Flashcards in 3.2.3. Vascular Pathology 2 of 3 Deck (88):
What is vasculitis?
Vessel Wall inflammation
Vessels of any type can be affected, but most vasculitides affect what?
Vessels of any type can be affected, but most vasculitides affect small vessels ranging in size from arterioles to capillaries to venules.
Pathogenic Mechanisms of vasculitis:
1. Immune-mediated inflammation
2. Direct invasion of vascular walls by infectious pathogens
3. Infections can also indirectly induce a noninfectious vasculitis (by generating immune complexes or triggering a cross-reactive immune response)
Most common form of vasculitis among older individuals in the U.S. and Europe; usually affects females
Temporal (Giant Cell) Arteritis
What artery does TGCA affect?
Granulomatous vasculitis that classically involves branches of the carotid artery
Depending on the location of the blockage, there are three specific findings associated with TGCA. What are they and what artery is involved with each?
Is there anything else that presents with TGCA?
Presents as headache (temporal artery involvement), visual disturbances (ophthalmic artery involvement) and jaw claudication. Flu-like symptoms with joint and muscle pain (polymyalgia rheumatica) are often present.
Lab results for TGCA?
ESR is elevated (>100)
What does a biopsy reveal with TGCA? How useful is the biopsy?
Biopsy reveals inflamed vessel wall with giant cells and intimal fibrosis
Lesions are segmental; diagnosis requires a biospy of a long segment of vessel, and a negative biopsy DOES NOT exclude disease
What is the treatment and cost of not treating associated with TGCA?
Treatment is corticosteroids; high risk of blindness without treatment
Where does Takayasu Arteritis hit?
Granulomatous vasculitis that classically involves the aortic arch at branch points
Who does Takayasu Arteritis generally affect?
Presents in adults <50 years old (classically, young Asian females)
How does Takayasu Arteritis present?
as visual and neurologic symptoms with a weak or absent pulse in the upper extremity (“pulseless disease”).
Lab results for Takayasu Arteritis?
Takayasu Arteritis treatment
Treatment is corticosteroids.
Medium vessel vasculitis means it affects what type of arteries?
Involves muscular arteries that supply organs.
What is Polyarteritis Nodosa and where does it affect?
Involves muscular arteries that supply organs.
How does Polyarteritis present and hwat arteries are affected with these symptoms?
Is anything else associated generally with PN?
Classically presents in young adults as hypertension (renal artery involvement), abdominal pain, with melena (mesenteric artery involvement)
neurologic disturbances and skin lesions also seen.
What serum is associated with PN?
Associated with serum HBsAg (Hep B surface antigen)
What are the stages for PN?
Lesions of varying stages are present. Early lesion consists of transmural inflammation with fibrinoid necrosis; eventually heals with fibrosis
What interesting finding is associated with PN?
Aneurysms can occur around these first stage segments, producing a “string of pearls” appearance on imaging
Treatment of PN?
Treatment is corticosteroids and cyclophosphamide; fatal if not treated
Who is affected by Kawasaki Disease?
Classically affects Asian children <4 years old
How does Kawasaki Disease present?
Presents with nonspecific signs including fever, conjunctivitis, erythematous rash of palms and soles, and enlarged cervical lymph nodes
What two lethal conditions do we associate with Kawasaki's and what causes them?
Coronary artery involvement leads to risk for:
Thrombosis with myocardial infarction
Aneurysm with rupture
What is the treatment for Kawasakis?
Treatment is aspirin and IVIG; disease is self-limited
Remember aspirin can cause Reye’s syndrome in children, and the nonspecific nature of the symptoms of Kawasaki mimic those of a viral infection, so you have to be careful before treat with aspirin
What is Buerger's Disease also called?
Thromboangiitis Obliterans (Buerger Disease)
What is Buerger's Disease?
Necrotizing vasculitis involving digits
How does Buerger's Disease typically present?
Presents with ulceration, gangrene, and autoamputation of fingers and toes
A cool physical exam finding is associated with Buerger's...what is it?
Raynaud Phenomenon - Color changes
The fingers will go white due to anemia, then blue/cyanotic due to oxygen deprivation after a while and when the fingers get restored blood flow, they turn red
God Bless America.
How do we treat Buerger's?
Highly associated with heavy smoking; treatment is smoking cessation
Granulomatosis with Polyangiitis is also called what?
Wegener Granulomatosis - True story, they changed the name because Wegener, the leading researcher on this condition, was a Nazi according to Ike
Where and what is affected by Granulomatosis with Polyangiitis?
Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys
Who gets affected and what are the presentations of Wegener's?
Classic presentation is a middle-aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis
What lab values do we see with Wegener's?
Elevated serum c-ANCA levels correlate with disease activity
Biopsy reveals large necrotizing granulomas with adjacent necrotizing vasculitis
Treatment is cyclophosphamide and steroids; relapses are common.
What is microscopic Polyangiitis?
Necrotizing vasculitis involving multiple organs, especially lung and kidney
How is the presentation of microscopic polyangiitis different from that of Wegener's?
Presentation is similar to Wegener granulomatosis, but nasopharyngeal involvement and granulomas are ABSENT.
Lab values for Microscopic Polyangiitis?
Serum p-ANCA levels correlate with disease activity.
What is Church-Strauss Syndrome and where does it affect?
Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially the lungs and heart
What two secondary conditions are associated with Church-Strauss?
Asthma and peripheral eosinophilia are often present
Lab values for Church-Strauss?
Serum p-ANCA levels correlate with disease activity.
What causes Henoch-Schonlein Purpura? Who gets it?
Vasculitis due to IgA immune complex deposition; most common vasculitis in children
Presentation of Henoch-Schonlein Purpura
Presents with palpable purpura on buttocks and legs, GI pain and bleeding, and hematuria (IgA nephropathy)
What usually precedes Henoch-Schonlein Purpura
Usually occurs following an upper respiratory tract infection
Treatment of Henoch-Schonlein Purpura
Disease is self-limited (usually resolves on its own); can treat with steroids if severe
How does Behcet's present?
Classically presents as a clinical triad of recurrent oral aphthous ulcers, genital ulcers, and uveitis
What is mortality related to in regards to Behcet's? Where can the vascular ruptures manifest?
There can be gastrointestinal and pulmonary manifestations, with disease mortality related to severe neurologic involvement or rupture of vascular aneurysms.
How do we treat Behcet's?
Immunosuppression with steroids or TNF-antagonist therapies are generally effective.
Most common bacteria/fungi to cause infctious vasculitis?
Aspergillus and Mucor
Etiology of infectious vasculitis?
Can be part of localized tissue inspection, or less commonly, can arise from hematogenous spread of microorganisms during septicemia or embolization from infective endocarditis
What type of aneurysms are related to infectious aneurysms?
Can cause mycotic aneurysms
What is Raynaud's Phenomenon?
exaggerated vasoconstriction of arteries and arterioles in the extremities, particularly fingers and toes (occasionally earlobes, nose, lips); “red, white, & blue” -- proximal vasodilation, central vasoconstriction, and distal cyanosis
What is Primary Raynaud's?
Raynauds caused by exaggerated central and local vasomotor response to cold or emotion
What is Secondary Raynaud's?
Raynaud's caused by vascular insufficiency due to arterial disease caused by other entities
What is different about primary and secondary Raynaud's?
In Secondary, it assymetrically affects the extremities, does not stay constant (it worsens, in fact). The opposite for primary.
What diseases are associated with Secondary Raynaud's?
SLE, scleroderma, Buerger disease, even atherosclerosis
What are the three main chemicals that precipitate prolonged vessel contraction?
cocaine, epinephrine, thyroid hormone
What is Takotsubo cardiomyopathy also known as and what is it?
Takotsubo cardiomyopathy (“broken heart syndrome) -- associated with emotional duress
sudden cardiac death from “cardiac Raynaud” of sufficient duration (20-30 min)--> MI + elevated catechol levels (increase HR and contractility)
What are Varicose Veins?
abnormally dilated, tortuous veins produced by prolonged, increased intraluminal pressure leading to vessel dilation and incompetence of venous valve
What causes Esophagus Varicose Veins and what do they lead to?
caused by liver cirrhosis → portal vein hypertension → opening of portosystemic shunts that increase blood flow into veins at the GI jxn
rupture can lead to massive upper GI hemorrhage
What are hemorrhoids?
Hemorrhoids: varicose dilation of the venous plexus at the anorectal junction (prolonged pelvic vascular congestion due to pregnancy or straining to defecate)
What are Thrombophlebitis & Phlebothrombosis
largely interchangeable terms for venous thrombosis and inflammation; deep leg vein involvement accounts for 90% of cases
What is the relationship between prolonged immobilization and Thrombophlebitis/Phlebothrombosis
prolonged immobilization → venous stasis → DVT in lower extremities
pulmonary embolism most serious complication
What is Trousseau Sign?
Trousseau Sign (migratory phlebitis): paraneoplastic syndrome where venous thrombosis appear and disappear
What are Superior and Inferior Vena Cava Syndromes?
neoplasms that compress or invade the SVC or IVC
What is lymphangitis?
Lymphangitis: acute inflammation elicited by the spread of bacterial infections to lymphatics
Most common cause of lymphangitis and how does it present?
Group A Strep most common agent
manifest by red, painful subq streaks and painful enlargement of draining lymph nodes
What is Lymphadema?
Lymphedema: increases hydrostatic pressure in the lymphatics distal to the obstruction and causes increased interstitial fluid accumulation
What is the difference between primary and secondary lymphedema?
primary: isolated congenital defect
secondary: obstructive; blockage of a previously functional LN
Example of primary lymphedema
familial Milroy disease - lymphatic agenesis or hypoplasia
What is a possible complication of secondary lymphedema?
chylous ascites/chlothorax/ chylopericardium: milky accumulations of lymph from rupture of dilated lymphatics
What types of neoplasms can we have in the vasculature?
Vascular neoplasms can be endothelial derived (e.g. hemangioma, lymphangioma, angiosarcoma) or can arise from cells that support or surround blood vessels (e.g. glomus tumor, hemangiopericytoma)
How do benign tumors usually present in the vessels?
Benign Tumors and Tumor-Like Conditions: usually produce obvious channels filled with blood cells lined by a monolayer of normal-appearing endothelial cells
What are examples of Vascular Ectasias conditions?
1. Nevus flammeus
2. port wine stain
3. spider telangiectasias
4. hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)
What is Nevus flammeus?
Famous character from Harry Potter
What is Nevus flammeus?
Nevus flammeus: birth mark
What is a port wine stain and what is it associated with?
port wine stain: special form of nevus flammeus
do not fade with time
In the distribution of the trigeminal n. associated with Sturge-Weber syndrome
What are spider telangiectasias? What bodily states are they associated with?
non-neoplastic vascular lesions grossly resembling a spider
radial, pulsatile arrays of dilated subq arteries or arterioles about a central core that blanch with pressure
associated with hyperestrogenic states
- pregnancy or liver cirrhosis
Describe hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease). What causes it?
mutations in genes that encode components of TGF-beta signaling pathway
malformations comprised of dilated capillaries and veins present at birth
What are hemangiomas?
Hemangiomas: normal or abnormal vessels filled with blood
Most common type of Hemangioma?
Where do we see capillary hemangiomas?
skin and subq tissue, mucous membranes of oral cavities and lips, liver, spleen and kidneys
Describe Juvenile Hemangiomas and how they progress
arise in the skin and grow rapidly for a few months, then regress by 1-3 y/o
What are Cavernous hemangiomas? How do they compare to juvenile?
large, dilated vascular channels
more infiltrative, involve deep structures and do not spontaneously regress
What type of hemangioma is linked to Hippel-Lindau disease and what is it?
The disease consists of vascular lesions in cerebellum, brain stem, retina, pancreas, and liver
What are pyogenic granulomas? How do they present?
capillary hemangiomas that present as rapidly growing red pedunculated lesions of the skin, gingival or oral mucosa
bleed easily and are often ulcerated