Exam 3: Vascular Pathology Pt.3 Flashcards
What is a form of pediatric vasculitis that we think has an autoimmune origin and is believed to be due to a viral trigger and manifests with an acute fever and large aneurysms in the coronary arteries?
Kawasaki Disease
In what disease does a young child have a “strawberry tongue”, swollen extremities, cervical lymphadenopathy and in 20% of cases it places the child at risk for a lethal myocardial infarction?
Kawasaki Disease
How does Kawasaki Disease manifest?
- widespread inflammation
- oral erythema (“strawberry tongue”)
- swollen extremities
- desquamtation or blistering of erythematous skin
- cervical lymphadenopathy
Does Kawasaki Disease have a genetic influence? If so, who or how?
yes, Asian descent are more likely to develop it
- siblings of affected ind are 10-20x more likely to develop it
What age group, sex, and how common is Kawasaki Disease?
- exclusively young children, most under 4 yrs
- males
- 1 in 25,000 under age 5
T/F. Children that have Kawasaki disease can be given Ibuprofen or acetaminophen to help with the symptoms.
False– it has no response to those drugs
What is am extremely rare condition that occurs in children, typically 4-12 yrs, that are given aspirin while recovering from a viral infection, and is a medical emergency?
Reye Syndrome
What are the most common viral infections associated with Reye Syndrome?
- influenzae
- varicella-zoster virus (chickenpox)
What is effected in children that develop Reye Syndrome?
Liver --> steatosis and hepatic dysfunction Brain --> encephalopathy - seizures - confusion - lethargy - loss of consciousness - coma/death
- inflammatory reaction
- vomiting
- diarrhea
- rashes
What is a T cell-mediated, type IV hypersensitivity, that causes destructive “necrotizing granulomas” w/in kidneys, lungs, trachea, and sinuses, and causes hemoptysis and hematuria?
Wegener Granulomatosis
hemoptysis = coughing up blood
hematuria = blood w/in urine
What four main things does Wegener Granulomatosis cause?
- chronic granulomatous inflammation
- widespread necrotizing vasculitis
- kidney damage–> glomerulonephritis
- pneumonitis
What individuals is Wegener Granulomatosis most likely to develop in and what may it cause within 1 year?
middle-aged males and cause lethal kidney damage w/in 1 year of most patients (80%)
What is the most common manifestation of Wegener granulomatosis and what is it associated with?
pneumonitis: ass. with:
- cough
- chest pain
- dyspnea
- hemoptysis
What other disease does Wegener Granulomatosis have a similar manifestation as? What makes them different?
Polyarteritis nodosa
– but Wegener Granulomatosis involves lungs and upper respiratory tract or sinuses
What vasculitis disease is directly related to tobacco smoking and is believed to develop in genetically predisposed inds?
Buerger Disease
How does Buerger Disease manifest in its earlier stages? What about the advanced stages?
- cold and cyanotic extremitis
- vascular claudication
- Raynaud phenomenon
- soft tissue ulcerations
- gangrenous necrosis
- leads to amputation
Describe that onset of Buerger Disease. What arteries are effects?
acute onset causing severe transmural vasculitis in small-to-medium arteries of extremities:
- tibial arteries and radial arteries
What may help Buerger Disease to improve? How long does it take for severe ischemia and permanent vascular occlusion to occur if one continues to smoke?
STOP smoking
if continue–> that may occur in 5-8 years
What age group is characteristically affected by Buerger disease? What sex? Any ethnicity have greater risk?
males 3x more likely
b/w ages 20-40 and heavy cigarette smokers
Asians (japan, korea, natives of india) and Ashkenazi jews
What condition develops following inappropriate and exaggerated vasoconstriction causing pallor or cyanosis of fingers?
Raynaud Phenomenon
may be Primary or Secondary
What may occur in the most severe or chronic cases of Raynaud phenomenon?
atrophy of skin, subcutaneous tissues, and underlying musculature
How does one get Primary Raynaud Phenomenon? What triggers it and who is most common affected?
idiopathic and triggered by cold exposure and emotional stress
young adult females
What conditions may cause Secondary Raynaud Phenomenon?
- Buerger Disease
- lupus (SLE)
- CREST syndrome
- severe atherosclerosis
- after smoking
- following trauma
What is an acute coronary syndrome that develops following excessive and prolonged coronary artery vasospam and develops when the individual experiences extreme emotional distress?
Takotsubo Cardiomyopathy (cardiac Raynaud’s or Stress-induced cardiomyopathy or Broken heart syndrome”
Takotsubo Cardiomyopathy has been reported to develop following what situations?
- learning of death of a loved one
- motor vehicle accidents
- learning of significant diagnosis/illness
- being surprised
What group of individuals is Takotsubo cardiomyopathy more common in? (age, sex, race)
- older adult postmenopausal females
- Asian or Caucasian inds
What causes the heart injury associated with Takotsubo cardiomyopathy?
emotional event–> causes release of catecholamines into blood –> causes severe cardiac ischemia and prolonged coronary artery vasospam
catecholamines increase HR and strength of contraction and combined with the ischemia = injury
What do individuals who experience Takotsubo cardiomyopathy suffer from?
prolonged arrhythmia–> typically ventricular fibrillation, and experience sudden cardiac death
may have M.I. if ischemia persists for duration of 20-30 mins
What is the term for “inflammation of a vein”, but is also used to describe inflammatory conditions that cause blood to clot w/in a vein? What is the MC form?
Thrombophlebitis
Deep Vein Thrombosis (90%)
What term describes when blood clots within a vein, WITHOUT presence of inflammation?
Phlebothrombosis
What are dilated and tortuous superficial veins that MC are located in the leg and thigh?
Varicose veins
smaller ones –> telangiectasia or “spider veins”
What causes varicose veins?
following chronic intravascular pressure in veins of lower extremities causing:
- valvular incompetence
- stasis and congestion of venous blood
- pitting edema
- pain
T/F. Emboli frequently develop at the site of varicose veins.
False (even though they do frequently develop in deep veins of lower extremity)
What is the average age of Dx for varicose veins? What fraction of females and males get them?
age 55
1/3 of all women
1/5 all males
What are risk factors for developing varicose veins?
- obesity
- having been pregnant
- wearing high-heeled footwear
- having a family Hx
What may occur with severe cases of venous congestion due to more sever varicose veins?
- poor wound healing
- ulcerations (“varicose ulcers”)
- infections
- stasis dermatitis
What is a chronic inflammatory skin condition that develops on the lower extremities following periods of prolonged lower extremity venous insufficiency?
Stasis dermatitis (venous eczema)
What is the chronic inflammatory skin conditions, Stasis dermatitis, believed to develop from?
- harmful effects of increased venous pressure
- reduced tissue perfusion/hypoxia
- increase in matrix metalloproteinase (MMP)
What individuals is Stasis Dermatitis most likely to develop in?
if have varicose veins and affects older adults
What is the appearance of the skin of someone with Stasis Dermatitis?
- more pigmented or erythematous appearance
- thickening of skin or flaking
What are complications of Stasis Dermatitis?
- pain
- cosmetic concerns
- nonhealing cutaneous ulcers–> may cause bacterial skin infs (i.e. cellulitis)
What is Superior Vena Cava Syndrome?
develops when blood flow through SVC is obstructed
What are causes for Superior Vena Cava Syndrome? Which two are the MC reasons?
- lung cancer (MC)
- implanted hardware (pacemaker)
- mediastinal lymphoma (MC)
- thrmobosis w/in SVC
What are the clinical features associated with Superior Vena Cava Syndrome?
- venous distention of head, neck, and upper extremities
- Congested Upper Extremity Veins = UE cyanosis/edema
- HAs
- dizziness
- facial swelling
Which is MC, Superior or Inferior Vena Cava Syndrome?
Inferior Vena Cava syndrome is MC
What develops when blood flow through the inferior vena cava is obstructed?
Inferior Vena Cava Syndrome
What are causes of Inferior Vena Cava Syndrome?
- Hepatocellular carcinoma (liver CA)
- Thrombus of lower extremity veins (DVT)
- renal cell carcinoma (kidney CA)
- pregnancy
What are the clinical features of Inferior Vena Cava Syndrome?
- pitting edema in lower extremities
- lower extremity cyanosis
- kidney dysfunction –> proteinuria
What is Lymphedema?
an abnormal collection of fluid w/in interstitium and lymphatic channels, resulting from an obstruction of lymphatic drainage
congenital or acquired
can be Primary or Secondary
What occurs distal to the obstruction in Lymphedema?
- lymphatic channels have elevated hydrostatic pressure
- interstital fluid accumulates
- tissues become engorged
- peau d’orange or brawny induration
What is a thickening and hardening of the skin due to fibrosis at the site of skin that has been chronically inflamed? (is ass. with Lymphedma)
Brawny induration
What is a rare inherited congenital condition that develops following the agenesis or hypoplasia of lymphatics?
Primary Lymphedema (aka Milroy disease)
What causes Milroy Disease?
(primary lymphedema)
- autosomal dominant condition due to mutation in FLT4 gene
- interfere with formation of endothelial lining of lymphatic system and absent or underdeveloped lymphatics
Which is MC, primary or secondary lymphedema?
secondary lymphedema
How does Secondary Lymphedema develop? What are common stimuli for it?
following inflammation/fibrotic, infectious, or neoplastic diseases
- tumors
- surgery-related scarring
- radiation-related scarring
- inflammation-related blood clots or scarring
- filariasis-related scarring
What is an acute inflammation of the lymphatic channel due to infectious process?
Lymphangitis (“bacterial lymphangitis”)
What do most cases of Lymphangitis develop from?
bacterial infection due to minor skin trauma
Streptococcal and Staphylococcal organisms are common causes
What are characteristics of Lymphangitis?
- acute inflammation
- painful subcutaneous red streaks in area of lymph channel
- fever, chills, malaise, HA, anorexia, muscle aches
- possible bacteremia and SEPSIS
What do vascular tumors develop from?
vascular endothelial cells (MC) and surrounding vascular CT
most are benign
What are benign tumors of capillary endothelial cells and are well-differentiated with little-to-no risk of malignant transformation?
Hemangioma
Where are hemangiomas mostly discovered? But where do 30% of all hemangiomas?
on the skin of face, neck, head of young children
but 30% are located with the liver
What are the common “strawberry hemangiomas” discovered early in life and are present in 1 in 200 newborns called? How do they manifest?
Juvenile hemangiomas
grow rapidly for few months–> by age 1-3 start to fade –> then ~90% self-resolve w/in 1st decade of life
Whar are hemangiomas that develop on the gingiva, oral mucosa, or skin of pregnant women? What are they thought to be due to?
Pyogenic granulomas
hormonal fluctuations of pregnancy–> therefore sometimes called “pregnancy tumors”
What are hemangiomas that develop w/in bone and are MC discovered w/in bones of spine or skull?
Interosseous hemangiomas
Where are Interosseous hemangiomas most likely to develop?
in spine or skull
-when in spine–> commonly involve single VB–> called “vertebral hemangioma”
What percent of the population do Vertebral hemangiomas affect? What sex is more commonly affect?
10% of adult population
males 2x
no clinical significance
What is a lymphatic endothelial tumor that develops following a Kaposi sarcoma herpesvirus infection?
Kaposi Saracoma
What are an AIDS-defining illness and is much more likely to develop among individuals in the crisis phase of AIDS?
Kaposi Sarcoma
What is characteristically required for one to develop Kaposi Sarcoma?
The Kaposi Sarcoma Herpesvirus typically becomes latent and the combination of this virus and immunosuppression s required
(there AIDS inds)
How do the areas of Kaposi Sarcoma appear? Where are they MC?
- raised pink, red, or purple patch or patches that form from the vascular tumor (Maculas–> “purpura”)
- often raised and asymptomatic lesions
- endothelial cells are irregular and high amounts of inflammatory cells
MC on Lower Extremities
What is the MC HIV-related malignancy?
Kaposi Sarcoma
very aggressive
What are malignant endothelial tumors of vasculature that contain fairly well-differentiated endothelial cells or highly anaplastic endothelial cells?
Angiosarcoma
high anaplastic–> is aggressive and it is assumed to be this until proven otherwise
How are Angiosaracomas able to spread? Where do they most likely spread to?
spread via hematogenous or lymphatic systems–> to lungs or liver
What is the prognosis for anaplastic angiosarcomas?
poor, about 30% of all inds survive another 5 years after Dx
Where are angiosarcomas most commonly discovered?
- skin
- w/in breast
- w/in various soft tissues
- w/in liver
(but could be anywhere with vascularity)
How do angiosarcomas that develop on the skin manifest?
- small well-documented red nodules
- asymptomatic, but may have pain or itching
- as larger, they manifest with tissue necrosis or hemorrhage
What are risk factors for developing angiosarcomas?
- soft tissue trauma (surgery or foreign body)
- exposure to env. poisons (arsenic or PVC)
What are Hepatic angiosarcomas characteristically associated with?
shaving Hx of working in PVC manufacturing industry–> long latency period and will develop several years after exposure
What is the placement of an intravascular stent known as?
endovascular stenting
What is a stent?
a tubular support structure that is inserted into blood vessel to maintain the patency of the vascular lumen at site of arterial stenosis
In what patients is Endovascular stenting a very common procedure for? What else can it be used for?
- common in patients with–> coronary artery disease
ALSO for: - hold down intimal flaps in someone w/ arterial dissection
- limit vasospasm in someone with Prinzmental angina
What is the term for the condition of being open, expanded, or unobstructed?
patency
How is endovascular stenting typically performed?
following expansion of a balloon angioplasty to open the site of vascular stenosis
What is the term for replacing or bypassing a diseased vessel?
Vascular Grafting
like to bypass an artery involved in CAD
What are two things that vascular grafts can be composed of?
- Synthetic material
2. Autologous grafts (autologous = cells or tissues obtained from the same ind)
What vessels are commonly used for Autologous grafts?
great saphenous vein or left internal mammary artery
What is the name of the procedure when vascular grafts are used to manage CAD?
coronary artery bypass graft (CABG, pronounced like “cabbage”)
What is the limited long-term utility of using the great saphenous vein for a vascular graft? what about for the internal mammary artery?
great saphenous vein–> 50% chance of lasting 10 years
internal mammary artery–> 90% chance of lasting 10 years
What are compression stockings used for?
- encourage venous return
- external pressure to minimize lymphedema
What else should one do that is using compression stockings to help the swelling of legs?
- leg elevation
- recommendations to stay active
- weight loss
- manual lymphatic drainage massage
- regular skin evaluations
