Vasculitides Flashcards
What are the vasculitides?
A group of diseases characterized by non-infectious necrotizing vasculitis and resultant ischemia
Name the large vessel vasculitides
GCA
PMR
Takaysu’s Arteritis
Name the medium vessel vasculitides
PAN
Kawasaki disease
Name the small vessel vasculitides
WG
MPA
CSS
HSP
Cryoglobulinemic vasculitis
Hypersensitivity vasculitis
Name the Antineurtrophil Cytoplasmic Antibody
(ANCA) Associated Vasculitides
WG
MPA
CSS
Proximal muscle pain (NOT weakness)
Polymyalgia Rheumatica
Polymyalgia Rhematica is characterized by diffuse pain and stiffness primarily affecting what part(s) of the body?
The shoulders and pelvic girdle muscles
Polymyalgia Rheumatica is associated with which disease processes?
giant cell (temporal) arteritis
cancer
Is Polymyalgia rheumatica:
unilateral or bilateral?
symmetrical or asymmetrical?
bilateral and symmetrical
What patient demographic is polymyalgia rheumatica commonly seen in?
older, white women
Pneumonic for Polymyalgia Rheumatica: SECRET
S – symmetric proximal stiffness/pain
E – elderly
C – constitutional symptoms/Caucasian
R – rheumatism (arthritis)
E – ESR elevated
T – temporal arteritis
In a patient with polymyalgia rheumatica, when should you suspect cancer?
Suspect when ESR fails to normalize (Cancer may present as PMR)
Chronic vasculitis which affects large arterial branches of the aortic arch
Temporal (Giant Cell) Arteritis
Temporal (giant cell) arteritis most commonly involve which vessels?
cranial vessels or aortic arch and branches
What is the most ominous sign in temporal (giant cell) arteritis?
Vision loss
Definitive diagnosis of Temporal (giant cell) arteritis
biopsy (multiple)
Caution: skip lesions with this disease so may need multiple biopsy locations
Which disease should you expect if you see tender inflamed, cordlike temporal arteries with absent pulse on physical exam?
Temporal (giant cell) arteritis
What disease process is constantly seen with temporal (giant cell) arteritis?
Polymyalgia Rheumatica
“curtain coming down”
Amaurosis fugax
“Pulseless disease”
Takayasu’s Arteritis
Takayasu’s Arteritis targets what?
Targets aorta and major branches (Subclavian > aortic arch > ascending aorta > carotids > femoral)
Also known as “aortic arch syndrome”
What are the 6 criteria for Takayasu’s Arteritis?
Age of disease onset ≤ 40 years
Claudication of upper extremities
Decreased brachial artery pulse
Blood pressure difference >10 mm Hg between arms
Bruit over subclavian arteries or aorta
Arteriogram abnormality (Narrowing of the aorta or a major branch)
Which of the following vasculitides is described below?
Chronic vasculitis, unknown etiology
80-90% women
Age of onset 10-40
Most cases from Asia and Africa
Asian women <40 year old most common
Takayasu’s Arteritis
What is the definitive diagnosis for Takayasu’s Arteritis?
angiography
Which vasculitide is known as the classic vasculitis?
Polyarteritis Nodosa
Which vasculitide results from Hepatitis B 30% of the time?
Polyarteritis Nodosa
What organ does Polyarteritis Nodosa spare?
Lungs
Which types of medication can cause Polyarteritis Nodosa?
Sulfa
Gold
penicillamine
Which vasculitide is also known as Mucocutaneous lymph node syndrome?
Kawasaki Disease
Which of the vasculitides is primarily in children, known as a childhood vasculitis?
Kawasaki Disease
What is the typical age range for kids diagnosed with Kawasaki Disease?
Typically under 5 years of age
What vessels does Kawasaki Disease primarily affect?
affects the coronary arteries
What physical exam finding should alert you immediately to Kawasaki disease?
Strawberry tongue (prominent papillae)
What is the initial treatment for Kawasaki Disease that is typically CI in children?
ASA
What is the hallmark pathologic lesion for Wegener’s Granulomatosis?
Necrotizing, granulomatous vasculitis
What is Wegener’s Granulomatosis referred to as now?
Granulomatosis with Polyangiitis
What is the Wegener’s Granulomatosis triad?
Upper respiratory symptoms
Lower respiratory symptoms
Focal segmental glomerulonephritis
What vasculitide is described in the following description:
Can be fatal – even if you treat it
“antibody to neutrophils in their own cytoplasm”
Caucasians most common
Wegener’s Granulomatosis
How to diagnose Wegener’s Granulomatosis?
Labs: c-ANCA (Antineutrophil cytoplasmic antibody)
Definitive: biopsy
c-ANCA is seen in which vasculitide?
Wegener’s Granulomatosis
Which of the following vasculitides is described below:
Affects everybody
Present with palpable purpura
Can test with ANCA (Will be ANCA positive 70%)
Microscopic Polyangiitis
What are the five main clinical manifestations of Microscopic Polyangiitis?
Kidney inflammation ~80% (Segmental necrotizing glomerulonephritis)
Weight loss >70%
Skin lesions >60%
Nerve damage >60% (Vasculitis neuropathy)
Fevers >55%
Which vasculitide is described below:
Consists of small vessel vasculitis, extra vascular granulomas, and
hypereosinophilia
In patients with pre-existing asthma and allergic rhinitis
ANCA positive 50%
Churg-Strauss Syndrome
What vasculitide is also known as angiitis granulomatosis?
Churg-Strauss Syndrome
What organ of the body is always involved in Hypersensitivity Vasculitis?
the skin
Which vasculitide is known as the “cold antibody in the blood”?
Cryobulinemia
In Cryobulinemia, 90% of cases are associated with what?
Hepatitis C (drug use)
What is characteristic of Henoch-Schonlein Purpura?
IgA in vessel walls
What vasculitide is described below:
Immune complex-mediated vasculitis
Small vessel vasculitis
Occurs after URI or drugs
IgA in vessel walls
Males > females
Henoch-Schonlein Purpura
What are the two categories in Hypocomplementemic Urticarial
Vasculitis Syndrome?
Hypocomplementemic Urticarial Vasculitis
Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)
In Hypocomplementemic Urticarial Vasculitis, what do you need to rule out?
Rule out SLE and cryoglobulinemia
Vasculitis with triad of recurrent oral and genital ulcers, and uveitis or
iritis
Behcet’s Disease
What is a strong risk factor for Behcet’s Disease?
HLA-B51
Which vasculitide is described below:
Rare disease of young adults
Vestibuloauditory dysfunction, interstitial keratitis, and non-reactive
syphilis test
Follows URI
Questionable association with chlamydia
Cogan’s Disease
What vasculitide looks just like Meniere’s
disease – except for the age
Cogan’s Disease
Thromboangiitis obliterans
Acute inflammation, thrombosis of arteries and veins of hands and
feet
Buerger’s Disease
