Vasculitis: VASCULITIS Is A Primary Inflammatory Disease Process of The Vasculature
Vasculitis: VASCULITIS Is A Primary Inflammatory Disease Process of The Vasculature
Vasculitis: VASCULITIS Is A Primary Inflammatory Disease Process of The Vasculature
Division of Rheumatology
Vasculitis 1
Determinants of the Clinical Manifestations of Vasculitis:
Classification of Vasculitis
Large-sized Vessels
Giant Cell Arteritis
Takayasu’s Arteritis
Medium-sized Vessels
Polyarteritis Nodosa
Kawasaki’s Disease
Small-sized Vessels
Anti-Neutrophil Cytoplasmic Ab (ANCA) Associated
Wegener’s Granulomatosis
Microscopic Polyangiitis
Churg-Strauss Syndrome
Vasculitis 2
Classification of Vasculitis
Small-sized Vessels(cont.)
Immune-Complex mediated:
Henoch-Schonlein purpura
Cryoglobulinemia
Hypocomplementemic Urticarial Vasculitis
Vasculitis associated with SLE, Rhuematoid
arthritis, or other autoimmune diseases
Serum-sickness or drug-induced vasculitis
Classification of Vasculitis
Vasculitis 3
Sequelae of Vasculitis
Stenosis and/or occlusion of involved
vasculature resulting in organ ischemia
or infarction
Diagnostic Approaches
Vasculitis 4
Giant Cell Arteritis
(Temporal Arteritis)
Vasculitis 5
Vasculature involved
Thoracic aorta and major branches:
Vasculitis 6
Muscular Artery
adventitia
media
intima
Vasculitis 7
Temporal Artery Biopsy
Giant Cell
Vasculitis 8
Clinical Manifestations
Constitutional
Fatigue
Weight loss
Fever
Headache
66% of patients
Most commonly temporal, but frontal or occipital
pain also common
Jaw pain(claudication)
30% of patients
Clinical Manifestations
Visual loss
Acute onset partial or complete visual
field loss in 15% of patients
Arm claudication
5% of patients
Vasculitis 9
Laboratory Abnormalities
Vasculitis 10
Giant Cell Arteritis Pathogenesis
Vasculitis 11
Giant Cell Arteritis Pathogenesis
Vasculitis 12
Optic Nerve Ischemia
Vasculitis 13
Thoracic Aortic Aneurysm
Diagnosis
Vasculitis 14
Giant Cell Arteritis of Temporal Artery
Treatment
Glucocorticoids
Prednisone 1 mg/kg q d with
tapering regimen over 4-6 months
Vasculitis 15
Polyarteritis Nodosa
Vasculature involved
Vasculitis 16
Epidemiology of Polyarteritis Nodosa
Age: 20-70 years-old
Incidence
2-4/1,000,000 annual incidence
Vasculitis 17
Prognosis of Polyarteritis Nodosa
Vasculitis 18
Polyarteritis Nodosa
Clinical Manifestations
Constitutional symptoms
Fatigue
Weight loss
Fever
Gastrointestinal
Abdominal pain
Abdominal catastrophes
Shock secondary to aneurysmal rupture and
resultant hemorrhage
Shock secondary to sepsis from intestinal ischemia
or infarction
Vasculitis 19
Clinical Manifestations
Kidney
Hypertension
Renal Insufficiency
Skin
Nodules or ulcers
Purpura
Digital gangrene
Vasculitis 20
Angiogram of Superior Mesenteric Artery
Vasculitis 21
Vasculitis of Interlobar Artery of the Kidney
Renal Arteriogram
Vasculitis 22
Vasculitis 23
Dermal Vasculitis
Dermal Vasculitis
Vasculitis 24
Mononeuritis Multiplex
Nerve Biopsy
Vasculitis 25
Digital Gangrene
Treatment
5 yr survival untreated: 13%
Disease onset
Prednisone 1 mg/kg q d
Oral cyclophosphamide 2 mg/kg q d
Duration of treatment
At least one year
+HBV PAN
Interferon-α
Lamivudine
Vasculitis 26
Wegener’s Granulomatosis
Granuloma
Vasculitis 27
Vasculature involved
Upper respiratory tract arterioles and capillaries
Kidney
Glomerulonephritis (“pauci immune”)
No immune deposits
Skin
Vasculitis 28
Epidemiology of Wegener’s Granulomatosis
Prevalence: 5-7/100,000
Clinical Manifestations
Upper Respiratory Tract
Chronic Sinusitis
Chronic Otitis
Lower Respiratory Tract
Pulmonary nodules
Alveolar hemorrhage(hemoptysis)
Kidney
Glomerulonephritis(crescentic)
Peripheral Nervous System
Mononeuritis multiplex
Skin
Purpura
Vasculitis 29
ANCA associated
Vasculitis 30
ANCA in Wegener’s Granulomatosis
Vasculitis 31
Mortality of Wegener’s Granulomatosis
Vasculitis 32
Pulmonary Nodules
Granulomatous Inflammation
Vasculitis 33
Pulmonary Hemorrhage
Vasculitis 34
Necrotizing Glomerulonephritis*
* “Pauci-immune” Glomerulonephritis
Palpable Purpura
Vasculitis 35
Palpable Purpura
Vasculitis 36
Treatment Regimen
Prednisone 0.5-1 mg/kg q d (tapered) plus
cyclophosphamide 2 mg/kg q d for approximately
one year
85-90% response rate
75% complete remission
30-50% at least one relapse
Vasculitis 37
Henoch Schonlein Purpura
Age: 5-7 years old (range: 5-15)
Children: 20/100,000
50% preceded by upper respiratory tract infection
Adults: <1/100,000
Vasculature involved
Gastrointestinal tract
Submucosal arterioles/venules
Kidney
Glomerulonephritis(mesangial)
Skin
Dermal arterioles, capillaries, and
postcapillary venules
Vasculitis 38
Clinical Manifestations
Abdominal pain (“purpura” of the small
bowel, i.e., submucosal hemorrhage)
Intussusception
Hematuria/proteinuria
Renal insufficiency infrequent
Purpura
Arthralgia/arthritis
Pathogenesis
Vasculitis 39
Purpura of the Buttocks
Vasculitis 40
IgA Deposition in Dermal Vasculature
HSP Glomerulonephritis
Vasculitis 41
IgA Deposition in the Mesangium
Vasculitis 42
Treatment
Supportive
Hydration
Bed rest
Analgesia
Non-steroidal antiinflammatory
agents
Vasculitis
Edward Dwyer, M.D.
Division of Rheumatology
Vasculitis 43