Vasculitis

Question 1

Vasculitis

Answer 1

Group of conditions where there is focal inflammation of blood vessels

Question 2

Main type of vessel mostly affected by vasculitis

Answer 2

Arterial system

Question 3

Arteritis

Answer 3

Inflammatory diseases that are primary lesions of arteries

Question 4

Can necrosis occur in severe cases of arteritis ?

If yes what is the consequence of necrosis

Answer 4

Yes

superimposed occlusive thrombosis , Rupture of vessel and aneurysm

Question 5

During healing of arteritis, can permanent stenosis of lumen occur ?

Answer 5

Yes

Question 6

Pheblitis

Answer 6

Inflammation of vein with infiltration of layers of the veins and formation of thrombus

Edema , stiffness , pain, red cord formation

Question 7

Is there etiologic or morphological classification of vasculitis

Answer 7

Not really , diseases overlap too much

Question 8

Pathogenic classification of vasculitis

Answer 8

Direct infection

Immunologic - immune complex mediated

Immunologic - antineutrophil cytoplasmic autoAntibody mediated

Immunologic - direct antibody attack

Immunologic - cell mediated

Immunologic- unknown

Question 9

Direct infection category

Answer 9

Bacterial

Rickettsial

Spirochetal

Fungal

Viral

Question 10

Immune complex mediated classification

Answer 10

Infection

Henoch schonlein purpura

SLE & rheumatoid arthritis

Drug induced

Serum sickness

Cryoglobulinaemia

Question 11

Immunologic - antineutrophil cytoplasmic autoAntibody mediated category

Answer 11

Wegeners granulomatosis

Microscopic polyarteritis nodosa

Churg strauss syndrome

Question 12

direct antibody attack category

Answer 12

Good pasture syndrome

Kawasaki disease

Question 13

cell mediated Category

Answer 13

Allograft organ rejection

Paraneoplastic vasculitis

Question 14

Unknown immunological category

Answer 14

Giant cell

Takayasu

PAN Classic

Question 15

Classification based on vessel size

Answer 15

Large vessel ( giant cell arteritis, takayasu arteritis )

Medium sized vessel ( pan classic , kawasaki, thromboangitis obliterans)

Small vessel ( wegener granulomatosis, churg Strauss, micro PAN, henoch schonlen purpura, essential cryoglobulinaemia, cutaneous leucocytoclastic)

Question 16

2 main common mechanisms

Answer 16

Immune mediated

Direct invasion by microorganisms

Question 17

Can physical agents cause vascular inflammation

Answer 17

Yes

Question 18

Between directly induced infectious vasculitis and infection triggered immune vasculitis which one will respond to immunosuppressive therapy and which one will be worsen

Answer 18

infection triggered immune vasculitis will respond to immunosuppressive therapy

Devastating consequences if immunosuppressive therapy is administered in directly induced infectious vasculitis

Question 19

Immune mediated vasculitis pathogenesis ( Immune complexes)

Answer 19

Immune reactants & complements present in the serum
( In SLE => DNA-antiDNA complex)
( in cryoglobulinaemia => ig-complement complex)

10% from drug hypersensitivity ( drugs ( act like hapten)-serum protein complex)) penicillin or gold

After infectious agents => HBsAg & HBsAg-anti HBsAg complexes accumulate ( seen in pan , membrane-proliferative glomerulonephritis)

Question 20

Immune mediated vasculitis pathogenesis ( antineutrophil cytoplasmic autoantibodies)

Answer 20

Cytoplasmic ANCA

Perimuclear ANCA

Question 21

C-ANCA

Answer 21

Bind Proteinase 3

Associated with wegeners granulomatosis

Question 22

P ANCA

Answer 22

Specific for myeloperoxidase

Found in microscopic polyarteritis and churg strauss syndrome

Question 23

Polyarteritis nodosa

Answer 23

Group of systemic necrotizing vasculitides and usually thrombosis

Widespread distribution of arterial lesions
Medium sized and small muscular arteries
Multiple organ systems
Widespread ischemic tissue injury
Focal random and episodic

Question 24

What vasculature is spared in polyarteritis nodosa

Answer 24

Pulmonary vasculature

Question 25

Polyarteritis nodosa pathology

Answer 25

Lesions ( mostly in kidneys, heart, skeletal muscle, gi, NS) - affect whole circumference of smaller arteries or segment of large artery Acute phase - whole thickness necrotic , neutrophils and eosinophils infiltration, intense inflammation in adventitia Chronic phase- fibrosis , chronic inflammatory cell infiltration, thrombus organisation. Possible formation of micro aneurysm

Question 26

Percentage of patient with polyarteritis nodosa that have HBsAg in serum

Answer 26

30%

Question 27

Clinical features of polyarteritis nodosa

Answer 27

Disease of young Variation depending on organs Severe cases have fever, leucocytosis, high ESR, prostration ``` Symptoms from ischemia in affected organs : Paresthesia with nerves Angina and cardiac failure in heart Stroke from cerebral Acute abdomen , intestinal infarction ``` Severe and rapidly fatal Can be prolonged over years with remission and relapse Death from lesions in kidney, heart , other organs and hypertension

Question 28

Prognosis of polyarteritis nodosa

Answer 28

Poor

Question 29

Treatment of polyarteritis nodosa

Answer 29

Corticosteroids Cytotoxic drugs Treatment of HPT

Question 30

Giant cell arteritis ( temporal or cranial arteritis)

Answer 30

Acute and chronic , often granulomatous, inflammation of large and medium arteries Can be systemic

Question 31

Commonest form of vasculitis

Answer 31

Giant cell arteritis

Question 32

Arteries involved in giant cell arteritis

Answer 32

Carotid Especially temporal artery

Question 33

Cause of giant cell arteritis

Answer 33

Unknown

Question 34

Pathology of giant cell arteritis

Answer 34

Partial destruction of artery wall due to inflammation of whole thickness Infiltration in wall by multinucleate langhans, foreign body giant cells , phagocytosis of elastic lamina Begin in media Often involves long segment of artery with skip areas Fibrous thickening at end stage of intima Scaring of media Thrombotic occlusion of lumen

Question 35

Clinical features of giant cells arteritis

Answer 35

Variable symptoms Could just manifest as weakness , malaise , low grade fever , weight loss ``` More Specific symptoms: Headache Pain radiating into jaws, neck, tongue Facial pain Visual disturbance and blindness Cerebral infarction Intermittent claudication of jaw Localized reddening Tenderness Pain Nodularity of arteries Scalp sensitive to pressure ```

Question 36

Treatment of giant cells arteritis

Answer 36

Steroid therapy