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Vascular Part II Flashcards

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1
Q

What is vasculitis and the clinical presentation?

A

Inflammation of vessels
Clinical presentation may depend on the location of the affected vessel, but pt frequently show nonspecific signs and symptoms
Fever, malaise,arthralgias, myalgias

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2
Q

How is vasculitis classified?

A

Infectious vs noninfectious

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3
Q

What is noninfectious vasculitis mediated by?

A

immunologic injury
Immune complex deposition or autoAbs

Immunosuppressive therapy needed

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4
Q

What is immune complex vasculitis and what may it be seen in?

A

Deposition of antigen-ab complexes in vascular walls

may be seen in SLE, drug hypersensitivity, secondary to exposure to infectious agent

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5
Q

What are antineutrophil cytoplasmic antibodies?

A

ANCA
heterogenous group of abs reactant with cytoplasmic enzymes found in neutrophil granules, monocytes, and endothelial cells
Activate neutrophils which release ROS

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6
Q

Anti-proteinase 3 or C-ANCA is directed against what?

A

Neutrophil azurophilic granule constituent

Associated with GPA or Wegener granulomatosis

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7
Q

What is anti-myeloperoxidase or p-ANCA directed against

A

Lysosomal constituent involved in generating oxygen free radicals
Seen in microscopic polyangiitis or Chur-Strauss syndrome

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8
Q

How are ANCAs useful?

A

Diagnostic markers for ANCA-associated vasculitides

Titers often mirror inflammation lvls so generally follow disease severity

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9
Q

What is giant cell arteritis?

A

also called temporal arteritis
Most common vasculitis in older pts
Focal granulomatous inflammation of medium and small aa
Chiefly cranial vessels, can involve aorta

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10
Q

What is the morphology of Giant cell arteritis?

A

chronic (T-cell) inflammation of aa in head, especially temporal arteries
Medial granulomatous inflammation
Multinucleated giant cells
Fragmentation of elastic lamia
Healed sites - scarring of media and intimal thickening

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11
Q

What arteries are involved in Giant cell arteritis?

A

Temporal
Cranial vessels
Opthalmic a (50%) –> vision loss
Aorta

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12
Q

What is the clinical presentation of temporal arteritis?

A

Presents with headache and facial pain

Systemic symptoms - flulike

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13
Q

How is giant cell arteritis diagnosed?

A

Surgical biopsy of temporal a of 1 cm

Sites of involvement may be patchy and focal

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14
Q

Treatment for giant cell arteritis ?

A

Steroids or antitumor necrosis factor therapy

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15
Q

What is Takayasu arteritis?

A

narrowing of lumen; vasculitis of aortic arch and major branch vessels
Pulseless disease
Pulmonary, coronary and renal aa may be involved
upper extremities pulseless

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16
Q

Giant cell arteritis typically seen in what pts?

A

50 and older

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17
Q

Describe a typical pt with Takayasu arteritis?

A

Weak pulse and low BP in upper extremities
Less than 50 y/o
ocular and neurologic disturbances
HTN occuring secondary to renal a disease

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18
Q

What are the histological features of Takayasu arteritis?

A

same as Giant cell arteritis but in a pt less than 50

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19
Q

What is Polyarteritis nodosa? PAN?

A

Systemic vasculitis
Immune complex mediated
Necrotizing vasculitis involving small-medium aa
No ANCAs

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20
Q

What vessels are involved in Polyarteritis nodosa (PAN)?

A 
renal vessels
Heart
Liver
GI tract
NOT pulmonary vessels
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21
Q

Describe a typically pt with PAN?

A

any age group, but classically young
1/3 have chronic hep B
(HBsAg-HBsAb)

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22
Q

What is usually effective in treating Polyarteritis nodosa?

A

Immunosuppressive therapy

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23
Q

Describe the lesions seen in Polyarteritis nodosa?

A

Transmural necrotizing inflammation - contains neutrophils, eosinophils, lymphocytes and Mo
Fibroid necrosis of vessel wall
Inflammation not circumferential
Thickened intima

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24
Q

In PAN, the inflamed vessel wall may become susceptible to what?

A

thrombus formation/occlusion -> ischemic
Aneurysm
Rupture

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25
What is Kawasaki disease?
Acute arteritis of infants and small children (80% less than 4 yo)
26
What vessels does Kawasaki disease typically involve
Coronary arteries
27
What can Kawasaki disease lead to?
Affected sites may form aneurysms -> thrombosis or rupture -> acute MI
28
What is the presenting picture of Kawasaki disease?
Erythema of conjunctiva, oral mucosa, palms and soles Rash Cervical LN enlargement (mucocutaneous LN syndrome)
29
What is the treatment for kawasaki disease?
Usually self-limited. | IVigs and aspirin are indicated to lower risk of cornonary event
30
What is Microscopic polyangiitis?
Necrotizing vasculitis involving arterioles, capillaries and venules
31
What vessels are affected in microscopic polyangiitis?
Vessels of many organ systems | Renal glomeruli and lung capillaries most common
32
What are commonly associated with Microscopic polyangiitis?
``` Necrotizing glomerulonephritis (90%) and pulmonary capillaritis MPO-ANCA ```
33
Describe the histologic findings of Microscopic polyangiitis
Segmental necrotizing inflammation with fibrinoid necrosis | Many apoptotic neurtrophils w/i and around vessel walls (leukocytoclastic vasculitis)
34
What can improve long-term survival w Microscopic polyangiitis?
Cyclosporine and steroids
35
What is Churg-Strauss syndrome?
Small vessel necrotizing vasculitis | Associated with Athma, allergic rhinitis, hyereosinophilia, lung infiltrates, extravascular granulomas
36
What is Churg-Strauss syndrome associated with that sets it apart from PAN or m. polyangiitis?
Eosinophils, granulomas, asthma, allergic rhinitis Less than half show ANCA positivity
37
What is the cause of mortality in pts with Churg-Strauss syndrome? What possibly causes this?
Many organ systems may be involved but eosinophilic infiltrates are implicated in cardiomyopathy that develops in 60% of pts Cardiac involvement -> death in approx half of pts
38
What is Behcet disease
Vasculitis of small-medium vessels w the additional findings: Aphthous ulcers of oral cavity Genital ulcers Uveitis
39
What is the typical triad that presents with Bechet disease?
Apthous ulcers of the oral cavity Genital ulcers Uveitis
40
Describe vessel inflammation in Bechet disease
neutrophilic and morhpologically nonspecific | May involve visceral organ systems with subsequent aneurysm formation
41
What is the marker for Microscopic polyangiitis
MPO-ANCA
42
What is the marker for Bechet disease?
HLA-B51
43
What is mortality related to in a pt with Bechet disease?
Sever neurologic involvement or rupture of vascular aneurysms
44
What is effective i the treatment of Bechet disease?
Steroids or TNF-antagonist therapies
45
What is Granulomatosis with polyangiitis?
GPA or Wegener granulomatosis | Necrotizing vasculitis
46
What triad comes with Granulomatosis with polyangiitis?
Necrotizing granulomas of the upper and lower respiratory tracts Necrotizing or granulomatous vasculitis, most prominently in respiratory tract Focal necrotizing, often crescentic, glomerulonephritis
47
What is the marker for Granulomatosis with polyangiitis?
PR3-ANCA - 95%
48
What is the Tx for granulomatosis with polyangiitis?
Immunosuppressive therapy Cyclophosphamide, steroids, and TNF antagnonists *untreated, 80% mortality in one year
49
What are the clinical features of granulomatosis with polyangiitis?
Male, 40 yo | Persistent pneumonitis and sinusitis, renal disease, and nasopharyngeal ulceration
50
What is the morphology of granulomatosis with polyangiitis?
Upper respiratory tract: Sinonasal and pharyngeal inflammation with granulomas and vasculitis LRT: multple necrotizing granulomas, may coalesce and cavitate
51
What is thromboangiitis obliterans?
Acute and chronic thrombosing vasculitis of small and medium vessels Tibial arteries Radial arteries
52
Describe a pt with thromboangiitis obliterans
``` Young adult Smokers Vasculitis in distal extremities Spasms Vascular insufficiency of extremities ```
53
What is the morphology of thromboangiitis obliterans?
Acute lesions: neutrophilic infiltrates with mural thrombi and containing microabscesses, ofthen with giant cell formation Secondary involvement of adjacent vein and nerve
54
What are the symptoms of a pt with thromboangiitis obliterans?
Nodular phelbitis Raynaudlike cold sensitivity Leg claudiction Vascular insufficiency --> pain even at rest, skin ulcers, and ultimately gangrene
55
What is Raynaud?
Excessive vasospasm of small arteries and arterioles | Fingers and toes
56
What are the symptoms of Raynaud?
pain pallor Cyanosis Prlonged vasospasm -> tissue necrosis
57
What is primary Raynaud?
``` induced by cold or emotion Symmetric involvement of digits 3-5% general population Young women Benign course ```
58
What is secondary raynaud phenomenon?
``` Component of another arterial disease such as SLE Scleroderma Thromboangiitis obliterans Asymmetric involvement of digits Worsens with time ```
59
What is myocaardial vessel vasospasm?
Excessive vasoconstriction of myocardial aa or arterioles Cardiac raynaud Histo: contraction band necrosis
60
Myocardial vessel vasospasm may cause what?
ischemia or infarct sudden cardiac death Fatal arrhythmia Ischemic dilated cardiomyopathy
61
What is myocardial vessel vasospasm caused by?
circulating vasoactive agents, which may be endogenous Epinephrine - release by pheochromocytomas Increased sensitivity of vessels to catecholamines - Thyroid hormone Increased caatecholamine - extreme psycological stress
62
What are varicose veins?
Abnormal dilation of veins with valvular incompetence, secondary to sustained intraluminal pressure Stasis, congestion, throbus, edema and ischemia of overlying skin (stasis dermatitis)
63
Varicose veins are common with what type of pts?
10-30% of adults Obesity and pregnancy increase risk Hereditary venous defects Prolonged standing
64
What are esophageal varices caused by?
Portal HTN (often due to cirrhosis) opens portosystemic shunts. Blood directed to veins at gastroesphageal junction, rectum (hemorrhoids), and periumbilicus (caput medusa)
65
Why are esophageal varices clinically important?
they may fatally rupture | Called exsanguination
66
what are hemorrhoids?
dilation of venous plexus at anorectal junction Extremely common Pain, bleeding and may ulcerate Secondary to pregnancy and chronic constipation
67
What is thrombophlebitis?
Venous thrombosis and inflammation
68
What does thrombophelbitis almost always involve?
>90% deep veins in the legs | Can be asymptomatic
69
What leads to increased risk of DVT in LE ?
prolonged inactivity | Systemic hypercoagulability - Factor V leiden
70
What is Trousseau sign?
migratory thrombophlebitis Malignancy-associated hypercoagulability Pts w cancer may experience hypercoagulability as a paraneoplastic syndrome
71
What is Trousseau sign often seen with?
mucin-producing adenocarcinomas Mucin - thrombogenic Adenocarcinomas of lung, ovary, pancreas
72
What is the classic case of Trousseau sign?
Venouts thromboses appear at one site, disappear, and reappear at a different site
73
What are hemangiomas?
Common benign tumor showing localized increase in neoplastic blood vessels Sites: skin, mucous membranes of head/neck and liver
74
Describe capillary hemangioma
Most common Skin, mucous membranes Unencapsulated Thin-walled capillaries, tightly packed together
75
Congenital (juvi/strawberry) hemangiomas
Variant of capillary hemangioma Present at birth Grows rapidly then begins to regress at 1-3 years
76
Describe cavernous hemangioma
Irregular, dilated vascular channels making a lesion with an indistinct borer Unencapsulated Skin, liver, CNS DO NOT REGRESS thrombosis, and dystrophic calcification common More likely to bleed
77
Describe Pyogenic granuloma
``` Lobular capillary hemangioma Not pyogenic or granuloma Ulcerated polypoid variant Proliferating capillaries Rapidly growing, often oral mucosa - ulcerate ```
78
What is a simple lymphangioma?
Benign lymphatic analog of hemangiomas No RBCs Subcutis of head/neck and axillae Exudate-filled, blisterlike blebs composed of small lymphatic channels
79
What is a cavernous lympangioma?
``` Cystic hygroma Neck or axilla of children Can be large Seen in Turner syndrome Simple surgical resection difficult Dilated lymph vessels ```
80
What is a glomus tumor?
``` Benign tumors arising from glomus bodies (thermoregulation), Distal fingers Smooth muscle origin PAINFUL Excision curative ```
81
What is a Bacillary angiomatosis?
Vascular proliferation in response to gran negative Bartonela bacilli Lesions localized, forming red papules Proliferation of capillaries with plump endothelial cells Occurs in immunocompromised
82
How can bartonella bacilli be identified?
PCR or visualized with Warthin-Starry stain
83
What is an effective treatment for Bacillary angiomatosis?
Macrolide antibiotics
84
What is epithelioid hemangioendothelioma
``` Neoplastic endothelial cells Plump and cuboidal - resemble epithelium Vascular channels difficult to recongize Metastasisin 20-30% Variable clinical behavior ```
85
What is Kaposi sarcoma?
Vascular tumor caused by HHV8
86
What is AIDS-associated KS
Most common form seen in US Most common AIDS related malignant tumor May spread to LN and viscera Occur anywhere in skin and mucous membranes, LN, GI tract or viscera
87
What is Chronic/Classic KS?
Older men from middle eastern Mediterranean or eastern european descent NOT associated with HIV Tumor localized to skin Ashkenasi jew Red purple cutaneous plaques and nodules on LE
88
What is African/edemic KS?
Not associated with HIV Pts younger than 40 Can involve LN Cutaneous lesions are sparse, can be aggressive
89
What is Transplant-associated KS?
Not associated with HIV T cell immunosuppression Can spread to LN and viscera can be aggressive and fatal
90
Describe patches
Pink-purple, usally confined to distal LE | Dilated, irregular EC-lined spaces, interspersed lymphocytes, plasma cells, an Mo
91
Describe raised plaques
Dilated, jagged vascular channels lined by plump spindle cellsaccompanied by perivascular aggregates
92
Describe nodular lesions
neoplasic LN and visceral involvement African and AIDS associated diseases Plump spindle-shaped cells, slitlike vascular spaces filled with erythrocytes
93
Describe angiosarcoma
``` Malignant endothelial tumor Older; m=f May occur anywhere Common: skin, soft tissue, breast, liver Locally invasive and may metastasize 5 year survival around 30% Chronic lymphedema can develop, ipsilateral arm years after radical mastectomy for breast cancer Dilated lymphatic vessels ```
94
What would best confirm angiosarcoma?
CD31
95
What is vascular ectasis?
Common lesions characterized by local dilations of preexisting vessels; they are not true neoplasms Nexus flammeus Spider telangiectassis Hereditary hemorrhagic telangiexcasia
96
What is Nevis flammeus?
Birthmark Macular cutaneous lesion that show only dermal vessel dilations; most regress Port wine stain: variant that persist and grow along w child Sturge-weber syndrome: port-wine stain + leptomeningeal angiomatous masses, mental retardation, seizures, hemiplegia, and skull radiopacities
97
What are spider telangiectasis?
Minute subcutaneous arterioles, often pulsatile, arranged in radial fashion around a central core Typically occur above the waist Associated with hyperestrogenic states - pregnancy or cirrhosis
98
What is hereditary hemorrhagic telangiexcasia?
Also older-weber-tendu disease Rare, AD disorder caused by mutations in genes encoding components of the TGF-B signaling pathway Multiple small aneurysmal telangiexcasia on skin and mucous membranes Pts present with epistaxis, hemoptysis, or GI or GU bleeding

CVM II (4 decks)

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