• Direct attack by an agent.
• A process directed specifically at
components in vascular tissue (e.g.
ANCA, anti-basement membrane
• Secondary to an inflammatory process
(e.g. immune complex disease).
Cause of ANCA-associated vasculitis (AAV)
C-antineutrophil cytoplasmic antibodies (CANCA) against proteinase 3 (PR3)
– Associated with GPA and MPA
– Disease activity is parallel to C-ANCA titer.
P-ANCA against myeloperoxidase (MPO) is
non-specific marker for vascultiis syndrome
and other autoimmune diseases
Peripheral neuropathy of more than one
Pathogenesis: nerve infarction resulting
from widespread destruction of
Causes: vasculitis (esp PAN), diabetes
or other autoimmune conditions (eg.
Vasculitis syndrome (Vasculitides)
More than three organs involved
Fever, fatigue, weight loss, granuloma
Airway (ENT, Lung)
Giant Cell Arteritis
• Occurring primarily over the age of 50
• Frequent features include fatigue,
temporal headaches, jaw claudication,
loss of vision, scalp tenderness,
polymyalgia rheumatica and aortic arch
• Rarely involves the skin, kidneys and
• The ESR is usually highly elevated
Myalgia, low-grade fever, fatigue, weight loss and
an elevated ESR.
May combined with temporal arteritis
Morning stiffness is usually the predominant
Muscular pain is often diffuse and is accentuated
by movement; pain at night is common.
Muscle strength is unimpaired although the pain
makes interpretation of muscle testing difficult.
Rx: Corticosteroid treatment is for at least 2
Affecting aorta and its major branches.
most commonly in females under 40 years of
• Systemic phase: malaise, fever, night
sweats and fatigue.
• Occlusive phase: upper limb claudication,
headaches, postural dizziness and visual
• Reduced or absent upper limb pulses.
• Arterial bruits over the carotid, abdominal
and subclavian vessels.
An acute febrile disease occuring most
commonly in infants and children under 5
years of age.
Sore red eyes , red lips tongue or mouth.
Redness or swelling of the hands and feet.
Rash all over the body.
Vasculitis, especially of the coronary arteries,
is the most serious and life-threatening
complication of the disease.
Granulomatosis with polyangiitis, GPA
2011-4 前舊名 WEGENER'S GRANULOMATOSIS
Young and middle age adults
Small and occasionally medium-sized
– Necrosis, granuloma formation and vasculitis of
the upper and lower respiratory tracts.
Glomerulonephritis in 75% of patients.
Fever, malaise, weight loss, arthralgia, ,
sinusitis, nasal or oral ulceration, purpura
Eosinophilic granulomatous angiitis
Small sized artery granulomatous
• History of atopy.
• Constitutional symptoms – fever, anorexia,
• Peripheral neuropathy.
• Skin involvement – nodular lesions over
• Peripheral eosinophilia.
Microscopic polyangiitis, MPA
Pathogenesis is presumed to be due to
circulating immune complexes.
Cutaneous involvement – palpable purpura or
urticaria-like lesions are most common, less
common are livedo reticularis, ulcerations or
Systemic involvement – arthritis,
hemorrhage or colic are most common.
Skin biopsy: leukocytoclastic vasculitis
Kidney biopsy: pauci-immune segmental
A specific vasculitic
syndrome which is due to
IgA immune complexes
and IgA deposition within
the vessels and the kidney.
mainly in children and
The full spectrum includes
palpable purpura, nephritis,
arthritis and gastrointestinal
Antiphospholipid Antibodies Syndrome (APS)
1 clinical + 1 lab criteria
1. Vascular thrombosis
» arterial, venous, or small-vessel thrombosis
1. Pregnancy morbidity
» One or more late-term (>10 weeks’ gestation) spontaneous
» One or more premature births at or before 34 weeks’
» Three or more spontaneous abortions before 10 weeks’
Laboratory criteria: on at least 2 occasions at least 12 weeks apart
(1) anticardiolipin (aCL),
(2) anti–b2 glycoprotein I (anti-b2 GPI),
(3) Lupus anti-coagulant
A type of vasculitis
genital ulcers, and
Minor or major aphthous, or herpetiform ulceration
observed by physician or patient that recurred at least 3
times in one 12-month period
Plus 2 of the following criteria:
Aphthous ulceration or scarring observed by physician or
Anterior uveitis, posterior uveitis, or cells in vitreous on
slit lamp exam.; or retinal vasculitis observed by
Erythema nodosum observed by physician or patient,
pseudofolliculitis or papulo- pustular lesions; or acneiform
nodules observed by physician in postadolescent patients
not receiving corticosteroid Tx
Read by physician at 24-48 hr
International Study Group for Behcet's Disease: Criteria for the diagnosis of
Behcet's disease. Lancet 335:1078-1080, 1990.
Therapy of Vasculitis
with or without
2. Cytotoxic drugs
initially: 1 mg/kg/day prednisone
tapering of the dosis: 5-10 mg every week,
from 15 mg/day only 1 mg every several
week, treatment at least for 2 years
pulsus steroid: 1 g/day for 3 days
0.5 mg/kg/day after megadose pulse therapy
Indication for addition of
cytotoxic drug to prednisone
on initial evaluation, rapidly
progressive vasculitis with significant
prednisone in high daily divided dosis
is not controlling the activity and
progression of vasculitis
prednisone dose cannot be tapered
to tolerable level and still controll the
Other Treatment of Vasculitis
Intravenous immunoglobulin, IVIG
1. Anti-TNFα: (−)
2. Anti-CD20, Rituximab for ANCA-associated
Vasculitis is a syndrome and disease
Classification depends on vascular size
and clinical manifestations
ANCA, anti-cardiolipin antibody are key
Treated by steroid and cytotoxic drugs