Vasculitis

Question 1

Arteritis involving elastic arteries

Answer 1

• Giant cell arteritis
• Takayasu arteritis

Question 2

Arteritis Involving muscular arteries

Answer 2

• Kawasaki disease
• Polyarteritis nodosa

Question 3

Immune mediated small vessel vasculitis

Answer 3

• Henoch-Schonlein purpura
• Lupus vasculitis
• Rheumatoid vasculitis

Question 4

Pauci immune mediated small vessel vasculitis

Answer 4

• Wegener granulomatosis
• Churg-Strauss angiitis
• Microscopic polyangiitis

Question 5

What are ANCA’s?

Answer 5

• Antineutrophil cytoplasmic antibodies
• Group of antibodies against enzymes in the azurophil or primary granules in neutrophils, in lysosomes of monocytes and in endothelial cells

Question 6

ANCAs as a diagnostic marker

Answer 6

• Levels reflect degree of inflammatory activity
• Etiology may be linked to neutrophils activated by ANCA cause endothelial injury

Question 7

ANCA’s role in pathology

Answer 7

• Can directly activate neutrophils, stimulating release of reactive oxygen species and proteolytic enzymes
• This activation can cause destructive interactions b/w inflammatory cells and endothelial cells

Question 8

ANCA immunofluorescent pattern of staining of neutrophils

Answer 8

• Anti-proteinase 3 (PR3-ANCA formally c-ANCA): cytoplasmic localization of staining target antigen being proteinase 3 (PR3)
• Anti-Myeloperoxidase (MPO-ANCA formally p-ANCA): perinuclear staining specific for myeloperoxidase (MPO)

Question 9

Polyarteritis nodosa organs affected

Answer 9

• Small or medium sized arteries
• Arterioles, capillaries and venules not involved
• Renal and GI involvment common
• Pulmonary vessels spared
• Generally a disease of young adults

Question 10

Polyarteritis nodosa microscopic features

Answer 10

• Segmental transmural necrotizing inflammation
• Fibrinoid necrosis
• Fibrous thickening of vessel wall
• All stages coexist in different vessles or the same vessel

- NO GLOMERULONEPHRITIS

- 30% are positive for Hepatitis B antigen

- NO assoc. w/ c-ANCA

• Elevated ESR, leukocytosis

Question 11

Polyarteritis nodosa slide 1

Answer 11

Question 12

Polyarteritis nodosa slide 2

Answer 12

Question 13

Polyarteritis nodosa slide 3

Answer 13

Question 14

Polyarteritis nodosa slide 4

Answer 14

Question 15

PAN clinical features

Answer 15

• May have acute, subacute or chronic lesions
• Htn, wt loss, fatigue, melana
• Renal artery involvement often a cause of death
• Symptoms are variable
• Rapidly accelerating hypertension, abdominal pain and GI hemmorhage
• Depend on the organs involved
• Kidneys, heart, skeletal muscle, mesentary and skin are most commonly affected
• Fever and weight loss are common
• W/o treatment it is usually fatal
• Anti-inflammatory and immunosuppressive therapy can result in remissions and cures in most patients

Question 16

Microscopic polyangiitis microscopic features

Answer 16

• Histologically similar to PAN
• Granulomatous inflammation absent
• Fragmentation of neutrophils
• Immunosuppression induces remission and improves long term survival

Question 17

Microscopic polyangiitis clinical presentation

Answer 17

• Hemoptysis, arthralgia, abdominal pain, hematuria, proteinuria, bowel hemorrhage, palpable cutaneous purpura
• Muscle pain and weakness
• Causes: immune rxn to drugs, microorganisms and tumor antigens
• MPO-ANCA previously p-ANCA (perinuclear antineutrophil cytoplasmic antibodies): 70% of pts.
• Involved arteries, capillaries and venules
• All lesions tend to be of the same age
• Palpable purpura of the skin, mucous membranes, lungs, brain, heart, GIT, kidney and muscle
• Skin biopsy often diagnostic
• May see glomerulonephritis and pulmonary capillaries