Gross- frothy fluid

Microscopic- lungs are full of fluid but there are no PMNs thats how you know its not pneumonia

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24
Q

Pulmonary Edema- Si/ Sx

A

Symptoms- paroxysmal noctural dyspnea, orthopnea, SOC on exertion
Signs- Rales/ Ronchi, evidence of HF- JVD, hepatomegaly, pedal edema

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25
Pulmonary edema- appearance on CXR
kerley B lines and blunted costophrenic angles
26
Adult respiratory Distress Syndrome- (ARDS or DAD) MCC
Has a huge variety of causes- Sepsis is most common | also- shock, trauma, oxygen toxicity, drugs, gas, drowning, aspiration, trauma, pneumonia, chemo
27
ARDS- pathogenesis
Any of the causes can damage either the type I pneumocytes or the capillary endothelium. Damage to one quickly progresses to damage to the other
28
ARDS- first phase
Acute/ exudative phase has 3 charecteristics leaky endothelium --> pulmonary edema necrosis of type I pneumocytes --> hyaline membrane formation which serves as a barrier to gas diffusion damage to type II pnemocytes --> atelactasis
29
ARDS- second phase
repair/ organization | Type II pneumocytes flatten out and become type I pneumocytes.
30
ARDS- long term sequellae
Pts may end up with permanent and progressive fibrosis or may end up healed
31
ARDS- presentation
Acute onset of SOB + hx of inciting event - LHF | Hypoxemia will be unresponsive to supplemental oxygen
32
ARDS CXR
bilateral opacities
33
Pulmonary embolus- Etiology
Form in legs or pelvis normally in the femoral vein and make their way to the lung. Normally a thrombus but can also be air, bone marrow, or fat
34
Pulmonary embolus- risk factors
Virchow's triad- statis, hypergoagulable state, endothelial damage
35
Pulmonary embolism- pathology
Do not form an infarction but cause hemorrhagic necrosis due to the dual blood supply
36
Pulmonary embolism- presentation
may be asymptomatic but can cause coughing, SOG, dyspnea, pleuritic chest pain
37
What happens if you have several asymptomatic pulmonary emboli
Pulmonary HTN
38
What is the main complication of a saddle embolus
sudden death
39
Pulmonary HTN- etiology
Anything that increases the vascular pulmonary resistance
40
Pulmonary HTN- Causes
normally secondary to another cause. Heart: Mitral stenosis, LHF, L --> R shunt COPD can cause chronic hypoxia induced vasoconstriction Recurrent pulmonary emboli Idiopathic Genetic- BMPR2 mutation immune disease pulmonary fibrosis Primary is very rare can be from familial from BMPR2 mutation
41
Pulmonary HTN- pathology
This is the only time there will be atheroslcerosis in the pulmonary pulmonary arteries. So it is pathonemonic Onion skinning of the pulmonary arteries Plexiform lesions of the arteriolar wall means that the HTN was severe
42
What do plexiform lesions resemble
Glomeruli
43
Pulmonary HTN- presentation
start as asymptomatic but later pts have fatigue, SOB, dry cough, peripheral edema
44
what is a major complication of Pulmonary HTN
Cor Pulmonale
45
Spirometry measures for obstructive vs Restrictive
``` FEV1 FEV1: FVC peak expiratory flow RV TLC VC ```
46
Spirometry in restrictive dz
``` FEV1 dec or no change FEV1: FVC inc or no change peak expiratory flow: no change RV variable RLC dec VC dec ```
47
Spirometry in obstructive dz
``` FEV1 dec FEV1: FVC dec Peak expiratory flow dec RV inc TLC inc VC dec ```
48
Restrictive lung dz- main categories
Chest wall disorders ie Kyphosis, phrenic nerve impairment, diaphragmatic paralysis, kyphosis, pleural dz Interstitial dz which are split into ARDS and chronic interstitial lung dz
49
3 categories of chronic interstitial lung dz
Fibrotic Granulomatous Eosinophillic
50
Restrictive fibrotic lung dz
``` Cryptogenic organizing pneumonia Idiopathic pulmonary fibrosis Phenoconioses CT disorders radiation pneumonitis drugs ```
51
Granulomatous lung dz
Sarcoidosis | Hypersensitivity pneumonitis
52
Eosinophillic lung dz
Primary Eosinophilia
53
``` Characteristics of Restrictive lung dz: Etiology Pathogenesis Hx lung description ```
Disease is due to common and repeated insults These insults trigger T cells and B cells to activate macrophages which in genetically predisposed individuals activate fibroblasts for collagen deposition for repair Hx is very important- occupations, hobbies, travel, allergies, smoking lungs will be stiff with dec compliance and inc elasticity
54
Pneumoconiosis description examples
medium sized air particles in the air get trapped in by macrophages in the lung which cannot break them down and so they stimulate fibrosis mostly seen in upper lungs except for asbestosis examples- coal workers pneumoconiosis, silicosis, asbestosis, silicatosis
55
``` Coal Workers Pneumoconiosis hx etiology pathology versions ```
hx- coal workers or miners coal dust is fileld with coal, organics, and silica coal- nbd, but turns lung black organics and silica cause damage coal macule- build up of coal dust in the lung, its dark, and leads to centrilobular emphysema Progressive massive fibrosis- immune response to coal can lead to scar tissue formation and is bad
56
``` Silicosis- Hx pathology chest x ray microscopy ```
normally occurs in sandblasters or stone grinders requiring massive amounts of long term exposure the silica is ingested by macrophages which cant break it down --> cytokine release --> fibrogenic factor release leading to a fibrogenic response. once the silica is there there dz progresses even once exposure stops CXR- egg shell calcification of the hilar LN microscopic- dense ball of collagen with visible flecks of silica visible under polarized light. Will be able to see these on many specimens, not always pathologic
57
Acute silicosis
One massive exposure leads to fibrosis | this is very rare
58
Asbestosis- class of disease
pneumoconiosis
59
``` Asbestosis- normal population mecahnism causes what dz risk factors for what dz pathology ```
seen in- fire profing, insulation, roofing, building demolition mechanism- long thin fibers go to the lower lobes and may penetrate --> irritation causes- pulmonary and/ or pleural fibrosis Risk factor for- lung cancer, more so than malignant mesothelioma Pathology- ferrunginous bodies
60
Ferrunginous bodies- what dz what are they
Asbestosis | asbestos fibers encrusted with Fe and protein
61
Silcatosis- etiologic agent
Organic material + divalent cation
62
Caplan Syndrome
Rheumatoid pneumoconiosis severe immune response to pneumoconiosis + rhematoid nodules Often seen in pts with RA and coal workers lung
63
Usual interstitial penumonitis- idiopathic pulmonary fibrosis
repeated bouts of alveolitis triggered by an unknown agent
64
UIP- describe the fibrosis
patchy interstitial fibrosis- heterogenous age and severity worse in subpleural spaces and near fissures may result in honeycombing
65
UIP- pathophysiology
Unknown agent activates TGF beta 1 with 3 outcomes activates fibro and myofibroblasts inhibits calveolin in fibroblasts (normally calveolin is inhibitory) reduces telomerase activity causing early senescence and apoptosis
66
UIP- prognosis
poor response to anti-inflammatory tx | Poor prognosis
67
Crytogenic Organizing pneumonia or bronchiolitis obliterans organizing pneumonia)
nodules of organizing fibrous tissue in the small airways- bronchioles, alveolar ducts and alveolia
68
COP/BOOP- etiology
unknown
69
COP/BOOP- pathology
homogeneous lesions
70
COP/BOOP- prognosis
good response to steroids
71
Hypersensitivity pneumonitis- types
farmer's lung (moldy hay), Pidgeon breeder's lung (pidgeon stool), Humidifier lung
72
Hypersensitivity pneumonitis- mechanism
immune response to a known inhaled allergen
73
Hypersensitivity pneumonitis- are there eosinophils or IgE Abs
NO EOSINOPHILS OR IGE ABS IN HYPERSENSITIVITY PNEUMONITIS
74
Hypersensitivity pneumonitis- location of the lesion
Bronchiolar centered inflammation- granulomas and interstitial lymphoid infiltration
75
Hypersensitivity pneumonitis- tx
avoid further exposure
76
Sarcoidosis- definition | what rxn takes place
multisystem granulomatous dz | CD4 Th cells react to an unknown Ag
77
Sarcoidosis- what organs are normally affected
``` lungs and hilar LN skin eye liver spleen ```
78
What immune cells are involved in sarcoidosis
CH4 Th
79
what is the Ag is involved in sarcoidosis
unknown Ag
80
What is typical population for Sarcoidosis
AA female
81
symptoms of sarcoidosis
if they are symptomatic- cough, dyspnea, chest pain, fever, weight loss
82
appearance of sarcoidosis on x ray
well circumcised lesion
83
Pathology of sarcoidosis
non caseating perhilar granulomas- follow the lymphatics | ie non caseating granulomas that are next to the lymphatics
84
What is associated with sarcoidosis
elevated ACE elevated Vit D inc Serum Ca
85
Pulmonary Eosinophilia- definition
inc eosinophils in blood, sputum or lavage pulmonary infiltrates (results in fibrotic lung dz?)
86
Pulmonary Eosinophilia- types
Acute- rapidly progressive Simple- transient or treatable Chronic
87
Pulmonary Eosinophilia- associated with what dz
``` Asthma Aspergillus parasites Churg- Strauss Collagen- Vascular Idiopathic ```
88
Alveolar Proteionosis
Autoimmune dz of inc surfactant production
89
Alveolar proteonosis- pathology
Anti GM- CSF Ab --> dec clearance of surfactant by macrophages.
90
Alveolar proteinosis- microscopy
accumulation of surfactant in alveolar spaces | PAS +
91
Desquamative interstitial pneumonia- causes
related to smoking
92
Desquamative interstitial pneumonia- pathology
accumulation of macrophages in alveolar spaces leading to mild inflammation
93
Desquamative interstitial pneumonia- prognosis and tx
good porpoises | responds to steroids
94
What causes pulmonary fibrosis
long standing inflammatory damage by PMNs leads to pulmonary fiborsis
95
What dzs cause pulmonary fibrosis
Autoimmune dz- RA, scleroderma, pneumoconiosis, drugs, radiation, hypersensitivity penumonitis, sarcoidosis Primary dz may be- usual interstitial pneumonitis
96
Pulmonary fibrosis- mechanisms
Restrictive- lungs are stiff due to fibrosis leading to dec complacence --> Hypoxemic b/c fibrosis is barrier to gas exchange --> Hypertensive- b/c of inc in vascular resistance
97
Pulmonary fibrosis- lung findings
honeycomb lung- alternating aerated and fibrotic areas | microscopic- inflammation, fibrosis, hyperplasia of pneumocytes

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