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Flashcards in Vascular Pathology 2 Deck (39)
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1

Vasculitis

Inflammation of vessels

Clinical presentation: depend on location affected and often have nonspecific S/S:
Fever, malaise, arthralgias, myalgias

2

Infectious vs noninfectious vasculitis

Noninfectious vasculitis: immunologic injury - immune complex deposition or autoantibodies; indicates immunosuppressive therapy

Infectious vasculitis - pathogenic organisms invading vessel wall

3

Immune complex vasculitis

Deposition of antigen-antibody complexes in vascular walls
◦ Incites an inflammatory reaction within the wall
◦ Antigen is often unidentified

May be seen in
◦ Systemic immunologic diseases (eg, SLE)
◦ Drug hypersensitivity
◦ Secondary to exposure to infectious agent

4

Antineutrophil cytoplasmic antibodies

Heterogeneous group of antibodies reactant with cytoplasmic enzymes in neutrophil granules, monocytes, and endothelial cells
◦ Anti-proteinase-3 (previously c-ANCA)
◦ Anti-myeloperoxidase (previously p-ANCA)

ANCA titers generally follow disease severity

ANCAs activate neutrophils, which then release reactive oxygen species

5

Giant cell (temporal) arteritis

Most common vasculitis in older patients, may present with constitutional symptoms

Chronic (T cell mediated) inflammation of arteries in the head, especially the temporal arteries
◦ Medial granulomatous inflammation, often with multinucleated giant cells
◦ Fragmentation of the elastic lamina
◦ Sites of involvement within an artery may be patchy and focal
◦ Healed sites of inflammation show scarring of the media and intimal thickening

Involvement of the ophthalmic artery may lead to vision loss

6

Takayasu arteritis

Similar histologic findings as those seen in giant cell arteritis, except ◦ Involves the aortic arch and major branch vessels (“pulseless disease”)
◦ Pulmonary, coronary and renal arteries may be involved
◦ Younger age group (<50)

May present with weak pulse and low blood pressure in the upper extremities

7

Polyarteritis nodosa (PAN) incidence

Systemic vasculitis, likely immune complex mediated

Involves (decreasing order of frequency)
◦ Renal vessels, heart, liver, GI tract
◦ Pulmonary vessels are spared

May affect any age group, but classically young adult

Almost 1/3 of patients have chronic hepatitis B
◦ HBsAg-HBsAb complexes are found in the involved vessels

8

Polyarteritis nodosa (PAN) characteristics

Transmural necrotizing inflammation, containing neutrophils, eosinophils, lymphocytes and macrophages

Fibrinoid necrosis of the vessel wall

Sites of inflammation are typically not circumferential

The inflamed vessel wall my become susceptible to ◦ Thrombus formation/occlusion
◦ Aneurysm
◦ Rupture

Immunosuppressive therapy usually effective

9

Kawasaki disease

Acute arteritis of infants and small children (80% ≤ 4 years)

Often involves the coronary arteries
◦ Affected sites may form aneurysms → thrombosis or rupture → acute MI

Presenting picture is usually erythema of the conjunctiva, oral mucosa, palms and soles; rash, and cervical lymph node enlargement
◦ “Mucocutaneous lymph node syndrome”

Usually self-limited, but IV Ig and aspirin are indicated to lower the risk of a coronary event

10

Microscopic polyangiitis

Necrotizing vasculitis involving arterioles, capillaries and venules

Affects vessels of many organ systems
◦ Renal glomeruli and lung capillaries most common

Most cases associated with MPO-ANCA

Segmental necrotizing inflammation with fibrinoid necrosis
◦ Many apoptotic neutrophils are usually seen
◦ “leukocytoclastic vasculitis”

11

Churg-Strauss Syndrome

Small vessel necrotizing vasculitis, associated with
◦ Asthma, allergic rhinitis, hypereosinophilia, lung infiltrates, extravascular granulomas

Inflammation may resemble PAN or m. polyangiitis, with the addition of eosinophils and granulomas

Less than half show ANCA positivity

Many organ systems may be involved.

12

Behcet disease

Vasculitis of small to medium vessels with the following additional findings:
◦ Aphthous ulcers of the oral cavity
◦ Genital ulcers
◦ uveitis

The vessel inflammation is neutrophilic and morphologically nonspecific, may involve visceral organ systems with subsequent aneurysm formation

Associated with HLA-B51

13

Granulomatosis with polyangiitis features

Necrotizing vasculitis featuring:
◦ Necrotizing granulomas of the upper and/or lower respiratory tracts
◦ Necrotizing or granulomatous vasculitis, most prominently in the respiratory tract
◦ Focal necrotizing, often crescentic, glomerulonephritis

Associated with PR3-ANCA (up to 95% of cases)

◦ Upper respiratory tract: sinonasal and pharyngeal inflammation with granulomas and vasculitis
◦ Lower respiratory tract: multiple necrotizing granulomas, which may coalesce and cavitate

Immunosuppressive therapy is usually successful (untreated, 80% mortality in one year)

14

Clinical features of Granulomatosis with polyangiitis

◦ M >F
◦ Avg age of 40
◦ Most patients have persistent pneumonitis and sinusitis, renal disease, and nasopharyngeal ulceration

15

Thromboangiitis obliterans

Acute and chronic thrombosing vasculitis of small and medium vessels, especially
◦ Tibial arteries
◦ Radial arteries

May lead to vascular insufficiency of the extremities

Patients are almost always smokers, and young adults

Inflammation may extend to involve adjacent veins and nerves (leading to pain)

Chronic ulcerations, which may lead to gangrene

16

Raynaud phenomenon

excessive vasospasm of small arteries and arterioles, especially in the fingers and toes

17

Primary Raynaud phenomenon

◦ Induced by cold or emotion; symmetric involvement of the digits ◦ Estimated 3-5% general population; young women
◦ Benign course

18

Secondary Raynaud phenomenon

◦ A component of another arterial disease such as SLE, scleroderma, or thromboangiitis obliterans; asymmetric involvement of digits
◦ Worsens with time

19

Myocardial vessel vasospasm

Excessive vasoconstriction of myocardial arteries or arterioles (“cardiac Raynaud”) may cause ischemia or infarct

Usually caused by circulating vasoactive agents, which may be endogenous
◦ epinephrine

Or exogenous
◦ cocaine

20

Varicose veins

Abnormal dilation of veins with valvular incompetence, secondary to sustained intraluminal pressure
◦ Stasis, congestion, thrombus, edema and ischemia of overlying skin (stasis dermatitis)

Embolism from thrombi of superficial lower extremity veins is rare.

21

Esophageal varices

Portal hypertension (often due to cirrhosis) opens portosystemic shunts which direct blood to veins at the gastroesophageal junction
◦ Esophageal varices (clinically important because they may fatally rupture)

the rectum
◦ hemorrhoids

and periumbilicus
◦ Caput medusa

22

Hemorrhoids

Dilation of the venous plexus at the anorectal junction

Extremely common, and cause pain, bleeding, and may ulcerate

23

Thrombophlebitis

Venous thrombosis and inflammation

Almost always (>90%) involves deep veins in the legs, and can be
completely asymptomatic

Single most important risk factor for developing a deep venous thrombosis (DVT) in the LE is prolonged inactivity/immobilization

Systemic hypercoagulability may also increase risk of DVT

Pulmonary embolism is the most serious potential consequence

24

Migratory thrombophlebitis (Trousseau sign)

◦ Patients with cancer may experience hypercoagulability as a paraneoplastic syndrome

◦ Particularly seen with mucin-producing adenocarcinomas (mucin is thought to be thrombogenic)

◦ In the classic case, venous thromboses appear at one site, disappear, and reappear at a different site

◦ Associated with adenocarcinomas of the lung, ovary, pancreas

25

Hemangiomas - general

common tumors showing a localized increase in neoplastic blood vessels

Common sites include skin and mucous membranes of the head and neck, and in the liver

Congenital (juvenile, or “strawberry”) hemangiomas often regress

26

Capillary hemangioma

most common

thin-walled capillaries, tightly packed together

27

Cavernous hemangioma

◦ Irregular, dilated vascular channels making a lesion with an indistinct border

◦ More likely to involve deep tissue, more likely to bleed

28

Pyogenic granuloma (lobular capillary hemangioma)

◦ type of capillary hemangioma (not pyogenic, not a granuloma)

◦ Rapidly growing, often in oral mucosa (where they may ulcerate)

29

Simple lymphangioma

◦ Appear very similar to capillary hemangiomas, but without RBCs

◦ Subcutis of head/neck and axillae

30

Cavernous lymphangioma (cystic hygroma)

◦ Neck or axilla of children

◦ Can be large (up to 15 cm)

◦ Large cavernous lymphangiomas of the neck are often seen in Turner syndrome