VASCULITIS

ANAPI. ARAGON. DE LEON. SAMSON.

OUTLINE:
A. Introduction
B. Diagnostic Classification
C. Large Vessel Vasculitis
1. Takayasu Arteritis
2. Giant Cell Arteritis
D. Medium Vessel Vasculitis
1. Polyarteritis Nodosa
2. Kawasaki Disease
E. Small Vessel Vasculitis
1. ANCA-Associated Small Vessel
Vasculitis
2. Anti-Glomerular Basement Membrane
Disease
3. Cryoglobulinemic Vasculitis
4. IgA Vasculitis (Henoch-Schönlein
Purpura)
5. Hypocomplementemic Urticarial
Vasculitis
6. Rheumatoid Vasculitis
 DEFINITION and DIAGNOSTIC EVALUATION
• VASCULITIS- inflammation of blood vessel walls
• Has diverse signs and symptoms
• Arterial wall is comprised of intima, media, and adventitia
• Etiology usually unknown, usually non infectious
• Clinical features include
1. Nonspecific symptoms of inflammation (e.g., fever, fatigue, weight loss, and
myalgias)
2. Symptoms of organ ischemia—due to luminal narrowing or thrombosis of the
inflamed vessels
 DEFINITION and DIAGNOSTIC EVALUATION
• Divided into:
1. Large-vessel vasculitis involves the aorta and its major
branches.
2. Medium-vessel vasculitis involves muscular arteries that
supply organs.
3. Small-vessel vasculitis involves arterioles, capillaries, and
venules.
 DEFINITION and DIAGNOSTIC EVALUATION
• Vasculitis can be categorized into infectious vasculitis and non
infectious
• e.i. infectious vasculitis of Rocky Mountain spotted fever (RMSF) is caused by
proliferation of Rickettsia rickettsii in endothelial cells and smooth muscle
cells in small vessels in many tissue
• E.i. Laboratory tests for cryoglobulinemic vasculitis comprise identification of
the infectious agent (hepatitis C virus serology), the autoantibodies
(rheumatoid factor assay), the pathogenic immune complexes (cryoglobulin
assay), secondary disturbances in inflammatory mediators (e.g.,
hypocomplementemia), and the evidence for end organ damage (hematuria
and proteinuria).
 DEFINITION and DIAGNOSTIC EVALUATION
• The most direct laboratory test for RMSF is immunohistologic
visualization of R. rickettsii in vessel walls in a skin biopsy specimen.
DEFINITION and DIAGNOSTIC EVALUATION
 LARGE VESSEL VASCULITIS
• Vasculitis affecting large arteries more often than other vasculitides.
Large arteries are the aorta and its major branches. Any size artery
may be affected.
• Demographic and clinical features are required to make the
distinction, especially age
 LARGE VESSEL VASCULITIS
GIANT CELL ARTERITIS/ TEMPORAL ARTERITIS
-patients more than 50 years old
- More common in Western countries
- inflammation of the aorta or larger arteries, with a
predominance of mononuclear leukocytes often
accompanied by multinucleated giant cells
TAKAYASU ARTERITIS
-younger than 50 years old
-usually affects women between 10 and 40 years of
age
-all race but higher in Southeast Asia, Central and
South America, Africa
- inflammation of the aorta or larger arteries with a
predominance of mononuclear leukocytes, often, but
not always, accompanied by multinucleated giant cells
 TAKAYASU ARTERITIS
Five types:
1. type I, aortic arch and its branches;
2. type II, descending thoracic and abdominal aorta;
3. type III, aortic arch and thoraco-abdominal aorta;
4. type IV, pulmonary artery in addition to the aforementioned
types; and
5. type V, coronary artery in addition to the other types
 TAKAYASU ARTERITIS
Six types:
1. type I, aortic arch and its
branches;
2. type IIa, ascending aorta,
arch, and its branches;
3. type IIb, ascending aorta,
arch, and its branches, and
descending thoracic aorta;
4. type III, thoracic aorta and
abdominal aorta;
5. type IV, abdominal aorta;
6. type V, combination of
type IIb and type IV
 TAKAYASU ARTERITIS
• Autoimmune or infectious + genes + environment = cell-mediated
immune injury
• no laboratory tests have been identified that are specific for this
disease.
• Commonly used as diagnostic evidence/support: ESR and CRP
• Biomarkers for evaluation: interleukin (IL)-6, serum amyloid A,
fibrinogen, complement split fragments, B cell activating
• factor (BAFF), interleukin (IL)-12, matrix metalloproteinase 9 (MMP-
9), and pentraxin 3 (PTX3)
 TAKAYASU ARTERITIS
• Diagnosis based on clinical signs and symptoms
• Biopsies not routine (unlike GCA)

low-grade fever,
malaise, night sweats, vasculitic stage:
pulseless disease,
arthralgia, anorexia, tenderness or pain
related to arterial
and weight loss, with over vessels
stenosis or occlusion
no clinical evidence for (angiodynia)
large vessel narrowing
 GIANT CELL ARTERITIS

• most common pathologic pattern in active GCA is transmural

inflammation (75%)

multiple HLA loci,

PTPN22, and other
intimal and medial
loci that influence immune-response
hyperplasia and
Th1, Th17, and Treg networks
fibrosis
cell function as risk
factors for GCA
 GIANT CELL ARTERITIS

• Temporal artery biopsy is the current gold standard for diagnosis of

GCA
• Other pertinent diagnostics: ESR, CRP, platelet count showing
thrombocytopenia
• Diagnosis= clinical + lab results + confirmation by biopsy
• Corticosteroids + azathioprine/prednisolone
 LARGE VESSEL VASCULITIS

• Granulomatous

Giant Cell • Branches of the carotid artery

• >50 years old
• Headache, visual disturbances, jaw claudication

Arteritis • Biopsy: inflamed vessel wall with giant cells and intimal fibrosis
• corticosteroids

• Granulomatous

Takayasu • Aortic arch

• <50 years old
• Visual and neurologic symptoms

Arteritis • Pulseless disease

• corticosteroids