6. Pathogenesis
• Direct attack by an agent.
• A process directed specifically at
components in vascular tissue (e.g.
ANCA, anti-basement membrane
disease).
• Secondary to an inflammatory process
(e.g. immune complex disease).
7. Antineutrophil cytoplasmic
antibodies, ANCA
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
15. MONONEURITIS MULTIPLEX
Peripheral neuropathy of more than one
nerve roots.
Pathogenesis: nerve infarction resulting
from widespread destruction of
epineural arterioles.
Causes: vasculitis (esp PAN), diabetes
or other autoimmune conditions (eg.
SLE, RA).
16. Vasculitis syndrome (Vasculitides)
1.
2.
3.
Diagnostic triad:
Palpable purpura
Glomerulonephritis
More than three organs involved
–
–
–
–
–
–
Fever, fatigue, weight loss, granuloma
Nerve
Airway (ENT, Lung)
Allergy
GI
Musculoskeletal
18. Giant Cell Arteritis
(Temporal arteritis)
• Occurring primarily over the age of 50
years.
• Frequent features include fatigue,
temporal headaches, jaw claudication,
loss of vision, scalp tenderness,
polymyalgia rheumatica and aortic arch
syndrome.
• Rarely involves the skin, kidneys and
lungs.
• The ESR is usually highly elevated
21. Polymyalgia Rheumatica
Myalgia, low-grade fever, fatigue, weight loss and
an elevated ESR.
May combined with temporal arteritis
Morning stiffness is usually the predominant
feature
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
years.
22. TAKAYASU’S ARTERITIS
Affecting aorta and its major branches.
most commonly in females under 40 years of
age.
Clinical features
• Systemic phase: malaise, fever, night
sweats and fatigue.
• Occlusive phase: upper limb claudication,
headaches, postural dizziness and visual
disturbances.
• Reduced or absent upper limb pulses.
• Arterial bruits over the carotid, abdominal
and subclavian vessels.
28. Kawasaki disease
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.
30. Granulomatosis with polyangiitis, GPA
2011-4 前舊名 WEGENER'S GRANULOMATOSIS
韋格納氏肉芽腫 (WG)
Young and middle age adults
Small and occasionally medium-sized
vessel vasculitis
Midline granuloma
– 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
C-ANCA related.
31. Eosinophilic granulomatous angiitis
(CHURG–STRAUSS SYNDROME)
Small sized artery granulomatous
inflammation.
Clinical features
• History of atopy.
• Constitutional symptoms – fever, anorexia,
weight loss.
• Asthma.
• Peripheral neuropathy.
• Skin involvement – nodular lesions over
pressure areas.
• Peripheral eosinophilia.
33. 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
necrosis.
Systemic involvement – arthritis,
glomerulonephritis, gastrointestinal
hemorrhage or colic are most common.
Skin biopsy: leukocytoclastic vasculitis
Kidney biopsy: pauci-immune segmental
necrotizing glomerulonephritis
36. IgA Vasculitis
(Henoch–Schönlein purpura)
A specific vasculitic
syndrome which is due to
IgA immune complexes
and IgA deposition within
the vessels and the kidney.
mainly in children and
young adults
The full spectrum includes
palpable purpura, nephritis,
arthritis and gastrointestinal
involvement.
39. Antiphospholipid Antibodies Syndrome (APS)
1 clinical + 1 lab criteria
Clinical criteria
1. Vascular thrombosis
» arterial, venous, or small-vessel thrombosis
1. Pregnancy morbidity
» One or more late-term (>10 weeks’ gestation) spontaneous
abortions
» One or more premature births at or before 34 weeks’
gestation
» Three or more spontaneous abortions before 10 weeks’
gestation
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
41. Recurrent oral
ulceration
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:
Recurrent
genital
ulceration
Eye lesions
Aphthous ulceration or scarring observed by physician or
patient
Skin lesions
Positive
pathergy test
Anterior uveitis, posterior uveitis, or cells in vitreous on
slit lamp exam.; or retinal vasculitis observed by
ophthalmologist
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.
45. Steroid Therapy
Glucocorticoids
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
47. Indication for addition of
cytotoxic drug to prednisone
on initial evaluation, rapidly
progressive vasculitis with significant
visceral involvement
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
disease
48. Other Treatment of Vasculitis
1.
2.
3.
4.
5.
Aspirin
Anticoagulants
Plasmapheresis
Intravenous immunoglobulin, IVIG
Biological agents
1. Anti-TNFα: (−)
2. Anti-CD20, Rituximab for ANCA-associated
vasculitis
49. Conclusion
Vasculitis is a syndrome and disease
spectrum
Classification depends on vascular size
and clinical manifestations
ANCA, anti-cardiolipin antibody are key
autoantibodies
Treated by steroid and cytotoxic drugs
Notes de l'éditeur
sensitivity of 91 percent and specificity of 96 percent.