Chapter 15: Part 2 Flashcards
1
Q
What values on PFT indicate a restrictive disease?
A
- Lung compliance: reduced
- FVC: decreased
- FEV1/FVC: NL
2
Q
Name the interstitial (restrictive) lung diseases that cause fibrosis.
A
- Idiopathic pulmonary fibrosis (IPF)
- Non-specific interstitial pneumonia (NSIP)
- Cryptogenic organizing pneumonia (COP)
3
Q
A disease caused by irritiants and toxins that cause waves of inflammation (endothelial activation/injury), causing wound healing and interstitial fibrosis, damaging lung tissue in the alveoli “SCARING LUNG TISSUE”
A
IPF (Interstitial pulmonary fibrosis)
4
Q
Idiopathic Pulmonary Fibrosis is a disorder where waves of inflammation and injury lead to fibrosis.
What clinical findings will we see in a patient?
A
- CXR: Basilar infiltrates that lead to honeycomb lung
- SOB (dyspnea)
- Velco-like crackles on exam
- Restrictive patterns on pulmonary function test (decreased lung volume, DLco and FVC)
5
Q
Idiopathic Pulmonary Fibrosis (IPF) has no known cause. However, what populations do we see it highest in?
A
1. Increasing age (>50)
2. Smokers
3. Industrial areas
6
Q
What is the pathogenic mechanism of IPF?
A
- Env triggers + genetics damage epithelium.
- Injured/activated epithelium release factors => + interstitial fibroblasts.
- Interstitial fibroblasts cause abnormal signaling via P13K/AKT.
- Fibroblasts deposit collagen => interstitial fibrosis => respiratory failure
7
Q
Idiopathic pulmonary fibrosis is a disease most common in which age group?
A
>50
8
Q
Diagnosis of IPF requires what?
A
Classic findings on CT or biopsy of the lungs showing UIP (usual interstitial pneumonia).
9
Q
On biopsy, what do we see in IPF (Idiopathic Pulmonary Fibrosis)?
A
4 different findings because IPF occurs in a wave:
- Normal areas
- 2. Inflammed areas
- 3. Fibroblast foci
- 4. Peripheral honeycombing (cystic spaces of air)
10
Q
Microscopically, what is the hallmark of Idiopathic Pulmonary Fibrosis; there is coexistence of what?
A
- Patchy interstitial fibrosis***, which varies in intensity and age
- Both early fibroblast proliferation(fibroblastic foci) and late (collagenous) lesions, bc inflammation occurss in waves
- NL tissue
- Honeycomb fibrosis (cystic spaces)
11
Q
What is honeycomb fibrosis?
A
Cystic spaces of air that are lined with type 2 pneumocytes or bronchiolar epithelium
12
Q
What indicates that we have an early lesion formed by IPF?
A
Fibroblastic proliferation (fibroblastic foci)
13
Q
What is the course of IPF?
A
Progressive. If diagnosis is made, most people die 3-5 years after unless transplant
14
Q
What are potential treatments to IPF?
A
- Lung transplant
- Meds that stop fibrosis (tyrosine kinase inhibitors and TGF-B inhibitor)
15
Q
Why is it important to recognize pt’s with Nonspecific Interstitial Pneumonia?
A
Have much better prognosis than those w/ UIP
16
Q
What is a major morphological difference between the lesions of Nonspecific Interstitial Pneumonia and Idiopathic Pulmonary Fibrosis?
A
- In NSIP the inflammation/ pathy fibrosis are SAME stage of development and will be mild-moderate
- Thus, is NO heterogeneity.
17
Q
What 4 morphologial findings are does Nonspecific Interstitial Pneumonia NOT have?
A
- - NO fibroblastic foci
- - NO honeycombing
- - NO hyaline membranes
- - NO granulomas
18
Q
What is this?
A
Nonspecific Interstitial Pneumonia (NIP);
Uniform pattern of inflammation; interstitium is thick and has inflammatory cells; all same age
19
Q
Which population is most likely to be affected by Nonspecific Interstitial Pneumonia?
A
- Female NON-smokers in their 60s
20
Q
How do patients with Cryptogenic Organizing Pneumonia (COP) present and what is seen radiographically?
A
1. 50/60s
- Pneumomonia like symptoms (cough and SOB)
- Patchy SUBpleuralor OR peribronchial areas of airspace consolidation
.
21
Q
What is the hallmarkhistology seen with Cryprogenic Organizing Pneumonia, inflammation of the small airways (bronchioles)?
A
Intraalveolar fibrosis: plugs of loose, swirly CT (fibroblast foci called Masson bodies in alveoli, alveolar ducts and bronchioles, which is the bodies attempt at fibrosis and scar formation.
Walls will be okay (not harmed)
22
Q
How do we diagnose COP?
A
Diagnosis of exclusion: make sure not infection, toxins, other CT disorder.
23
Q
Does honeycombing or interstitial fibrosis occur in COP (crytogenic organizing pneumo)
A
NO
24
Q
Prognosis and Tx of Cryprogenic Organizing Pneumonia?
A
- Some recover spontaneously
- Most need oral steroids x 6 months +for complete recovery
25
Which 3 **autoimmune/CT disease** can **manifest** as i**nterstital lung disease?**
**1. RA**
**2. Systemic sclerosis**
**3. SLE**
26
If ILD occurs as a manifestation of RA, SLE, Systemic sclerosis, **how are they diagnosed and what is the prognosis?**
They are **not dx as PURE diseases**.
Outcome is linked to **UNDERLYING (SLE) condition**
27
Which 2 **chronic interstitial lung diseases** are categorized as _**granuloma-forming diseases**?_
* **1. Sarcoidosis**
* **2. Hypersentitivity Pneumonitis**
28
**What is this?**
**Granuloma inflammation:** nodular collection of **epithloid** **macrophages, giant cells** surrounded by a **ring of lymphocytes**
29
What is **sarcoidosis**?
Diisease characterized by the growth of tiny collections of i**nflammatory cells** that **form non-caseating/non-necrotizing granulomas** in any part of your body — most commonly the lungs and lymph nodes.
30
What is the most common clinical pattern/organs of involvement seen with **Sarcoidosis**?
* **_Bilateral hilar LN_**
* **_Lungs_**
Also occur in **eye** and **skin.**
31
What is the diagnostic feature of **Sarcoidosis**?
**Noncauseating granulomas** in the tissue
32
What immune cells are high in patients with **Sarcoidosis**?
**CD4+** T cells that secrete **TH1** cytokines like **IFN-y** and **IL-2.**
33
Which cytokine found in the **bronchoalveolar fluid** of pt with **Sarcoidosis** is a marker of disease activity?
**_TNF,_ (forms granulomas)** in **bronchoalveolar fluid =\> Sarcoidosis**
34
What are the stages of **_Sarcoidosis_**?
Which is most common?
Do they go in order?
* **Stage 0** (no abnormalities)
* **Stage 1** (hilar lymphandopathy)
* **Stage 2** (hilar lymyphandopathy and lung infiltration)
* Most common: 25-30% of cases have this.
* **Stage 3** (lung infiltration)
* **Stage 4** (Fibrosis)
* **NO, THEY DO NOT GO IN ORDER.**
35
Is **sarcoidosis** a progressive disease?
**_no_**
36
What is this and how do you know?
**_Sarcoidosis_**:
Different from other diseases that cause non-causeating granulomas **SHARD BORDER between the granuloma and normal tissue**
37
What are 2 characteristic morphological findings within giant cells of pt with Sarcoidosis?
* 1. **Asteroid bodies:** _stellate inclusions_ in giant cells
* 2. **Schaumann bodies:** concentration of _Ca2_+ and _proteins_ in giant cells
38
**What is this?**
**Schaumann bodies** (concentrations of Ca2+ and proteins), indicative of **Sarcoidosis**.
39
**_What is this?_**
**Asteroid bodies (**stellate inclusions), indicative of **sarcoidosis**.
40
A biopsy of where is a very good diagnostic source for Sarcoidosis?
**_Bronchial mucosa_**
## Footnote
ppl have alot of **granulomas** in the **bronchial submucosa**
41
Which LN's are most frequently affected by **Sarcoidosis**?
**_Hilar_** and **_mediastinal_**
42
Which 3 sites other than lungs and LN's are also commonly affected by Sarcoidosis and what is seen in each?
- **Spleen** - may be **enlarged**; granulomas often form **small nodules**
- **Liver** - may be **enlarged**; scattered granulomas often in **portal triad**
**- Bone marrow** - l**esions in phalangal bones of hands and feet**; small areas of bone resorption within marrow cavity w/ widening of the bony shafts
43
What 3 types of **skin lesions** may be encountered in **_Sarcoidosis_**?
- **Subcutaneous nodules**
- **Erythema nodosum**
- Flat, slightly red and scaling, resembling those of **SLE**
44
**Scattered granulomas** associated with **Sarcoidosis** may be found in the **liver**, most often where?
**Portal triads**
45
How are the **eyes** and **glands** affected in **Sarcoidosis**?
**Eyes**: sicca syndrome (dry eyes), iritis, uveitis
## Footnote
**Mikulicz syndrome**
46
Elevated serum levels of what enzyme and ion may be seen in **_Sarcoidosis_**?
**- ↑↑↑ ACE**
- **↑ 1ᾳ-hydroxylase** --\> **hypercalcemia**
47
Who does **Sarcoidosis** most commonly occur in?
1. Young patients **below 40**
2. 10-fold more likely in **African Americans**
48
What is the recovery of **Sarcoidosis**; how does death most often occur?
**60-70%** recover
**20%** have lung disease
Die from **cardiac, pulmonary or CNS involvement**
49
What is this?
**Hypersensitivity Pneumonitis**
Different from Sarcoidosis because we see granuloma, but it i**s NOT surrounded by NL lung tissue.**
50
What is **Hypersensitivity Pneumonitis?**
A spectrum of **immunological mediated interstitial diseases** caused by i**nhaled antigens** and **granulomas formation** in airways.
51
**Hypersensitivity pneumonitis** affects what structures in the lungs in constrast to asthma?
It AFFECTS **_ALVEOLAR WALLS_**
52
Diagnosis of **_Hypersensitivity Pneumoniitis_** requires **_history_**.
Why is it SO important?
- Disorders such as **_Pigeon breeder's lung_** (rxn to bird shit), **_Farmers lung_** (actinomycetic spores in hay) and **_Hot tub lung_** (rxn to Mycobacterium avium complex/MAC) are often missed unless a history is taken. -
53
Presence of **noncaseating granulomas** in 2/3's of pt's with **hypersensitivity pneumonitis** suggests what type of hypersensitivity rxn?
**Type 4 T-cell mediated**
54
The histologic changes of **hypersensitivity pneumonitis** are characteristically centered around which lung structures?
**Bronchioles**
55
What are 3 histomorphological changes seen with **hypersensitivity pneumonitis?**
1. **Interstitial pneumonitis** w/ lymphocytes, plasma cells, and MO
2. N**oncaseating granulomas**
3. **Interstital fibrosis w/ fibroblastic foci, honeycombing,** and obliterative bronchiolitis (late stages)
56
What are the 3 Interstitial Lung Diseases that are related to **smoking**?
1. **DSIP** (Desquamative Interstitial Pneumonia)
2**. RB-ILD** (Respiratory Bronchiolitis-Interstitial Lung Disease)
3. **LCH** (Langerhans-cell Histiocytes)
57
**What is this?**
**_LCH (Langerhan Cell Histiocytosis):_**
* **1. Langerhan cells** that look like **stars, forming lesions**
* **2. Eosinophils**
* **3. Varying fibrosis** and **cysts**
58
**What is this?**
**_DSIP (Desquamative Interstitial Pneumonia):_**
1. Alveolar **spaces** are **stuffed** with brown **"smokers MO"**
2. Mild inflammation and little fibrosis
59
**What is this?**
**How do we know?**
**_RB-ILD_**
1. **MO with a brown tint "smokey"** in _bronchioles_ (less than DSIP)
2. **Peribronchiolar metaplasia:** ciliated respiratory epitheliid cells move to alveolar septa
3. If severe, **fibrosis**
60
**Desquamative interstitial pneumonia** is MC in what age group associated w/ what risk factor; what are the sign's/sx's?
1. **Smokers** in between **40-50 YO**
2. **Gradual** onset of **SOB, dry cough** that lasts **weeks- months** and **clubbing of fingers**
61
**Treatment of DSIP and outcome likelihood?**
**1. Stop smoking and steroids**
**2. 95% survive**
62
**Respiratory bronchiolitis-interstitial lung disease** is part of a spectrum with desquamative interstitial pneumonia, except when does it present and what are the sx's like?
* **Presents earlier (30-40 YO)**
* **Less symptomatic**
63
In **RB-ILD,** where are the **MO** located?
**1st and 2nd order respiratory bronchioles**
64
**Langerhan Cell Histiocytosi**s is most common in who?
**Young smokers**
65
Histo in pt w/ **Pulmonary Langerhans Cell Histiocytosis** will show what?
What is a **complication**?
**Stellate shaped nodules** that **scar** =\> **trap air** =\> form a **cyst** that can **pop**. If it pops, causes a **pneumothorax**.
66
The langerhans cells of **Pulmonary Langerhans Cell Histiocytosis** will stain positive for what and negative for?
**Positive**: **S100** and **CD1a**
**Negative**: **CD68**
67
Which 2 smoking causing ILD are **reversible**?
* **1. RB-ILD**
* **2. LCH**
68
What is the **unique RLD?**
**PAP (Pulmonary Alveolar Proteinosis)**
69
What is **PAP (Pulmonary Alveolar Proteinosis)?**
**Accumulation of surfactant** throughout **alveoli** and **airs****_paces_** caused by either a :
* 1. **Defect in GM-CSF** (granulocyte MO colongy stimulating factor).
* 2. Dysfunction o**f pulmonary MO**
70
What is the most common cause of **PAP**?
**Autoimmune (90%);** patient has autoAB againist GM-CSF =\> dysfunction =\> a**lveolar MO cannot break down surfactant properly**
71
**Pulmonary Alveolar Proteinosis (PAP)** can be treated how if autoimmune vs. secondary?
- **Autoimmune** = **Sub cutaneous injections of GM-CSF**
- **Secondary** = Tx the underlying disorder
72
**Pulmonary Alveolar Proteinosis (PAP)** is characterized by what morphological changes within the alveoli?
1. P**roteineosous edema in alveolar spaces with surfactant protein**
2. MINIMAL inflammation
73
What is found in the alveolar precipitate of **Pulmonary Alveolar Proteinosis (PAP)** and what does it stain positive for?
* **Surfactant protein** and **cholesterol clefts**
* **+PAS** (periodic-acid Schiff)
74
What is the standard of care and provides underlying benefit for pt's with **Pulmonary Alveolar Proteinosis (PAP)** regardless of cause?
Whole-lung lavage
75
How do adult pt's with **Pulmonary Alveolar Proteinosis (PAP)** present (signs/sx's)?
**Cough** + **shit ton of sputum** w/ **chunks of jelly** material
76
In what disorder do you see this?
**PAP (pulmonary alveolar proteinosis)**: alveoli are filled with protein-lipid edema, but the walls of the alveoli are NL
77
**PAP** is most often associated with a mutation in what gene?
**_ABCA3_**
78
**Fractures** of which **bone** cause a high risk for **PE**?
**Hip**
79
How soon can **pulmonary infarct** be seen on CXR and **what is seen?**
* **12-36 hours**
* **Wedge-shaped d/t the radiating patterns of the vessels**
80
**Pulmonary infarction** d/t a PE is more likely in who?
**Ppl without adequate CV functioning**
81
In pt's w/ adequate CV function, which arterial supply supplies the lung parenchyma following PE; what is seen (hemorrhage/infarction) when a PE is thrown?
* **Bronchial** arterial supply
* Ptient will **hemorrhage,** but **NOT INFARCT**
82
How does pulmonary infarction appear morphologically in the **early stages** and w**hat is seen on the apposed pleural surface?**
* Begins **hemorrhagic: raised red-blue area.**
* **Fibrinous exudate**
83
What occurs **48 hours afte**r pulmonary infarct and what are the morphological changes as time progresses?
- **RBC lyse** --\> **infarct** becomes **paler** =\> r**ed-brown** as hemosiderin is produced
- Over time, **fibrosis** begins at margins as **a gray-white** peripheral zone ---\> contracted **scar**
**SUMMARY: Goes from _hemorrhagic =\> fibrosis_**
84
What is the difference in where small and large PE go to?
* **Large PE:** main pulmonary artery, branches or at the birfurcation of the pulmonary trunk, creating a saddle embolism
* S**mall PE:** go out more peripherally =\> cause hemorrhage or infarct.
85
Which type of **emboli** are often seen in pt's who **die** **after chest compressions performed during CPR?**
**Small bone marrow emboli;** however, it **DOES NOT** mean he died **BECAUSE of** it.
86
How can we tell if someone died from a clot?
**Prescence of lines of zahn**
87
**PE** have what 2 deleterious pathophysiologic consequences?
**1. Respiratory compromise:** area is **ventillated**, but **NOT perfused.**
**2. Hemodynamic compromise: increased resistance** to pulmonary BF
88
**Blood clots** that occlude large pulmonary arteries are alsmost always d/t _____ .
What is the usual souce?
**Emboli**
**DVT**
89
**PE's** usually occur in what people?
Ppl with a predisposed condition that **makes them more likely to clot.**
90
Majority of infarcts associated w/ **PE affect which lobes** **and occur as (single/multiple) lesions?**
**Lower lobes**
**Multiple lesions**
91
Besides PE, what else can block a vessel?
1. Blood clot (thromboembolism)
2. Bone marrow/ fat
3. Cancer
4. Septic emboli
5. Air
6. Foreign material
7. Parasites
92
What is this?
**Talc embolism** are often seen in **IV drug users**
93
What is this?
Septic embolus: Infective materal **rom heart valve that** causes a local infection, **forms** an **abcess** (c**yst).**
94
In what condition do septic emboli occur?
**Endocarditis**
95
**Septic emboli** can go to what other places, besides the lung and cause what?
* **1. _Skin_: janeway lesions**
* **2. _Retina_: roth spots**
* **3. _Nail bed_: Splinter hemorrhage**
96
If a pulmonary infarct is caused by an infected embolus, there may be i**ntense _neutrophilic_ inflammatory rxn**, and these lesions are knowna as what?
**Septic infarcts**
97
Vast majority of PEs are (60-80%) \_\_\_\_\_\_\_
Minority (5%): \_\_\_\_\_\_\_
Remaining \_\_\_\_\_\_\_
* **Silent (60-80%)**
* Cause **acute cor pulmonale, shock, death d/t saddle**
* Pulmonary infarction
98
How is **PE** usually **DX**?
**Spiral CT angiography**
99
**Pulmonary HTN** is defined as **mean pulmonary artery pressure** ≥\_\_\_\_\_\_mmHg at rest
**greater than 25** mmgHg at rest (cannot measure with BP cuff)
100
What are the 5 classification of **pulmonary HTN** as classified by the WHO?
1**. Pulmonary arterial HTN** - primarily vascular disease \*\*\*
2. pHTN **d/t left-heart failure**
3. pHTN **d/t chronic pulmonary parenchymal disease or hypoxemia \*\*\***
4. pHTN **d/t to _thromboembolic_ pulmonary HTN**
5. **Multifactorial**
**\*\*\*Most common**
101
**Mutation** of _____ is indicated in **primary pulmonary HTN.**
**BMPR2** mutation on **Chr2q33 =\> endothelial cell** and **vascular SM proliferation =\> thickening and occlusion of pulmonary vasculature.**
102
All forms of **pulmonary HTN** are associated with what 3 things?
1. **Hypertrophy of medial SM layer** in pulmonary and elastic arteries
2. **Atherosclerosis** in pulmonary a.
3. **RV hypertrophy**
103
Pulmonary HTN affects which BVs?
**Arterioles and small arteries**, causing m**edial hypertrophy** and in**timal fibrosis**
104
**What is this?**
**_Plexiform lesions,_ tangled knot of BVs** that is **VERY characteristic** of **long-standing, severe pulmonary HTN**
105
What are the 3 **pulmonary hemorrhage syndromes?**
* **1. Goodpasture**
* **2. Granulomatosis with polyangiitis (Wegners)**
* **3. Idiopathic pulomonary hemosiderosis**
106
What finding in patients is common in **_all_** pulmonary hemorragic syndromes?
Pts will have bright red **hemoptosis** bc hemorrhaging though lungs. All will have **hemosiderin laden MO.**
107
What is **Goodpasterues syndrome?**
Disorder where **autoAB against noncollagenous domain of collagen 4** destroy _BM in glomerulus_ and **_aveoli_**.
108
In **Goodpasture Syndrome** autoantibodies destroy the glomerular BM in renal glomeruli and pulmonary alveoli giving rise to what disease in each system?
**- Rapidly progressive glomerulonephritis (RPGN)**
**- Necrotizing hemorrhagic interstitial pneumonitis**
109
Which **sex** and **age group** is most often affected by Goodpasture Syndrome?
* **_Males_ in _teens or 20_ who are _a_****_ctive smokers_**
110
What is unique about immunofluorescene of lung in Goodpatures?
**Linear** pattern of deopsition on **BM** of lung d/t **anti-BM AB** with **RBC present**
111
What is the **histology** of the damage to alveolar walls in **Goodpasture Syndrome?**
1. **Focal necrosis**
2. **Intraalveolar hemorrhage**
3. h**emosiderin-laden macrophages**
112
What type of **hypersensitivity reaction** is **Good Pastures**?
**_Type 2_**
113
What are the diagnostically important features of **Polyangiitis w/ Granulomatosis** (Wegener), **in contrast to Sarcoidosis?**
* **GPA (Wegners): _Capillaritis_** and **poorly formed granulomas**
* **Sarcoidosis**: **Rounded**, **well defined granulomas**
114
**What is this?**
**GPA**: white is inflammed lung and in the middle is coagulated blood; capilartitis and poorly formed granulomas
Lungs are more prone to bleed.
115
Which biopsy technique may provide the only tissue necessary for diagnosis of Polyangiitis w/ Granulomatosis (Wegener)?
**Transbronchial biopsy**
116
What is **pneumonia**?
**Pneumonia** is any infection to the lung parenchyma/
117
lHow can we classify **pneumonia**, a pulmonary infection.
**1. Clinical setting**
2. Anatomic distribution **(broncopneumonia or lobar pneumonia)**
118
**Community-Acquired Pneumonia** can be due to what 2 things?
**1. Bacterial**
**2. Viral**
119
What **patterns** can **bacterial** **pneumonia** produce
**1. Bronchopneumonia: patchy consolidation**
**2. Lobar pneumonia: consolidated (air fluid level in cystic space)**
120
Lobar pneumonia has an **acute progression.**
## Footnote
**Name the staqes.**
121
What are the **4 stages of the inflammatory response** for **lobar pneumonia**?
1) **Congestion**: Lung is boggy and red d/t vascular engorgement and intra-alveolar fluid with neutrophils
2) **Red hepatization**: Congestion with massive oozing of alveolar spaces w/ lots of neutrophils; RBCS, fibrin =\> lung is red, firm and airless lk liver
3) **Gray hepatization:** RBC distengrate, leaving fibrin + inflamm cells
4) **Resolution**: fibrosis and macrophage clean-up
122
What is a complication of **lobar pneumonia?**
**1. Abcess/cyst, walling itself off.**
**2. Emphyma (pleural space filled with blood)**
**3. Bacteremia.**
123
What bacteria cause **typical pneumonia?**
1. **1. S. pneumo**
2. **2. H. influenza**
3. **3. S. aereus**
4. **4. K. pneumoniae**
5. **5. P. aeruginosa**
124
What are 2 acute phase markers **made in the liver** that are specific for bacterial infection and can be useful in dx **community-acquired bacterial pneumonia**?
**- CRP**
**- Procalcitonin**
125
What is this?
**Lobar pneumonia**
126
How do we clinically diagnose someone with lobar pneumonia?
**XRAY**
127
What is the **most common** cause of **community-acquired pneumonia?**
**_Strep. pneumoniae_**
128
How do we dx someone with c**ommunity-acquired bacterial pneumonia** d.t **Streptococcus pneumoniae?**
**_Sputum_ culture that shows _neutrophils w gram (+) lacet-shaped diplococci_**
129
What is this?
**_CABP due to Strep pneumo:_ Gram + lancet shaped diplococci in pairs and chains**
130
Vaccination for **Strep Pnemo i**s recommeded for who?
1. **Infants**
2. Patients **over 65 YO**
3. Pts with **Resp disease/smokers**
131
What is a cause of common cause of **DANGEROUS/VIRULENT pneumonia** in **children**?
What is recommended?
**H. Influenza**
**_Vaccinate kid 5 years or younger_ for H. Influenza type B**
132
Pneumonia caused by what bacteria is a **pediatric emergency** due risk of **acute epiglottitis w/ high mortality rate?**
**H. influenzae**
133
What is the pulmonary consolidation associated w/ **H. influenzae** typically like?
**Lobular and Patchy**
134
Which patient population is especially susceptible to bacterial pneumonia by **Moraxella Catarrhalis?**
**Elderly**
135
What is the **most common cause** of **gram-negative bacterial pneumonia** and who does it most commonly affect?
- **Klebsiella pneumoniae**
- Debilitated and malnourished people, particularly **chronic alcoholics**
136
What type of sputum is characteristic of **Community Acquired Klebsiella pneumoniae?**
Thick, sputum w alot of mucus and blood (**currant jelly sputum)**
137
**Pseudomonas aeruginosa** is a common cause of pneumonia in whom and in what setting?
* **-Hostpital acquired**
* -**Cystic Fibrosis** and **immunocompromised**
138
What bacteria cause **atypical ("walking) pneumonia?**
* **1. Mycoplasma pneumoniae**
* **2. Legionella pneumopila**
* **3. Chlamydia pneumonia**
* **4. Chlamydia psittaci**
139
What is the difference between **typical** and **atypical pneumonia?**
* **_Onset:_**
* **_Symptoms:_**
* **_CXR:_**
* **_Age group:_**
**_Typical pneumonia:_**
* Onset: **_Abrupt_**
* Symptoms: **_Mainly respiratory_**
* CXR: C**onsolidated (not patchy)**
* Age group: Y**oung children or older adults**
**_Atypical pneumonia_**
* Onset: **_Slower onset_**
* Symptoms: **_Systemic sx (may not have resp sx)_**
* CXR: **_Patchy infiltrates_**
* Age group: **_Older children - teens- young adults_**
140
Which is **atypical pneumonia a**nd which is **typical**?
Left is typical bc it is consolidated
Right is atypical bc it is patchy and has lines
141
Which **bacteria** cause of **pneumonia** live in **warm freshwater** (i.e., AC units, misters, hot tubs); **what does it live inside?**
**- Legionella pneumophila**
**- Lives in amoebas**
142
What type of **pneumonia** affected more than 1 person at once at the American LEgion because they were drinking **_FRESHWATER_**
**Legionella pneumophila induced pneumonia**
143
What are the 2 common modes of transmission for L**egionella pneumophilia?**
**1. Airborne**
**2. Drink contaminated water**
144
What is the gram stain and morphology of **Legionella pneumophila?**
Gram **_NEGATIVE_** **_bacillus_**
145
**Mycoplasma pneumoniae** infections are common in which age groups and occur most often how?
**- Children and young adults**
- **Sporadically** or as l**ocal epidemics** (i.e., schools, military camps, prisons)
146
What is unique about **Mycoplasma pneumonia?**
ISmallest free-living organism and has **NO CELL WALL so it is EASILY TREATED**
147
Which pattern of bacterial pneumonia is often **multilobular** and frequently **bilateral** and **basal**?
**bronchopneumonia**
148
What are causes of **community-acquired _viral_ pneumonia?**
* **1. Influenza (H1N1)**
* **2. SARS**
* **3. RSV (respiratory syncytial virus)**
149
**Tissue destruction** and **abscess formation** as a complication of pneumonia are most common associated w/ what 3 organisms?
1. **- Type 3 pneumococci**
2. **- Klebsiella**
3. **- S. aureus**
150
What are the **2 virulence factors** of **H. Influenza virus**, allowing it to cause infections; function of each?
1. - **Hemagglutinin**: allows for attachment to cells; via **sialic acid residues**
2. - **Neuraminidase (NA)**: allows release of replicated virus from cells by cleaving **sialic** **acid residues** on **hemagglutinin**
151
What is the action of **Tamiflu**?
Tamiflu prevents the spread of H. influenzae by **blocking neuraminidase,** preventing cleaving of sialic acid residues =\> prevents virus from being able to infect other cells.
152
153
What is the difference between **antigenic drift** and **antigenic shift in regards to H. Influenza?**
Which causes **epidemic** and **pandemic?**
**Antigenic drift:** point mutations to HA/NA antigens =\> virus is slightly altered =\> spread increases =\> epidemic but is similar enough to the original virus to allow for some immunity
Ex. seasonal flu
**Antigenic shifts:** antigenic RNA segments of HA/NA switch between species through recmobination =\> forming a new virus that no one is immune to =\> **pandemic**
154
If **influenza viru**s gains entry into pneumocytes what are the cytopathic changes that it can elicit which contribute to its pathogenesis?
- **Inhibits Na+ channels** --\> electrolyte + H2O shifts = **fluid accumulation in alveolar lumen**
- **Inhibits** host **mRNA translation and activates caspases** --\> **cell death via apoptosis**
155
What was the last pandemic we had?
H1N1 swine flu in 2009 in MX: bird flu + human =\> swine flu in pigs.
Transmitted from pigs =\> humans via inhalation
156
Which bacteria is most commonly found as cause of **superimposed bacterial infection on a viral pneumonia?**
S. aereus
157
Which family of viruses does **SARS, outbreak that began in ChInA** belong to?
**Coronavirus**
158
Why is **SARS** a distinct coronavirus in terms of infection?
Coronavirus causes many URI, however, **SARS** is a **LRI** that can spread t/o body.
159
What are the most common cause of **PNEUMONIA** in **NEONATES**?
**Bacterial** pneumonias from **mom**
* **1. Group B strep**
* **2. Gram (-) bacilli**
* **3. Listerialis**
160
What are 5 major **viral** causes of **pneumonia** in **children \>1 month**?
- **_RSV_**\*\*
- Parainfluenza virus, Influenza A and B, Adenovirus, Rhinovirus
161
What are 4 major **bacterial** causes of **pneumonia** in **children \>1 month?**
* **- S. pneumoniae**
* **- M. catarrhalis**
* **- H. influenzae**
* **- S. aureus**
162
In **older children and adloscesncents,** how do causes of pneumonia change?
1. **Less** likihood of **RSV**
2. Also consider **M. pneumoniae** and **C. pneumonia (a.trpical)**
163
**Respiratory syncytial virus (RSV)** belongs to what family of viruses?
**Paramyxoviruses**
164
What are the sx of **RSV in infants?**
1**. Rhinorrhea and wheezing, scarycough**
## Footnote
**2. Wheezing, SOB and fast breathing**
**3. Cyanosis bc mucus builup and bb cant breath**
165
How does **respiratory syncytial virus (RSV)** spread from the **nasopharynx to lower respiratory tract;** what is the effect of viral replication on this process?
* 1. RSV virus attaches and infects epithelial ells in the nose.
* 2. Cells slough off and carry the virus to the LRT.
* 3. Viral replication causes epithelial cells to slough off, i**nvasion by inflamm cell**s, **increase mucus production** and **damage to cilia, causing mucus to build** up.
166
Which family of viruses does _**Human Metapneumovirus (MPV)** b_elong to?
**Paramyxovirus**
**Cause pneumo in _very young, very old_ and _immunocompromised_**
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**_ALL VIRAL INFECTIONS_ = _SIMILAR MORPHO CHANGES_**
What are the 3 major morphological changes seen in upper respiratory viral infections?
**1. Mucosal hyperemia** (too much blood in vessels)
2. Infiltration o**f submucosa w lymphocytes and plasma cells**
3. **Make too much mucus**
168
How does the **inflammatory rxn** (location and immune cells) differ between **bacterial vs. viral causes of pneumonia?**
- **Bacterial** = **Neutrophillic** inflammation **INSIDE alveoli**
- **Viral** = **Lymphocytic** inflammation in **interstitiam (WALLS of alveoli)**
169
When **viral pneumonia** is complicated by **ARDS** what is seen lining the **alveolar** **walls**?
**_Pink hyaline membranes_**
170
Patient comes in with pneumonia. How do we determine if viral or bacterial?
What is the **onset**, **fever**, and **infiltration** seen on CXR like for **bacterial vs. viral causes of pneumonia?**
**_Bacteria_**: **abrupt** onset with **high fever,** **lobar/considated**
**_Viral_**: **gradual** onset with **no/low feve**r and **diffuse infiltrates** on XR
171
In _________ pneumonia, **epidemics are common.**
What are the exceptions?
**_Viral= common._**
**Bacterial pneumo** is **not assx w epidemics e_xcept Legionells and pertussis_**
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What do you hear on **lung exam** in **Bacterial vs. Viral pneumo?**
## Footnote
**involve pleura?**
* _Bacterial_: **crackles (d/t parenchyma involvement)**
* **may involve _pleura_**
* _Viral_: **Wheezes (d/t _airway involvement)_**
* **will not involve pleura**
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Bacterial pneumo **_is/isnt_** assx with bacteremia
Viral pneumo **_is/isnt_** assx with viremia
Bacteria: may
Viral: not
174
Which is **bacterial pneumo** and which is v**iral pnemo?**
L: bacteria bc intraalveolar neutrophil inflammation
R: viral bec interstitial lymphocytic inflamm
175
**What is this?**
**RSV**, a paramyxovirus (they all look the same on histo): collection (syncitium) of nuclei
176
Bacterial causes of pneumonia are not typically associated w/ epidemics, except those caused by what 2 types?
**- Legionella**
**- Pertussis**
177
A red, flat to slightly bumpy (**maculopapular**) rash that **starts on the forehead** and spreads **to** the face, neck, torso, and finally the **feet** is associated with what virus?
**Measles (paramyxovirus)**
178
What is the gram stain and shape of **Bordatella pertussis?**
**Gram _negative_ _bacillus_**
179
What are the 4 major virulence factors of **_Bordetella_ _pertussis_?**
* **- Pertussis toxin:** activates G proteins --\> ↑**cAMP**
* - **Extracytoplasmic adenylate cyclase**: "weakens" immune cells
* - **Filamentous hemagglutinin:** binding to ciliated epithelial cells
* - T**racheal cytotoxin:** kills ciliated epithelial cells
180
While working the pediatric ED, you see a child with a **_sore throat_ and _fever_.** There is a **dark exudate on the child's _pharynx_,** which appears darker and thicker than that of strep throat; which bacteria is most likely responsible and what its gram stain/shape?
**- Corynebacterium diptheriae**
- **Gram** **+** **rods** - very **pleomorphic** and **club-shaped**
181
If Corynebacterium diptheriae is suspected why must you NOT scrape the pseudomembranes formed on the pharynx; may lead to what?
- May **bleed** and **systemic absorption** of lethal **EXOTOXIN** will increase
- Can cause: ***myocarditis*** and **neural _involvement_**( peripheral nerve palsies**, Guillan Barre-like syndro**me, and palatal paralysis/cranial neuropathies
182
Who gets l**ung abcesses?**
**-Complication of pneumonia** d/t S. aureus and K. pneumoniae (both cause lobar pneumo)
**-Ppl who aspirate:** Chronic alcoholics, elderly ppl who have had strokes, anaerobic bacteria
183
**Aspiration pneumonia** occurs most frequently in whom; what are the risk factors?
1. **D_ebilitated pts (elderly w stroke),_ u_nconscious pts,_ _chronic alcoholics_** who aspirate gastric contents/repeated vomitting.
Often times will have a l**ung abcess**
184
What type of damage is seen with **a_spiration pneumonia_ a**nd what is its typical course?
Often **_necrotizing_** and pursues _**sudden** clinical course_
- Frequent cause of **death**
185
In those who survive aspiration pneumonia what is a frequent complication that arises?
**_Lung abscesses_**
186
What are the 4 **anaerobic** oral **bacteria** commonly recovered in the culture of pt with **aspiration pneumonia?**
**- Bacteroides**
**- Prevotella**
**- Fusobacterium**
**- **_Pepto_**streptococcus**
187
Lung abscesses due to aspiration are more common on which side of the lung and are most often (single/mutliple)?
**Right** side of lung; most often **single**
188
What is the cardinal histologic change in all **lung abscesses?**
**Suppurative** destruction of the **lung parenchyma** within the central area of **cavitation**
189
Which pattern of tuberculosis arises in a nonimmune host vs. previously sensitized host?
- Non-immune = primary TB
- Previously sensitized = secondary TB
Primary infection can create a primary complex (localized caseation) that can heal or become latent. Upon reactivation, pt developes secondary TB.
190
Secondary pulmonary tuberculosis classically involves which area of the lungs?
**APEX** of o**ne or both lungs**
191
**_What is this?_**
**TB**: **_caseating granuloma_** "dust" necrosis surrounded by giant cells
192
Which chronic lung disease puts people at a particularly high risk for tuberculosis?
**Silicosis**
193
**Ghon complex** seen in primary TB is a combination of what?
**Parenchymal lung lesion** (Ghon focus- center undergoes caseous necrosis) + **LN involvemen**t
194
What type of pneumonia is most often a l**ocalized lesion in the immunocompetent p**t**, with or without LN involvement;** typically featuring a **granulomatous** **inflammatory** rxn?
**Chronic pneumonia**
195
What **funguses** cause **_Chronic pneumonia?_**
* **1. Histoplasma**
* **2. Blastomycosis**
* **3. Coccidiomycosis**
## Footnote
**Which tend to be endemic in diff areas.**
196
**Histoplasma capsulatum** infection is acquired via inhalation of what?
**Dust or soil** contaminated with **bird or bat shit**, which has small spores in it (**microconidia**)
197
**Histoplasma capsulatum** is endemic where?
- Endemic --\> **Midwest (Ohio/Mississippi Rivers) and Caribbean**
198
**Histoplasma capsulatum** typically causes what type of infection with what type of immune response; what is seen on CXR?
**Subclinical infection** that forms **granulomas w/ coagulative necrosis =\> coalesce by fibrosis and _calcify_. On XR looks like _coin lesions/calcifications_**
199
What is the morphology (aka shape) of the yeast forms seen with **Histoplasma capsulatum?**
**Thin-walled** yeast w/ **"pumpkin seed"** morphology
200
Granulomas in the lungs associated with Histoplasma capsulatum may liquefy and form cavities in pt's with what underlying disease?
**COPD**
201
How is the morphology of the disease caused by Histoplasma capsulatum different in immunosuppressed individuals?
- Causes f**ulminant disseminated histoplasmosis**
- Granulomas **do NOT form**
- Instead there are focal accumulations of **phagocytes FILLED w/ yeasts t**hroughout the body
202
**Blastomyces dermatitidis** is a soil-inhabiting **dimorphic** fungi that is endemic where in the US
- **Central** (**Ohio and Mississippi river valleys**) and **SE** United States
203
How do the lung lesions of ***Blastomyces dermatitidis a***ppear morphologically in a normal host?
**- Suppurative granulomas**
- **MO find it hard to kill, so yeast persists, leading to increase in neutrophiles**
204
What does **Blastomyces dermatitidis** yeast look like that distinguishes it from other fungi?
- **Round** w/ **B**ROAD-**B**ASED **B**UDDING
- **THICK**, double-contoured **cell-wall**
205
What parts of the body does Blastomyces Dermatitidis affect?
S**kin and larynx**, RARELY spreading
## Footnote
**Epithelial hyperplasia**
206
Where is Coccidioides immitis endemic and in the lungs what does the infection cause?
- Endemic in **SW** United States and **Mexico**
- Formation of granulomas w eosinophils
thick wall, dont bud, filled w endospores
207
What are the signs/sx's of pulmonary and cutaneous infection with **Coccidioides immitis?**
Sx are often subclinical and self-limited. Can disseminate, esp in immunocom
## Footnote
- Fever + cough + pleuritic chest pain
- **Erythema nodosum** or **erythema multiforme (the San Joaquin Valley fever complex)**
208
Which pt population and ethnic groups are at the highest risk for the rare disseminated infection caused by **Coccidioides immitis?**
What type of lesions dominate in the disseminated disease?
**- Filipino and African Americans**
**- Immunosuppressed**
**- Purulent lesions dominate**
209
A pt with known lung disease presents with **recurrent hemoptysis,** a tissue sample from the lungs shows this; what is the diagnosis and how do you know?
- **Aspergillosis**
**- Septate hyphae** w/ acute-angle branching (40 degrees)
210
A tissue biopsy of a pt with granulomatous lung disease shows this; what oganism is this most consistent with?
**Coccidioides immitis**
211
Opportunistic infections rarely cause infections in NL hosts, but can cause lifethreatening pneo im immunocompromised.
## Footnote
**List 3 common causes of _diffuse pulmonary infiltrates_ that can cause _pneumonia_ in an _immunocompromised person?_**
- CMV
**- Pneumocystis jiroveci**
- Drug rxn
212
Opportunistic infections rarely cause infections in NL hosts, but can cause lifethreatening pneumo im immunocompromised.
List 5 common causes of **focal pulmonary infiltrates** that can cause **_pneumonia_** in an **immunocompromised person?**
1. **- Gram-_negative_ bacterial infections**
2. **- S. aureus**
3. **- Aspergillus**
4. **- Candida**
5. **- Malignancy**
213
Which chronic lung disease puts people at a particularly high risk for **tuberculosis**?
**Silicosis**
214
Which critical mediator released from TH1 cells both in LN's and the lung enables macrophages to contain M. tuberculosis infection?
Which immune cells form the granulomas and caseous necrosis seen in M. tuberculosis infection?
Macrophages activated by IFN-γ in M. tuberculosisinfection differentiate into what?
**IFN-y**
**Th1**
**Epitheliod histiocytes that aggregate to form granulomas; some may aggregate to form giant cells**
215
Pt's with RA treated with what type of drugs are at an increased risk for tuberculosis reactivation?
**TNF** **antagonist**
216
Secondary pulmonary tuberculosis classically involves which area of the lungs?
**APEX** of **one** or both lungs
217
TB is inhaled. Once infected, TB resides and proliferates in MO.
VF: mycolic cell wall, cord factor (protect bacteria from being destryoed by forming granulomas by releasing TNF-a =\> activate other MO. How does this protect? Walls off bacteria in granuloma, created by newly activated MO).
Sulfatides prevent phagocyte=lysosome fusion, allowing the TB to survive in granuloma.
Infected =\> primary infection. 3 different outcomes: healed latent infection, systemic infection (milary TB) or reactivate and become secondary TB later in life.
**Primary pneumo will form a _small caseating granuloma_ in the lower or middle lobes**. After the lesion heals, becomes fibrotic and calcifies, forming a Ghon complex (calficiation of hilar LN and caseating granuloma in lung parenchyma). Most cases will heal and form a fibrotic scar.
TB can become progressive primary TB =\> become bactermic and become milary TB (affects several organs like liver and spleen).
Or if latent TB reactivates =\> secondary TB d/t immunosupression by downregulating TNF-a release. TNF-a keeps the inflammation contained. Bc gone=\> reactivation. What happens if to give a pt a TNF-a inhibitor? IF GIVING ANY1 a TNF-a inhibtor (Inflixomab), DO A PPD SKIN TEST. **_Reactivation will affect the UPPER LOBES_** and form caseating granumolas (also affecting kidney). Sx of rx: skin, hemoptysis and night sweat, cachexia.
What is the disosrder called in TB infects bones? **Potts disease**
**Granulomas are formed to wall off infection (activated MO).**
218
tx TB?
RIPE
1. Rifampin
2. Isoniazid
3. Pyrazinamide
4. Ethambutol
219
**Primary tuberculosis** almost always begins in which organ and what is seen morphologically as sensitization develops?
Lower part of upper lobe, middle lobe or lower lobe
**Ghon complex formation**: calficiation and caseous necrosis of granuloma and hilar LN
220
What is this?
Caseating granuloma seen in TB
221
What is the **most common opportunistic infection of AIDS patients, which causes pulmonary problems?**
**Pneumocystis jiroveci**
222
As a general rule of thumb which organisms causing **pulmonary infections** in HIV pt's are more likely with a CD4 count \>200, 50-200, and \<50?
- \>200 = **bacterial** and **TB** infections
- 50-200= **Pneumocystis jiroveci (carinii)**
- \<50 = **CMV**, **fungus**, and **Mycobacterium avium complex (MAC**
223
What is most likely seen on CXR of pt with HIV infected with **Pneumocystis jiroveci?**
**Diffuse** ***BILATERAL*** interstitial **infiltrates**
- Can also be **focal** or show nothing!
224
What is the characteristic morphology of the yeast, **Pneumocystis jiroveci?**
**Cup-shaped** yeast forms
225
What is the hallmark finding of **MAC infections** in patients with immunosuppressed (i.e., HIV, elderly, transplant); morphology of the organism?
- Abundant **ACID-FAST** **_bacilli_** **INSIDE macrophages**
- **Thin** mycobacteria seen as slender red forms
226
Which type of organism is the most common cause of **pulmonary infection** in the **early post-transplant** period (first few weeks)?
**BACTERIA**
227
Since a**cute rejection** following a lung transplant has a **similar** picture to **infections**, **diagnosis relies on what?**
**_Transbronchial_ biopsy**
228
What does **acute rejection** of a **lung transplant** look like on histo?
**Mononuclear infiltrates** around **small vessels, submucosa of airways** or both
