Chapter 14 Flashcards
1
Q
What is the final common result of ischemia medial damage and marfans that can lead to the formation of an aneurism?
A
Cystic medial degeneration
2
Q
Abdominal aortic aneurisms are characterized by severe atherosclerosis of the aorta, and are frequently covered by what?
How can we detect?
A
- Mural thrombus
- Pulsating mass in the abdomen
3
Q
Aortic dissections occur when blood enters a defect in the intima and travels though the tissure between the aortic media. Who do aortic dissection most commonly occur in?
A
- 1. HTN males 40-60 YO
- Pts with CT disorders (Marfan)
4
Q
What is the main risk factor in a aortic dissection?
A
HYPERTENSION
5
Q
How does a patient with an aortic dissection present?
A
Sudden onset of severe CP that radiates to the back between the scapula and moves downward as the disscection progresses.
6
Q
What is most commonly confused with a acute MI?
A
Aortic dissection
7
Q
Who are AAA more common in?
A
Men
Smokers
60s
8
Q
Thoracic aortic aneurism are most often due to what?
A
- HTN
- Marfans: Defective fibrillin and overactive TGF-B, which weakens elastic tissue
9
Q
Note that we often see __________ in pts with aortic dissections.
A
cystic medial degeneration
10
Q
- Large vessel vasculitis includes aorta and branches (2)
A
- Temporal (Giant cell) arteritis
- Takayasu arteritis
11
Q
- Med vessel vasculitis affects muscular arteries, which supply organs
A
- Polyarteritis nodosa (PAN)
- Kawasaki disease
- Buerger disease
12
Q
- Small vessel vasculitis affects arterioles, capillaries and venules.
A
- Wegener granulomatosis
- Microscopic polyangiitis
- Churg-Strauss syndrome
13
Q
Notice that __________ is the only vasculitis that involves the aorta.
A
giant cell arteritis
14
Q
_______ requires eosinophils and is associated with asthma and atopic individuals.
A
Churg-Strauss
15
Q
________ requires neutrophils and is associated with orogenital ulcers.
A
Behçet disease
16
Q
_________ is associated with young male smokers.
A
Buerger disease
17
Q
If we see a patient and ID immune complexes in the vascular wall, what do we do?
A
Assume DRUG HYPERSENSITIVITY!
Stop the drug => vasculitis should resolve.
18
Q
Antineutrophil cytoplasmic antibodies (ANCA) are what?
A
autoAB against enzymes inside of cytoplasm of neutrophils, monoxytes and endothelial cells.
ANCA bind to neutrophils => activate them => cause the release of ROS and cytokines => inflame and damage endothelium => vasculitis
19
Q
Do vasculitis assx with ANCA have immune complexes?
A
NO. ANCA produces a pausi-immune response => attack things inside of neutrophils but do not form immune comples. THUS THESE LESIONS WILL NOT HAVE IMMUNE COMPLEX DEPOSITS
20
Q
PR3-ANCA/c-ANCA) target PR3 and is assx with what vasculitis?
A
Wegners
21
Q
Anti-MPO/p-ANCA is asociated with what vasculitis?
A
1. Microscopic polyangiitis
2. Churg-Strauss
22
Q
Measuring ANCA can tell us
A
how serious the disease is.
23
Q
Immune complex vasculitis is seen in
A
- Lupus
- Drug hypersensitivity
- Secondary to exposure to infection (Hep B => Polyarteritis nodosa)
24
Q
_________ are the most common causes of aortitis…
A
Giant Cell Arteritis and Takayasu
25
**Non-infectious vasculitis** occur how?
**Immune-mediated**
26
In what vasculitis does fibrinoid necrosis occur?
**Small-cell vasculitis**
27
* Most common form of vasculitis in **older patients (\>50 YO); F**
**Giant cell arteritis**
28
**Giant cell arteritis s**tems from an immune response by which cells and causes inflammation of arteries where?
* **CD4+**
* **Proximal aorta, carotid artery and branches**
29
Histo in Giant cell arteritis
* **CD4+ cells** are going to infiltrate the **internal elastic lamina and the media.**
* MO invate the media and form **multinucleated giant cells** inside of a **granuloma**.
* **Fragmented ILA** and **intimal thickening** =\> **narrow lumen** and **ischemia**
30
Inflammation in Giant cell arteritis is what?
**Patchy and segmental**
31
Sx in giant cell arteritis
* 1. Temporal artery: HA, facial pain on temporal area
* 2. JAw claudication
* 3. Opthalmic artery: visual disturbacne and double vision
32
**Takayasu arteritis** most commonly affects who
**Japanese (AZN) females under 40.**
33
Takayasu Arteritis (aka pulseless disease)
**Granuloma vasculitis** of **medium** and l**arge arteries** characterized by **ocular disturbances** and marked **weak pulses/ low BP** in the **UE's**
34
Takayasu arteritis has similar histological findings as those seen in giant cell arteritis (T-cells infiltrate of media and intimal elastic lamina, granulomas and intimal thickening, patchy and focal), except involves which vessels?
Medium and large vessels: Aortic arch and nearby (pulmonary (1/2), carotid and renal); **NOT TEMPORAL**
35
Since many features of **Takayasu arteritis** are shared with **Giant Cell arteritis,** the distinction between the 2 is primarily made how?
- **AGE** of the pt
- **Takayasu** = **younger**(\<50 yo)
- **Giant cell** = **older** (\>50 yo)
36
Sx in Takayasi
* Initial sx are non-specific (fatigue, WL, fever, etc)
* Later
* **Vascular symptoms:** “pulseless disease” because the lumen of **brachiocephalic** / **carotid** / **subclavian arteries** narrows =\> decrease BP =\> w**eak/absent UE/carotid puls**es
* **Bruits** form d/t turbulent blood flow
* Vascular symptoms then go down to the abdominal aorta and distal aorta
* **Pulmonary HTN**
* **MI**
* **Renal HTN**
* **Limb claudication** (pain w walking that is relieved w rest
* **Ocular disturbances:** visual disturbances, retinal hemorrhage, blindness
.
37
**Polyarteritis Nodosa (PAN)** is what
**fibrinoid necrosis** that involves multiple m**edium sized arterie**s and involves many organs (SPEC RENAL), **but NOT the lungs** (pulmonary vessels).
38
**PAN** most commonly affects the \_\_\_\_\_, but not the \_\_\_\_\_
MC: **KIDNEYS**
**_NOT_**: **lungs**
39
In 1/3 of pt's with **polyarteritis nodosa** the vasculitis is attributable to chronic infection by which virus?
**Immune-complexes** composed of what?
HBsAg and anti-HBsAg = Chronic HBV
40
What process does PAN cause vasculitis
most likely **immune complex mediated**
41
List the 4 vessels most often involved in **Polyarteritis Nodosa** in descending order of frequency.
1. **- Kidneys**
2. **- Heart**
3. **- Liver**
4. **- GI tract**
42
Histo of **PAN**
* Polyarteritis nodosa results in a **beads on a string ap**pearance that are **_not circumferential_.** Because PAN causes a**cute flares and relapses , it makes lesions at varying stages.**
* Early lesions are ****_s_**egmental, transmural (occur across the entire wall= PAN) necrotizing inflammation** at **BRANCH POINTS** (NO GRANULOMAS)
* Often time we can see immune complexes in wall of BV.
* As the wall weakens, they can create an aneurism.
* Lesions that heal =\> **fibrosis**, creating the beads on a string.
43
In PAN are sites of inflammation **circumferential**
**NO**
44
**Classic Polyarteritis Nodosa** is characterized by what morphological pattern of inflammation?
**Segmental TRANMURALnecrotizing inflammation** of small- to medium-sized arteries that can heal and **fibrose**
45
During acute phase of Polyarteritis Nodosa the transmural inflammation of arterial wall shows a mixed inflammatory infiltrate and is often accompanied by what type of necrosis?
**Fibrinoid necrosis**
46
**Polyarteritis Nodosa** is typically a disease affecting which age group?
Typical course of the disease?
* **Young**
* **Remitting and episodic**
47
Does Polyartertis nodosa form granulomas?
**NO**
48
What is a "classic" presentation (signs/sx's) of **Polyarteritis Nodosa?**
**1. Rapidly accelerating HTN**
**2. Abdominal pain and bloody stools**
**3. Myalgias and peripheral neutitis**
49
Tx of PAN
**immunosupression**; because immune complex mediated
50
**Kawasaki disease**
who does it affect
Acute arteritis of the coronary arteries (medium) that affect **kids and infants under 4**
51
Clinical significance o**f Kawasaki Disease** stems from its predilection for which artery?
Leading to what?
Coronary artery
Aneurisms =\> thrombosis or rupture =\> MI
52
What is the morphology of the vasculitis seen in Kawasaki Disease; differs from PAN how?
Dense **transmural** inflammatory infiltrate w/ **LESS** prominent **fibrinoid necrosis** than in PAN
53
Kawasaki disease typically presents with what signs/sx's?
\*First Aid Mnemonic
**CRASH and burn**
- Conjunctival injection
- Rash (polymorphous -\> desquamating)
- Adenopathy (cervical LN enlargement = mucocutaneous LN syndrome)
- Strawberry tongue (erythema and blistering of oral mucosa)
- Hand and feet are red
- Burn = FEVER for longer than 5 days
54
If **Kawaksaki Disease** is recognized early in its course treatment with what will sharply reduce the risk of symptomatic coronary artery disease?
**IV immunoglobulin and Aspirin**
55
Microscopic polyangiitis generally affects which type of vessels?
Capillaries + small arterioles and venules
56
How do the age and distribution of the lesions seen in Microscopic Polyangiitis differ from PAN?
Tend to be of the same age and are distributed more widely
57
**Microscopic polyangiitis** affects vessels of many organ systems, but which 3 systems are most common?
* -**Kidneys** (90% have necrotizing glomerulonephritis)
* -**Lungs** (pulmonary hemorrhage)
* -**Skin** (palpalble pupura
*
58
What are other names for micrscopic polyangittis
1. **Hypersensitivity vasculitis**
2. **leukocytoclastic vasculitis**
59
MPA can be features of what 3 disorders
1. Henoch-Schonlein purpura
2. - Essential mixed cryoglobulinemia
3. - Vasculitis assoc. w/ CT disorders
60
What is seen on histo of **MPA**?
* **P-ANCA will be destroying neutrophils (thus, many fragmented PMNs) =\> cause _segmental_** _**fibrinoid** **necrosis**_
61
Does MPA form granulomas?
**NO**
62
Which immune cells are seen infiltrating in microscopic polyangiitis, many of which are undergoing apoptosis and giving rise to the term leukocytoclastic vasculitis?
NEutrophils (Fragment PMN)
63
What are the major clinical features (signs/sx's) seen with **microscopic polyangiitis?**
- Hemoptysis (c**ough up blood)**
- Hematuria and Proteinuria (p**ee blood/proteins)**
- Bowel pain or bleeding
- Muscle pain or weakness
- Palpable cutaneous purpura
64
What vasculitis is a pulmonary-renal syndrome?
**MPA**
65
What does MPA stain + for and and what other vasculitis has palpable purpure
1. **p-ANCA**
2. **Churgg-Strauss**
66
Q: How is MPA different from PAN?
* Small BV; lesions are same age and more widely distributed
67
What is **Churgg Straus disease** often misdx with?
**1. Allergies**
**2. Allergici rhinitis**
**3. Asthma**
**CHURGG!**
68
What is Churgg Strauss disease?
_small-vessel_ **_necrotizing_** vasculitis that involves mainly the **_heart_** _and **lungs**. !!!_
69
**Churgg-Strauss syndrome** may have inflammation that resembles PAN or microscopic polyangiitis with the addition of what 2 things?
**1. HIGH AMOUNT OF EOSINOPHILS**
**2. NECROTIZING GRANULOMAS**
70
What is the primary manifestation of Churg-Strauss Syndrome affecting the kidneys?
**_Focal_** and **_segmental_** glomerulosclerosis
71
What is the primary manifestation of **Churg-Strauss Syndrome** affecting the myocardium?
Clinically significant why?
Cardiomyopthy: MCC of death in this
72
* Cutanous involvement of Churg-Strauss Syndrome presents with what?
Palpalpe purpura, also seen in MPA
73
**Behçet Disease** is a vasculitis that affects **small-medium** vessels that presents w what triat?1
**1. Oral ulcers**
**2. Genital ulcers**
**3. Uveitis**
74
Which HLA haplotype is associated with **Behcet Disease?**
**HLA-B51**
75
Vessel inflammation i**n Behcet Disease** is due to what inflammatory cell type?
Recruited by which T cells?
**Neutrophils**, recruitsed by T**H17 cells**
76
Mortality seen in **Behcet Disease** is related to what 2 complications?
**1. Neruo involvemtn**
**2. Rupture of aneurism**
77
MPO-ANCA's (P-ANCA) are found in what 2 types of non-infectious vasculitis?
1. MPA
2. Churgg Strauss
78
C-ANCA (PR3) is assx with
**weCners**
79
Granulomatosis with Polyangitis (Wegener granulomatosis) is a c-ANCA (PR3-ANCA) + ***_necrotizing vasculitis_*** characterized by a triad of what?
* 1**) Necrotizing granuloma**s of upper or lower respiratory tract, or both
2) **Necrotizing or granulomatous vasculitis** most prominenetly in lungs
3) ***Focal necrotizing, often CRESCENTERIC, glomerulonephritis***
80
Granulomatosis with polyangiitis is a form of hypersensitivity mediated by what?
* **Inhalation of airborne toxin** =\> **T-cell mediated hypersensitivy** =\> develops c-ANCA (PR3-ANCA) =\>
81
Which sex is more often affected by **granulomatosis with polyangiitis** and at what age?
men 40 YO
82
What are the classic features (signs/sx's) of **granulomatosis with polyangiitis? (4)**
1. - Persistent **pneumonitis** w/ **bilateral nodular and cavitary infiltrates**
2. **- Chronic sinusitis**
3. - N**asopharyngeal ulcerations**
4. - Evidence of **renal disease**
83
How serious is **granulomatosis with polyangiitis** if left untreated?
**rapidly; 80% will die**
84
which vascilitis has
## Footnote
**granulomas** with **GEOGRAPHIC** patterns of **central necrosis?**
**WEGNERS (GPA)**
85
What may be seen radiographically in the lower respiratory tract of pt w/ granulomatosis with polyangiitis as multiple necrotizing granulomas coalesce?
**_centrally cavitating lesions_**
86
Which renal lesions may be seen early on in granulomatosis with polyangiitis?
Eventually progress to what in late stages?
**- Focal and segmental glomerulonephritis**
**- Crescenteric glomerulonephritis**
87
**_Thromboangiitis Obliterans (Buerger Disease)_** is STRONGLY assx with tobacco **smoke** in ppl **YOUNGER 35 YO** and affects **TIBIAL and RADIAL As**
* **Israeli**, **Indian**, and **Japanese** groups who have a hypersensitivity to tobacco products.
* **_Segmental_ thrombosi**s w/ **microabcesses** formed =\> total obliteration of distal vessel lumens =\> distal ischemia of the of distal extremities
* **Tibial and radial arteries**=\> loss of the fingers and toes.
* Inflammation of affected vessels can spread to veins and nerves =\> neuropathy =\>
* **Cold-induced Raynaud phenomenon**
* **Intermittent claudicatio**n (pain with XRCISE that stops with rest)
* **Superficial phlebitis (venous inflammation)**
* **Ulcers and gangrene of fingers and toes.**
* Causes **severe pain, even at rest**.
* Histo:
* Segmental, granuloma formation with thrombus that may have microabsesses that obliterates lumen of vessel.
88
Which 3 organisms can directly invade vessels and cause infectious vasculitis?
**- Pseudomonas**
**- Aspergillus**
**- Mucor**
89
**Infectious vasculitis** usually spreads how?
· **Infection of local tissue,** however, they can spread through the blood too.
90
**Vascular infections (infective vasculitis)** can weaken arterial walls and can result in what clinically significant events?
· **Mycotic aneurysms** or cause **thrombosis** and **infarction**
91
What is Raynaud’s phenomenon?
**Excessive vasospasm (RED, WHITE and BLUE)** of small arteries and arterioles, in the fingers and toes: proximal **vasodilation**, central **vasoconstriction** and **distal cyanosis**
92
How does Primary Raynaud's vs. Secondary differ in their symmetry of involvement of the digits?
**Primary** has **symmetrical** involvement of the digits and is **not associated with an underlying disease. It is caused cold or emotion.**
· Most common in young F, benign
- **Secondary** has **asymmetrical** involvement of the digits and is assx with a underlying condition: **SLE, scleroderma. Gets worse w time.**
93
**Excessive vasoconstriction** of myocardial arteries or arterioles which may cause **ischemia** or **infarct** is known as what?
**· Cardiac Raynaud**
94
What are **endogenous** and **exogenous agnts** that can **prolonged myocardial vessel contraction?**
* Endogenous: **EPI or a pheochromacytoma**
* Exogenous: **cocaine**
95
**Cardiac Raynaud** can result in what?
1. Sudden cardiac death
2. takotsubo cardiomyopathy “broken \<3” syndrome d/t emotional distress
96
**Varicose veins** are abnormally dilated, tortous veines caused by prolonged, **increased intraluminal pressure** and most often affect which veins?
**_Superficial veins_ or the upper and lower legs.**
97
What are complications of varicose veins?
* **Stasis**
* **congestion**,
* **stasis dermatitis** (pain and ischemia on overlying skin),
* **poor wound healing,**
* **ulcers**
98
How frequent do emboli arise from superficial LE veins?
**_RARE_**, compared to deep LE veins (DVT).
99
What can cause **esophageal varices** and why are they clinically important?
* Portal HTN: **opens shunt** and **sends blood to veins at GE junctio**n: they can **rupture** and kill.
100
What are hemorrhoids
**Dilation of venous plexus at anorectal junction**
101
What is **thrombophlebitis** and where does it most commonly occur?
**inflammatory proces**s that causes **blood clot to form** and block a **VEIN**, usually in your leg (forms DVT)
102
What is the most serious potential complication associated with DVT's and is often the first manifestation of thrombophlebitis
What are the most important risk factors for developing a DVT?
1. PE
2. prlong activity/not moving; hypercoagulbility
103
What is **migratory thrombophlebitis (Trousseau syndrome)?**
MCC in?
* **A clot that moves around the body:** appears at one site, disappears, then goes to another.
* Mucin secreting **ADENOCARCINOMAS, often a paraneoplastic syndrome**
104
What are the major causes of Superior Vena Cava Syndrome?
neoplasms
aortic aneurysms
105
What is **Superior vena cava syndrome**
pressure on the SVC is due to a neoplasm compressing it =\> **leads to cyanosis and dilation of the veins of the head, neck and arms a**nd can cause **respiratory distress** if **pulmonary vessels** are compressed
106
What is inferior vena cava syndrome
neoplasma compress IVC or thrombosis of hepatic, renal or LE veins that go up =\>
1. **LE edema,**
2. **distension of _superficial collateral vein_ in the lower abdomen.**
3. **If the kidneys are involved =\> _massive proteinuria._**
107
Which 2 cancers have a tendency to grow within veins and may ultimately lead to **inferior vena cava syndrome?**
May also be caused by thrombosis from where?
* **- Hepatocellular carcinoma**
* **- Renal cell carcinoma**
* **- Thrombosis of the hepatic, renal, or LE veins which propogate cephalad**
108
Most common agent of lymphangitis is
**Group A B-hemolytic Strep**
109
;/hat is lymphangitis and what most commonly causes it?
* acute inflammation and the spread of bacterial infection into the lymphatics =\> red painful subcutaneous streats and painful enlargement of draining LN
110
What is lympha**den**itis
**painful enlargment of draining LN**
111
\_\_\_\_\_\_\_ lymphedema is **congenital**, either an isolated defect or “**familial Milroy disease,”** which is **lymphatic agenesis**
Primary
112
**\_\_\_\_** lymphedema is a blockage of previously normal lymphatics d/y tumor, etx
secondary
113
**peau d’ orange** is an example of what
***secondary (obstructive lymphedema)***
* **Tumor cells block draining lymphati**c from **breast cancer,** causing the skin over it to look like **ORANG PEELS**
114
**local dilation of a structure**
**Ectasia**
115
A **permanent** dilation of a small vessels that forms a red l**esion in the skin or mucous membrane**. They can be congenital or acquired. What are they called and are they true neoplasms?
* **Telangiectasia; no**
116
**Nevus flammeus**
: a vascular ectasia that forms a light pink-purple birthmark on the head or neck d/t dilated vessels. They often regress by themselves
117
How do Cavernous Hemangiomas differ in terms of infiltration and regression as compared to Capillary Hemangiomas?
- Are **more infiltrative** and frequently involve **deep structures**
- **Do NOT spontaneously regress**
- Locally **destructive**!
118
\_\_\_\_\_\_\_ are very common tumors characterized by increased numbers of normal or abnormal BV
hemangiomas
119
Congenital (juvenile/strawberry) hemangiomas often what...
***REGRESS***
120
What is a capillary hemangioma
**most common; thin wall capillaries are tightly packed 2gether**
121
what is this
Cavernous hemangiomas: **irregular, dilated BV** form a lesion with a irregular border, often involving **deep tissu**e and are more likely to bleed
122
\_\_\_\_\_\_\_\_\_\_\_\_ (lobular capillary hemangioma) are actually r**apidly-growin**g hemangiomas that exist the **oral mucosa and can ulcerate.**
When do they commonly occur?
**Pyogenic granuloma**
**Granuloma gravidarium (pregnany tumor:**
123
What is a pyogenic granuloma
**_A CAPILLAR HEMANGIOMA!_**
not a pyogenic granuloma
124
