Vasculitides (Vasculitis)

Dr. Raid Jastania

Vasculitis

• Inflammation of the walls of the vessels• Causes of inflammation:

– Infectious, physical, chemical, immune, metabolic, ischemic

• Most important causes: Immune and infections• May affect any site• Symptoms:

– Fever, Malaise, Weight loss… – symptoms specific to the site involved.

Classification of Vasculitis• Direct infection

– Bacterial: Neisseria– Fungal: mucor,

aspergillous– Viral: Herpes zoster

• Immunologic:– A. Immune Complex

• Infection-induced: HepB, HepC

• SLE• Drug-induced

– B. Direct antibody• Goodpasture syndrome• Kawasaki disease (anti

endothelial antibody)

– C. ANCA-mediated• Wegener granulomatosis• Microscopic polyangiitis• Churg-Strauss syndrome

– D. Cell-mediated• Rejection

– E. Unknown:• Giant cell arteritis• Polyarteritis nodosa• Takayaso arteritis

Classification of Vasculitis

Giant Cell (Temporal) Arteritis

• Most common vasculitis

• Acute, or chronic, Granulomatous

• Affect large-small arteries

• Common in: Temporal artery, vertebral arteries, ophthalmic artery

Giant Cell (Temporal) Arteritis

• Clinical presentation– Old >50 years– Fever, fatigue, weight loss– Facial pain, headache along the course of the

temporal artery– Ophthalmic artery: visual disturbance, loss

Giant Cell (Temporal) Arteritis

• Segmental Process– Nodular thickening– Narrowing, thrombosis– Granulomatous inflammation– Affects the inner half of the media layer around

the internal elastic lamina– Lymphocytes, giant cells– Healing by fibrosis, scarring

Giant Cell (Temporal) Arteritis

• Pathogenesis– Not clear– Suspect T-cell immune response– HLA-DR association– Presence of CD4+ T-cells

Takayasu Arteritis

• Granulomatous vasculitis

• Medium-large arteries

• Ocular disturbance

• Weak pulse of upper arms

• Fibrous thickening of the aorta

• Female <40 years

• ? Immune disease

Polyarteritis Nodosa

• Small-medium arteries

• Not arterioles or capillaries

• Renal vessels

• Not pulmonary circulation

Polyarteritis Nodosa

• Segmental transmural necrotizing inflammation

• Kidney, heart, liver, GI, pancreas, testes, muscle, CNS, skin

• Aneurysm, infarction, ischemia• Fibrinous necrosis• Neutrophils, lymphocytes• Fibrous thickening

Polyarteritis Nodosa

• Clinical:– Young adult– Acute, subacute, chronic– Episodic – Fever, malaise, weight loss– Abdominal pain, melena– Muscle pain– neuritis

Kawasaki Disease

• Mucocutanous lymph node syndrome• Acute febrile illness of infancy and childhood• Large-medium-small arteries• Involve coronary arteries• Average age 4 years• Conjunctival, oral, erythema, erosion• Edema of hands and feet• Erythema of palms and soles• Lymph node enlargement

ANCA• Antibodies against neutrophil cytoplasmic

components• Heterogenous group• C-ANCA (cytoplasmic):

– antibodies against porteinase-3, – Seen in Wegener granulomatosis

• P-ANCA (perinuclear): – antibodies against myeloperoxidase, – Seen in Microscopic polyangiitis, and Churg-

Strauss disease

Microscopic Polyangiitis

• Necrotizing vasculitis• Arterioles, capillaries and venules• Skin, mucous membranes, lungs, brain, heart, GI,

kidney, muscle• Necrotizing glomerulonephritis• Pulmonary capillaritis• Hemoptysis, hematuris, proteinuria• Cutaneous purpura• P-ANCA in 80%

Churg-Strauss Syndrome

• Strong association with allergic rhinitis, bronchial asthma, eosinophilia

• Vessels in lung, heart, spleen, nerves, skin

• Granulomatous inflammation and eosinophils

• P-ANCA in 70% of cases

Wegener Granulomatosis

• Triad of:– Acute necrotizing granuloma of URT or LRT– Necrotizing or granulomatous vasculitis of

small-medium vessels– Renal disease: crescentic glomerulonephritis

• C-ANCA in 95% of cases