Vasculitis

Question 1

Classification of Vasculitis

Answer 1

a. Infectious (bacterial or fungi invading a vessel wall)b. Immunological (autoimmune)c. Immune complex mediatedd. Direct Ab attack (Goodpasture’s, Kawasaki)e. ANCA associated (e.g. Wegeners)f. Cell mediated (organ graft rejection)g. Unknown (GCA, Takayasu’s)

Question 2

Pathophysiology of vasculitis

Answer 2

infiltrate of neutrophils Fibrin depositionLeakage of RBCsLymphocytes and macrophages attractedFibrosis of vessel wall May progress direct to giant cell inflammation in giant cell vasculitis

Question 3

What are the most common small vessel vasculitides

Answer 3

Miscropic PolyangiitisWegener’s GranulomatosisChurg-Straus SyndromeHenoch Schonlein PurpuraCryoglobulinaemic VasculitisSerum SicknessRheumatoid and Lupus Vasculitis

Question 4

What are the manifestations of small vessel vasculitis?

Answer 4

a. Nephritis: usually necrotising and crescentic GNb. Pulmonary haemorrhage: haemorrhagic alveolar capillaritis i.e. bleeding in lungs), heals as interstitial fibrosisc. Pulmonary-Renal syndrome: microscopic polyangitis and Good pasture’s syndrome (preferential damage to kidneys and lungs) d. Purpura e. Abdo pain: bowel, liver haemorrahgef. Peripheral neuropathy, myalgia, arthralagia (invovlement of small arteries and aretioles)Usual treatment: withdrawl of inciting agent if knownHigh dose corticosteroids and cyclophosphamide

Question 5

What are the manifestations of small vessel vasculitis?

Answer 5

a. Nephritis: usually necrotising and crescentic GNb. Pulmonary haemorrhage: haemorrhagic alveolar capillaritis i.e. bleeding in lungs), heals as interstitial fibrosisc. Pulmonary-Renal syndrome: microscopic polyangitis and Good pasture’s syndrome (preferential damage to kidneys and lungs) d. Purpura e. Abdo pain: bowel, liver haemorrahgef. Peripheral neuropathy, myalgia, arthralagia (invovlement of small arteries and aretioles)Usual treatment: withdrawl of inciting agent if knownHigh dose corticosteroids and cyclophosphamide

Question 6

Pacui-immune vascultiis: Micrscopic polyangiitis

Answer 6

= necrotising systemic vasculitisAffects arterioles, capillaries and venulesCan sometimes involve small and medium size arteriesNo immune deposits! ANCA negative?Affect skip wise vasculitis (caution on biopsy) i.e. segmental vascular necrosis

Question 7

Importance of ANCA monitoring?

Answer 7

Response to Rx (i.e. marker for vasculitis)

Question 8

Pacui immune - Wegener’s Granulomatosis

Answer 8

= granulomatous inflammation, necrosis, and vasculitis predominantly affecting respiratory tract (triad of features of Wegener’s) Predominantly C-ANCA positive (75%)

Question 9

Pacui immune - Churg Strauss Syndrome

Answer 9

Granulomatous inflammationPeripheral blood eiosniphilasstham

Question 10

List the three pacui immune types

Answer 10

d

Question 11

IC vasculitis types

Answer 11

a. Rheumatoid and lupus vasculitisb. Cryoglobulinaemic vasculitisc. Henoch schonleign purpura

Question 12

Medium sized arteries vascultiies types

Answer 12

a. Polyarteritis nodosa

Question 13

Larger arteries vasculitis

Answer 13

Giant cell arteritis

Question 14

Dx of vasculitis

Answer 14

a. Clinical Features (determine general vasculitis)Then must determine specific type of vasculitis: b. Serology: ANCA, cryoglobulins, HBV, HCV, ANA’s and RFc. Pathology: get biopsy if possible