Flashcards in (IV) Vasculitides Deck (40):
1. What is the definition of vasculitis? What are the vascular consequences?
Inflammation and necrosis of a blood vessel with subsequent impairment of blood flow. Vessel
wall destruction ->perforation and hemorrhage into adjacent tissues. Thrombosis -> impairment of blood flow -> ischemia/infarction of dependent tissues. Complx: accelerated 2/2 ATH of the involved vessel, which contributes to
morbidity and mortality.
Histologic features of vasculitis?
Infiltration of the vessel wall by neutrophils, mononuclear cells, and/or giant cells
• Fibrinoid necrosis (panmural destruction of the vessel wall)
• Leukocytoclasis (dissolution of leukocytes, yielding “nuclear dust”)
Perivascular infiltration is a nonspecific histologic finding & not diagnostic of vasculitis,
immune mechanism does vasculitis occur?
Primary CNS vasculitis
IC mediated vasculitis:
cut leukocytoclastic angiitis.
(+/- cellular & humoral)
Primary CNS vasculitis
IC med vasculitis
IC mediated vasculitis:
Cut leukocytoclastic angiitis.
(+/- cellular & humoral)
Microscopic polyangiitis (MPA)
Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss)
Assoc with sys dz
Assoc with probably cause
Takayasu & GCA
PAN & Kawasaki
-Hypocomplementemic urticarial (HUVS, anti-Clq vasculitis)
Bechet & Cogan
Single Organ vasc
Cutaneous leukocytoclastic vasculitis
vasc with sys dz
Sarcoid & others
Vasc with probable eitology
Hep C cryoglobulinemic vasculitis
Hep B vasculitis
Drug-assoc IC vasculitis (HSR)
Drug-assoc ANCA vasculitis
bruits, asymmetric BP, absence of pulses
digital gangrene, mononeuritis multiplex, renovascular HTN
MCC Presentation for vasc
Headache or visual loss in the elderly->GCA
asymmetric pulses + bruits + <30 y/o ->Takayasu
rapidly prog pulm–renal syn->AAV
palpable purpura->IC mediated vasculitis
When to suspect vasc mimics??
Vasculitis mimics should be suspected when there is:
1. A new heart murmur (subacute bacterial endocarditis, SBE)
2. Necrosis of lower-extremity digits (cholesterol emboli)
3. Splinter hemorrhages (SBE)
4. Prominent liver dysfunction (hepatitis C)
5. Drug abuse (human immunodeficiency virus, HIV; hepatitis B/C; cocaine, etc.)
6. Prior diagnosis of neoplastic disease
7. Unusually high fever (SBE)
8. History of high-risk sexual activity (HIV)
What disorders can mimic Large arteries?
Fibromuscular dysplasia, radiation fibrosis, neurofibromatosis type I, congenital coarctation of
aorta, genetic diseases (Marfan syndrome, Loeys–Dietz syndrome), syphilitic aortitis, IgG4 disease
What disorders can mimic Medium arteries ?
Cholesterol emboli syndrome, atrial myxoma, fibromuscular dysplasia, lymphomatoid granulomatosis,
angioblastic T-cell lymphoma, thromboembolic disease, ergotism, type IV Ehlers–Danlos syndrome,
segmental arterial mediolysis, Grange syndrome, pseudoxanthoma elasticum
What disorders can mimic Cerebral arteries ?
Reversible cerebral vasoconstrictive syndrome, reversible posterior encephalopathy syndrome,
cerebral amyloid angioapthy, CADASIL syndrome, Susac syndrome, progressive multifocal leukoencephalopathy,
Moyamoya disease, intravascular lymphoma, infections
What disorders can mimic Small arteries ?
Infectious endocarditis, mycotic aneurysm with emboli, cholesterol microemboli syndrome, antiphospholipid
antibody syndrome, sepsis (gonococcal, meningococcal), ecthyma gangrenosum (Pseudomonas),
thrombotic thrombocytopenia purpura, cocaine, amphetamines, minocycline, hydralazine, HIV, hepatitis
C, amyloidosis, systemic rheumatic diseases (systemic lupus erythematosus, SLE; Sjögren’s syndrome), bacteremias
(SBE, Rickettsia), other systemic viral infections, common variable immunodeficiency, calciphylaxis,
livedoid vasculopathy (atrophie blanche), malignant atrophic papulosis (Degos disease)
To rule out vasculitis mimics:,
consider blood cultures, viral hepatitis studies, HIV testing, urinary toxicology
screening, echocardiography, antinuclear antibody (ANA), rheumatoid factor (RF), antiphospholipid antibodies,
and/or angiography/magnetic resonance angiography, depending on the clinical situation
Jaw claudication, visual loss, palpable, thickened,
tender temporal artery, or
Diminished temporal artery pulsation
Giant cell arteritis (GCA)
Absent radial pulses, difficulty obtaining a blood pressure
in one arm
Takayasu arteritis or large artery involvement in GCA
Sinus involvement, otitis media, scleritis
GPA (Wegener) or EGPA (Churg–Strauss syndrome)
Hypertension, renal vascular involvement
Polyarteritis nodosa or Takayasu arteritis
EGPA (Churg–Strauss syndrome)
Pulmonary–renal syndromes (hemoptysis and glomerulonephritis)
GPA (Wegener) and microscopic polyangiitis
subcutaneous nodules, “punched-out” skin ulcers, livedo reticularis, digital
palpable purpura, splinter hemorrhages, hemorrhagic macules, vesiculobullous
and urticaria lasting >24 hours
Primary systemic vasculitis never causes pancytopenia (must rule out SLE, B cell lymphoma, myeloma,
Westergren ESR and CRP. ESR >100 mm/h and CRP >10 mg/dL in the absence of bacterial infection and
widespread cancer should suggest vasculitis.
if against serine proteinase 3, usually GPA; sometimes MPA
highly specific for GPA with widespread systemic involvement
if against myeloperoxidase, consider MPA and EGPA; sometimes GPA.
If the p-ANCA is not against myeloperoxidase, inflammatory diseases
other than vasculitis should be considered (inflammatory bowel disease, infections). In some patients, ANCAs
may have predictive value for relapses and ongoing disease activity. ANCAs may be pathogenic as part of
several simultaneous or sequential events.
When are hepatitis serologies helpful when vasculitis is suspected?
The presence of hepatitis B surface antigen may be found in some patients (10% to 25%, depending on risk
factors) with PAN. Hepatitis C antibodies are often found in patients with essential mixed cryoglobulinemic
vasculitis and rarely in PAN.
What is the role of tissue biopsy in the diagnosis of vasculitis and in what type of vasculitis might
tissue biopsy be helpful?
Tissue biopsy is unquestionably the procedure of choice in the diagnosis of vasculitis. Some frequently
approached biopsy sites are as follows:
• Common sites: Skin, sural nerve (PAN, EGPA; only biopsy if abnormal electromyogram [EMG]/nerve
conduction velocity [NCV]), temporal artery (GCA), muscle (PAN), kidney (GPA, MPA; rare to see
vasculitis, usually see focal necrotizing glomerulonephritis with or without crescents), lung (GPA, MPA)
• Less common sites: Testicle (PAN), rectum/gut, liver, heart, brain (primary CNS vasculitis), sinus (GPA)
16. If tissue biopsy is not feasible, what alternative procedures can yield a diagnosis?
Angiography at the following sites may be helpful for diagnosis of certain types of vasculitis:
• Abdomen (celiac trunk, superior mesenteric, and renal arteries) for diagnosis of PAN
• Aortic arch for diagnosis of Takayasu arteritis and GCA with large-vessel involvement.
• Extremities for diagnosis of Buerger disease
• Cerebral sites for diagnosis of primary CNS vasculitis
List two characteristic (but not diagnostic) angiographic features of vasculitis
Irregular tapering and narrowing & Aneurysms (“beading”)