Vasculitis Flashcards by Iseoluwa Ojo

VASCULITIS refers to _____ and ____ of ____

inflammation and necrosis

blood vessels

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VASCULITIS only occurs in arteries

T/F

arteries, veins and capillaries

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In VASCULITIS

These vessels may be damaged by
__________,_________,__________,_________ or _____

immune mechanisms, infectious agents, mechanical trauma, radiation or toxins

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Majority of VASCULITIS are thought to involve immune mechanisms including;
- _______ of immune complexes
- ________ on vessels by _______
- various forms of _______

deposition

direct attack ; circulating antibodies

cell mediated immunity

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BASIC PRINCIPLES of vasculitis
Inflammation of the blood vessel wall

• Arterial wall is comprised of three layers:_____ intima, _______ media, and _________ adventitia

•Etiology is usually (known or unknown?)

endothelial; smooth muscle ; connective tissue

unknown

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BASIC PRINCIPLES of vasculitis

Clinical features include

Non specific symptoms of inflammation(e.g., ____,______,_____, and ______)

Symptoms of _______: due to _______ or ______ of the inflamed vessels

fever,fatigue, weight loss, and myalgias

Organ Ischemia; luminal narrowing or thrombosis

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BASIC PRINCIPLES of vasculitis

Divided into _________,________,_______

large-, medium-, and small-vessel

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BASIC PRINCIPLES of vasculitis

Large-vessel vasculitis involves the ____ and _________

Medium-vessel vasculitis involves _______ that supply organs.

Small-vessel vasculitis involves _____,______,________

aorta and its major branches.

muscular arteries

arterioles, capillaries, and venules.

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most cases of vasculitis are infectious.

T/F

are not infectious

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Mention 2 examples of large vessel vasculitis

Takayasu arteritis

Giant cell arteritis

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Mention 2 examples of Medium-vessel vasculitis

Polyarteritis nodosa

Kawasaki disease

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Mention 3 examples of Small-vessel vasculitis

Granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis

Microscopic polyangiitis

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Some vasculitides are associated with ________________(ANCA)

antineutrophil cytoplasmic antibodies

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Some vasculitides are associated with antineutrophil cytoplasmic antibodies (ANCA) :

These can be detected by ______ assays using the patient’s ______ and __________

indirect immunoflourescence

serum and ethanol fixed neutrophils

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C-ANCA-

P-ANCA-

Cytoplasmic antineutrophil cytoplasmic antibodies

Perinuclear antineutrophil cytoplasmic antibodies

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C-ANCA- associated with _____ antibody

P-ANCA-associated with _____ antibody

Proteinase-3- antibody

Myeloperoxidase antibody

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C-ANCA-

Majorly in _______ disease (__%)

Rarely in _____,____, and ______ disease

Granulomatosis with Polyangitis; 90

Microscopic polyangiitis; Eosinophilic granulomatosis with polyangiitis; infective endocarditis

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P-ANCA-

Majorly in _______ disease (__%), and ________(___%)

Rarely in _____, and ______ disease

Microscopic polyangiitis; 70

Eosinophilic granulomatosis with polyangiitis; 50

Granulomatosis with Polyangitis; PAN

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Atypical P-ANCA Is found in ____ and _____

Rarely in _____ and _____

PSC ; ulcerative colitis

Crohn’s disease, primary biliary cirrhosis

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GIANT CELL ARTERITIS (aka. __________,__________)

TEMPORAL ARTERITIS

GRANULOMATOUS ARTERITIS

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The most common vasculitis in older adults is ???

GIANT CELL ARTERITIS

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GIANT CELL ARTERITIS

Average age at onset is ___ years (___-___)

It involves a (focal or widespread ?) , (acute or chronic?) ,granulomatous inflammation

70; 50-75

Focal; chronic

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GIANT CELL ARTERITIS

Usually of the _______ (small or large ?) arteries and there branches

Male : Female =_:__

temporal

Large

1:3

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GIANT CELL ARTERITIS

Clinical features
Patients presents with _______ and _________

_____ symptoms occur in almost half of the patients

headache and throbbing temporal pain

Visual

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GIANT CELL ARTERITIS Clinical features ______ and _______ are present in the skin overlying the affected artery About half with clinical manifestation will have ______ involvement

Swelling and tenderness systemic

Lesions of temporal arteritis are focal and may be missed on biopsy T/F

GIANT CELL ARTERITIS Biopsy of the temporal artery may be negative in ____% of patients with classical manifestation of this disease

GRANULOMATOUS ARTERITIS Treatment These patients are treated with ____ and the response is (disappointing or excellent?)

steroids Excellent

TAKAYASU ARTERITIS (_____ syndrome) Aetiology: _______ basis has been proposed Refers to an inflammatory disorder of the ______ and ________

Aortic arch An autoimmune aortic arch and its major branches

TAKAYASU ARTERITIS Affects (young or old?) (men or women?) (<__years), 15-25 Male : Female= __:__

Young Women 30 1:9

TAKAYASU ARTERITIS Pathology Aorta is _____ and has _______

thickened; focal raised plaques

Aortic arch syndrome Pathology Branches of the aorta display ______ and _____, interfering with _____ and accounting for the synonym ‘ ______’

stenosis and occlusion blood flow pulseless disease

TAKAYASU ARTERITIS Clinical features General: Fever, malaise, arthralgia, night sweats Specific: Vascular symptoms Decreased bilateral _____ and _____ (so- called pulseless disease) _____,______ Bilateral carotid bruits Impaired _____ _____-induced muscular pain in one or more limbs _______ phenomenon Hypertension

brachial and radial pulses Syncope, angina pectoris vision; Movement; Raynaud

POLYARTERITIS NODOSA (PAN) A multisystemic disease This acute, necrotizing, vasculitis affects mainly __________-sized muscular arteries

small to medium

POLYARTERITIS NODOSA (PAN) Male:Female =___:__ 40-60 years

2:1

POLYARTERITIS NODOSA (PAN) __-ANCA (15 – 30% of cases) immunoflourescence mainly against _________

P myeloperoxidase

Pathology of PAN every vessel of appropriate size would be affected

F Not every vessel of appropriate size would be affected so a thorough search of the slide would be necessary

Pathology of PAN Lesions are _____ and may not involve the ________ of the vessel

patchy entire circumference

Pathology of PAN Most prominent feature is an area of the __________, fused into ________

media and adventitia an eosinophilic mass

Inflammatory response in PAN involves only some of the adventitia

F the entire adventitia (periarteritis)

In PAN Healed lesions have a _____ media & loss of fragmentation of the _________

fibrotic internal elastic lamina

internal elastic lamina is ??

What separates the intima from the media

Clinical features of PAN specific symptoms ____ involvement ________ involvement ____ involvement ______ involvement ___ involvement

Renal Coronaryartery Skin Neurological GI

Clinical features of PAN Renal involvement (∼___%): _______ , renal impairment Coronary artery involvement(∼__%) : increased risk of ________ Skininvolvement(∼___%): ____,_______, nodules

60; hypertension 35; myocardial infarction 40; rash,ulcerations

Clinical features of PAN Neurological involvement: polyneuropathy ( __________ ), ________ GI involvement: ______,_______, nausea, vomiting

mononeuritis multiplex; stroke abdominal pain, melena

PAN Usually spares the _____

lungs

KAWASAKI DISEASE (______ SYNDROME)

MUCOCUTANEOUS LYMPH NODE

KAWASAKI DISEASE (acute or chronic?) necrotizing vasculitis of _________ and _________

Acute; infancy; early childhood

KAWASAKI DISEASE i) _____ ii) ____ iii) conjunctival and ____ lesions (characteristic “___________” iv) lymphadenitis

Fever; rash ; oral Strawberry tongue

Kawasaki disease Male : Female = __:__

1:5

Strawberry tongue – {also seen in _______ besides Kawasaki

scarlet fever

Clinical features of Giant cell Artertis • Hardened and tender temporal artery Jaw _______ _____ loss Scintillating ______ ________ or permanent loss of vision Diplopia

claudication Vision scotoma Amaurosis fugax

Clinical features of Giant cell Artertis • Hardened and tender temporal artery Jaw claudication:______ when ______ Vision loss: due to _____ and ______ of the _______ and its branches Amaurosis fugax or __________ Diplopia

jaw pain when chewing inflammation and occlusion ophthalmic artery permanent loss of vision

In PAN, Small aneurysm may form T/F

Treatment of PAN ________ _______ therapy against ______ and ____ may be required.

Immunosuppression Antiviral HBV and HCV

KAWASAKI DISEASE Affects ____________ sized vessels

large to medium

Kawasaki disease May affect the ____ arteries and cause _____ in ____% _______ cause is suspected

coronary aneurysms 70 An infectious

Kawasaki Disease is not usually self-limited T/F

F Disease is usually self-limited

THROMBOANGIITIS OBLITERANS Occlusive inflammatory disease of _________ arteries in the _______ and _____

medium and small distal arms and legs

BUERGER DISEASE Exacerbated by ______ which plays an aetiological role in the disease

smoking

THROMBOANGIITIS OBLITERANS An increased prevalence of ____ and ____ haplotypes among patients with the disease lends credence to the idea that a genetic _________ is involved in the pathogenesis.

HLA-A9 and HLA-B5 Hypersensitivity to tobacco

THROMBOANGIITIS OBLITERANS Aka _________ DISEASE

BUERGER

BUERGER DISEASE Pathology Acute inflammation of the medium and small arteries, with involvement of the endothelium May lead to ______ and ____ of the lumen

thrombosis and obliteration

THROMBOANGIITIS OBLITERANS Symptoms usually begins between ___-___ years _______ in muscle during exercise

25- 40 Cramping pains

BUERGER DISEASE More in male or female? Male : Female = __:__

Female 1:9

BUERGER DISEASE Painful ischaemic disease can lead to ____ of the extremity with possible necessity of ____

gangrene amputation

HYPERSENSITIVITY ANGIITIS This is a broad category of inflammatory vascular lesions representing a reaction to foreign materials such as ______ or _____

bacterial products or drugs.

HYPERSENSITIVITY ANGIITIS It includes; A)___________ B)_____________

Cutaneous vasculitis Systemic hypersensitivity angiitis

HYPERSENSITIVITY ANGIITIS A) Cutaneous vasculitis i) May follow administration of drugs such as ____,______, and ____ ii) _____ or _____ infections

aspirin, penicillin and thiazide diuretics Bacterial or viral

HYPERSENSITIVITY ANGIITIS A) In Cutaneous vasculitis Palpable _____ of the lower extremities is typical Superficial cutaneous _____ display _______ and an acute inflammatory reaction The disease is usually ______

purpura venules; fibrinoid necrosis self limited

HYPERSENSITIVITY ANGIITIS Systemic hypersensitivity angiitis Also called __________ May also exhibit ______

microscopic polyarteritis skin lesions

microscopic polyarteritis Is always a feature of other vascular diseases

F May be an isolated entity or a feature of other vascular diseases

microscopic polyarteritis A feared complication is _____ involvement, characterized by rapidly progressive _______ and ______

renal glomerulonephritis and renal failure

microscopic polyarteritis It is associated with the presence of cANCA T/F

T But Majorly pANCA

EOSINOPHILIC/ALLERGIC GRANULOMATOSIS WITH POLYANGIITIS Also known as ________ SYNDROME

CHURG-STRAUSS

CHURG-STRAUSS SYNDROME occurs in young people with ____ and features _____

asthma; eosinophilia

EOSINOPHILIC/ALLERGIC GRANULOMATOSIS WITH POLYANGIITIS More in male or female?) Male:Female = __:__ __-__years

Male 2:1 40-60

CHURG-STRAUSS SYNDROME Affects ___-___ sized vessels ____% exhibit C-ANCA or P-ANCA

small to medium 70

CHURG-STRAUSS SYNDROME There is widespread necrotizing vascular lesions of ________ sized arteries in multiple organs

small and medium

ALLERGIC GRANULOMATOSIS AND ANGIITIS There is prominent involvement of the _____ Other organs involved are the ____,_____,_____, and _____

lungs kidneys, heart, liver and CNS

CHURG-STRAUSS SYNDROME Clinical features: ___________ attacks (chief complaint), Allergic _________, Skin ____, palpable _____ Gastrointestinal involvement: bleeding, ischemia, perforation

Severe allergic asthma rhinitis/sinusitis nodules; purpura

WEGENER GRANULOMATOSIS aka. “__________________ ” A (Local or Systemic?) necrotizing vasculitis

Granulomatosis with polyangiitis Systemic

WEGENER GRANULOMATOSIS Characterized by lesions of the ____,______ ,_____, and ___________ disease Aetiology is (known or unknown?)

nose, sinuses, lungs and renal glomerular unknown

WEGENER GRANULOMATOSIS Happens in who more?…men or women? Male:Female = __:___

Both equally 1:1

Granulomatosis with polyangiitis Affects ____ arteries >___% have ANCA in their blood; of those ___% are ___-ANCA, which suggests that _________ are responsible for the attack

small 90; 75 C; activated neutrophils

WEGENER GRANULOMATOSIS Pathology Features _______ necrosis and granulomatous inflammation composed of neutrophils and other leukocytes Mostly in the _____,_____, and ______

parenchymal respiratory tract, kidney and spleen

WEGENER GRANULOMATOSIS Immunosuppressive treatment with __________ leads to striking improvement

cyclophosphamide

WEGENER GRANULOMATOSIS Clinical Features Persistent bilateral ______ and chronic ____ are prominent _______ and ______ indicate glomerular involvement

pneumonitis; sinusitis Haematuria and proteinuria

RAYNAUD’S DISEASE Refers to _____ and ______ of the digits of the hands, feet, tip of the nose and ears

paroxysmal pallor and cyanosis

RAYNAUD’S DISEASE It is due to intense ________ in (old or young?) healthy (men or women?) It is often accompanied by ____ and _____

local vasospasm Young ; women paraesthesia and pain

RAYNAUD’S DISEASE Usually ___ organic change in vessel wall except later on when there is _____ Usually bilateral and symmetrical and may lead to ______ and ____ of the _____

No; intimal proliferation ulcers and gangrene of the tips of the digits

RAYNAUD’S PHENOMENON Refers to arterial _____ secondary to arterial ______

insufficiency narrowing

BEHCET DISEASE (Focal or Widespread?) vasculitis

Widespread vasculitis of many organs

BEHCET DISEASE Characterized by _____ and ______ ; ____ inflammation; occasional lesions in the CNS, GIT & CVS ___________ vessels are involved

oral and genital ulcers ocular Large and small

BEHCET DISEASE 20 - 35 years Who gets it more?..men or women Male:Female = ___:__

Both equally 1:1

INFECTIOUS ARTERITIS Caused by ______ _____ or _______ commonly In bacterial/TB ________, bacterial ______ or pulmonary TB

direct invasion Aspergillosis or mucormycosis meningitis; pneumonia

INFECTIOUS ARTERITIS It is important because it induces _____ leading to _____, worsening the condition

thrombosis infarct

RAYNAUD’S PHENOMENON Refers to arterial insufficiency secondary to arterial narrowing induced by various conditions such as i) ___ ii) _____ iii) ________

SLE SCLERODERMA BUEGER’S DX

More in male or female? GIANT CELL ARTERITIS - _____ Aortic arch syndrome- _______ PAN- ______

female more female only male more

More in male or female? Kawasaki- _________ BUERGER DISEASE- _______ CHURG-STRAUSS SYNDROME- _______

female more female more male more

More in male or female? BEHCET DISEASE- ____ RAYNAUD’S DISEASE- ______ WEGENER GRANULOMATOSIS- _____

equal women only equal

Which vasculitis have the following gender distribution Equal - _____________________ Men- ____________________

wegner’s and bechet churg Strauss and PAN

Name and nickname! Takayasu Kawasaki BUEGER’S disease Churg Strauss Wegner’s Microscopic polyangitis

Pulseless disease/ aortic arch syndrome Mucocutaneous lymph node syndrome Thromboangiitis obliterans Eosinophilic granulomatosis with polyangitis granulomatosis with polyangitis Systemic hypersensitivity angiitis

Names and nicknames!!! Pulseless disease/ aortic arch syndrome Mucocutaneous lymph node syndrome Thromboangiitis obliterans Eosinophilic granulomatosis with polyangitis granulomatosis with polyangitis Systemic hypersensitivity angiitis

Takayasu Kawasaki BUEGER’S disease Churg Strauss Wegner’s Microscopic polyangitis

________ vaaculotis mainly causes aneurysm _______can too

Kawasaksi PAN

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Vasculitis Flashcards

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