Vascular Part II Flashcards
What is vasculitis and the clinical presentation?
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
How is vasculitis classified?
Infectious vs noninfectious
What is noninfectious vasculitis mediated by?
immunologic injury
Immune complex deposition or autoAbs
Immunosuppressive therapy needed
What is immune complex vasculitis and what may it be seen in?
Deposition of antigen-ab complexes in vascular walls
may be seen in SLE, drug hypersensitivity, secondary to exposure to infectious agent
What are antineutrophil cytoplasmic antibodies?
ANCA
heterogenous group of abs reactant with cytoplasmic enzymes found in neutrophil granules, monocytes, and endothelial cells
Activate neutrophils which release ROS
Anti-proteinase 3 or C-ANCA is directed against what?
Neutrophil azurophilic granule constituent
Associated with GPA or Wegener granulomatosis
What is anti-myeloperoxidase or p-ANCA directed against
Lysosomal constituent involved in generating oxygen free radicals
Seen in microscopic polyangiitis or Chur-Strauss syndrome
How are ANCAs useful?
Diagnostic markers for ANCA-associated vasculitides
Titers often mirror inflammation lvls so generally follow disease severity
What is giant cell arteritis?
also called temporal arteritis
Most common vasculitis in older pts
Focal granulomatous inflammation of medium and small aa
Chiefly cranial vessels, can involve aorta
What is the morphology of Giant cell arteritis?
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
What arteries are involved in Giant cell arteritis?
Temporal
Cranial vessels
Opthalmic a (50%) –> vision loss
Aorta
What is the clinical presentation of temporal arteritis?
Presents with headache and facial pain
Systemic symptoms - flulike
How is giant cell arteritis diagnosed?
Surgical biopsy of temporal a of 1 cm
Sites of involvement may be patchy and focal
Treatment for giant cell arteritis ?
Steroids or antitumor necrosis factor therapy
What is Takayasu arteritis?
narrowing of lumen; vasculitis of aortic arch and major branch vessels
Pulseless disease
Pulmonary, coronary and renal aa may be involved
upper extremities pulseless
Giant cell arteritis typically seen in what pts?
50 and older
Describe a typical pt with Takayasu arteritis?
Weak pulse and low BP in upper extremities
Less than 50 y/o
ocular and neurologic disturbances
HTN occuring secondary to renal a disease
What are the histological features of Takayasu arteritis?
same as Giant cell arteritis but in a pt less than 50
What is Polyarteritis nodosa? PAN?
Systemic vasculitis
Immune complex mediated
Necrotizing vasculitis involving small-medium aa
No ANCAs
What vessels are involved in Polyarteritis nodosa (PAN)?
renal vessels Heart Liver GI tract NOT pulmonary vessels
Describe a typically pt with PAN?
any age group, but classically young
1/3 have chronic hep B
(HBsAg-HBsAb)
What is usually effective in treating Polyarteritis nodosa?
Immunosuppressive therapy
Describe the lesions seen in Polyarteritis nodosa?
Transmural necrotizing inflammation - contains neutrophils, eosinophils, lymphocytes and Mo
Fibroid necrosis of vessel wall
Inflammation not circumferential
Thickened intima
In PAN, the inflamed vessel wall may become susceptible to what?
thrombus formation/occlusion -> ischemic
Aneurysm
Rupture
What is Kawasaki disease?
Acute arteritis of infants and small children (80% less than 4 yo)
What vessels does Kawasaki disease typically involve
Coronary arteries
What can Kawasaki disease lead to?
Affected sites may form aneurysms -> thrombosis or rupture -> acute MI
What is the presenting picture of Kawasaki disease?
Erythema of conjunctiva, oral mucosa, palms and soles
Rash
Cervical LN enlargement (mucocutaneous LN syndrome)
What is the treatment for kawasaki disease?
Usually self-limited.
IVigs and aspirin are indicated to lower risk of cornonary event
What is Microscopic polyangiitis?
Necrotizing vasculitis involving arterioles, capillaries and venules
What vessels are affected in microscopic polyangiitis?
Vessels of many organ systems
Renal glomeruli and lung capillaries most common
What are commonly associated with Microscopic polyangiitis?
Necrotizing glomerulonephritis (90%) and pulmonary capillaritis MPO-ANCA
Describe the histologic findings of Microscopic polyangiitis
Segmental necrotizing inflammation with fibrinoid necrosis
Many apoptotic neurtrophils w/i and around vessel walls (leukocytoclastic vasculitis)
What can improve long-term survival w Microscopic polyangiitis?
Cyclosporine and steroids
What is Churg-Strauss syndrome?
Small vessel necrotizing vasculitis
Associated with Athma, allergic rhinitis, hyereosinophilia, lung infiltrates, extravascular granulomas
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
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
What is Behcet disease
Vasculitis of small-medium vessels w the additional findings:
Aphthous ulcers of oral cavity
Genital ulcers
Uveitis
What is the typical triad that presents with Bechet disease?
Apthous ulcers of the oral cavity
Genital ulcers
Uveitis
Describe vessel inflammation in Bechet disease
neutrophilic and morhpologically nonspecific
May involve visceral organ systems with subsequent aneurysm formation
What is the marker for Microscopic polyangiitis
MPO-ANCA
What is the marker for Bechet disease?
HLA-B51
What is mortality related to in a pt with Bechet disease?
Sever neurologic involvement or rupture of vascular aneurysms
What is effective i the treatment of Bechet disease?
Steroids or TNF-antagonist therapies
What is Granulomatosis with polyangiitis?
GPA or Wegener granulomatosis
Necrotizing vasculitis
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
What is the marker for Granulomatosis with polyangiitis?
PR3-ANCA - 95%
What is the Tx for granulomatosis with polyangiitis?
Immunosuppressive therapy
Cyclophosphamide, steroids, and TNF antagnonists
*untreated, 80% mortality in one year
What are the clinical features of granulomatosis with polyangiitis?
Male, 40 yo
Persistent pneumonitis and sinusitis, renal disease, and nasopharyngeal ulceration
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
What is thromboangiitis obliterans?
Acute and chronic thrombosing vasculitis of small and medium vessels
Tibial arteries
Radial arteries
Describe a pt with thromboangiitis obliterans
Young adult Smokers Vasculitis in distal extremities Spasms Vascular insufficiency of extremities
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
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
What is Raynaud?
Excessive vasospasm of small arteries and arterioles
Fingers and toes
What are the symptoms of Raynaud?
pain
pallor
Cyanosis
Prlonged vasospasm -> tissue necrosis
What is primary Raynaud?
induced by cold or emotion Symmetric involvement of digits 3-5% general population Young women Benign course
What is secondary raynaud phenomenon?
Component of another arterial disease such as SLE Scleroderma Thromboangiitis obliterans Asymmetric involvement of digits Worsens with time
What is myocaardial vessel vasospasm?
Excessive vasoconstriction of myocardial aa or arterioles
Cardiac raynaud
Histo: contraction band necrosis
Myocardial vessel vasospasm may cause what?
ischemia or infarct
sudden cardiac death
Fatal arrhythmia
Ischemic dilated cardiomyopathy
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
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)
Varicose veins are common with what type of pts?
10-30% of adults
Obesity and pregnancy increase risk
Hereditary venous defects
Prolonged standing
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)
Why are esophageal varices clinically important?
they may fatally rupture
Called exsanguination
what are hemorrhoids?
dilation of venous plexus at anorectal junction
Extremely common
Pain, bleeding and may ulcerate
Secondary to pregnancy and chronic constipation
What is thrombophlebitis?
Venous thrombosis and inflammation
What does thrombophelbitis almost always involve?
> 90% deep veins in the legs
Can be asymptomatic
What leads to increased risk of DVT in LE ?
prolonged inactivity
Systemic hypercoagulability - Factor V leiden
What is Trousseau sign?
migratory thrombophlebitis
Malignancy-associated hypercoagulability
Pts w cancer may experience hypercoagulability as a paraneoplastic syndrome
What is Trousseau sign often seen with?
mucin-producing adenocarcinomas
Mucin - thrombogenic
Adenocarcinomas of lung, ovary, pancreas
What is the classic case of Trousseau sign?
Venouts thromboses appear at one site, disappear, and reappear at a different site
What are hemangiomas?
Common benign tumor showing localized increase in neoplastic blood vessels
Sites: skin, mucous membranes of head/neck and liver
Describe capillary hemangioma
Most common
Skin, mucous membranes
Unencapsulated
Thin-walled capillaries, tightly packed together
Congenital (juvi/strawberry) hemangiomas
Variant of capillary hemangioma
Present at birth
Grows rapidly then begins to regress at 1-3 years
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
Describe Pyogenic granuloma
Lobular capillary hemangioma Not pyogenic or granuloma Ulcerated polypoid variant Proliferating capillaries Rapidly growing, often oral mucosa - ulcerate
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
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
What is a glomus tumor?
Benign tumors arising from glomus bodies (thermoregulation), Distal fingers Smooth muscle origin PAINFUL Excision curative
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
How can bartonella bacilli be identified?
PCR or visualized with Warthin-Starry stain
What is an effective treatment for Bacillary angiomatosis?
Macrolide antibiotics
What is epithelioid hemangioendothelioma
Neoplastic endothelial cells Plump and cuboidal - resemble epithelium Vascular channels difficult to recongize Metastasisin 20-30% Variable clinical behavior
What is Kaposi sarcoma?
Vascular tumor caused by HHV8
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
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
What is African/edemic KS?
Not associated with HIV
Pts younger than 40
Can involve LN
Cutaneous lesions are sparse, can be aggressive
What is Transplant-associated KS?
Not associated with HIV
T cell immunosuppression
Can spread to LN and viscera can be aggressive and fatal
Describe patches
Pink-purple, usally confined to distal LE
Dilated, irregular EC-lined spaces, interspersed lymphocytes, plasma cells, an Mo
Describe raised plaques
Dilated, jagged vascular channels lined by plump spindle cellsaccompanied by perivascular aggregates
Describe nodular lesions
neoplasic
LN and visceral involvement
African and AIDS associated diseases
Plump spindle-shaped cells, slitlike vascular spaces filled with erythrocytes
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
What would best confirm angiosarcoma?
CD31
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
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
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
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
