Vasculitis

Question 1

what is vasculitis

Answer 1

inflammation of blood vessels.

may result in vessel wall thickening, stenosis, and occlusion with subsequent ischemia

The clinical presentation varies according to the histologic type of inflammation, the size of the involved blood vessel segment, and the distribution of the involved vessels.

Question 2

what is large vessel vasculitis

Answer 2

causes chronic granulomatous inflammation predominantly of the aorta and its major branches.

Question 3

The two categories of large vessel vasculitis

Answer 3

temporal (giant cell) arteritis

Takayasu arteritis (TA).

Question 4

giant cell arteritis (3)

Answer 4

swedish

OVER 50 YEARS OLD WOMEN

typically affects the temporal arteries but may also (or only) affect the large vessels.

Question 5

Takayasu arteritis (3)

Answer 5

asian

young women under 50 year old

affects major arteries e.g. aorta

Question 6

symptoms giant and TA (7)

Answer 6

low-grade fever

malaise

night sweats

weight loss

arthralgia

fatigue

claudicant symptoms in both the upper and lower limbs.

Question 7

If untreated- giant and TA (2)

Answer 7

vascular stenosis and aneursyms can occur

results in reduced pulses and bruits.

Question 8

investigations giant and TA

Answer 8

ESR, PV and CRP and elevated.

Imaging such as MR angiography can detect thickened vessel walls and stenosis.

Question 9

treatment giant and TA (2)

Answer 9

corticosteroids, starting at 40-60mg prednisolone and gradually reducing.

Steroid sparing agents such as methotrexate and azathioprine may be added.

Question 10

Small to medium vessel vasculitis

Answer 10

This group can be divided into ANCA positive and negative conditions.

Question 11

ANCA + conditions

Answer 11

Granulomatosis with polyangiitis (GPA/ wegners)

Microscopic polyangiitis (MPA)

Renal-limited vasculitis (RLV)

Churg Strauss Syndrome (CSS)

Question 12

(GPA) or Wegeners symptoms

Answer 12

ENT symptoms are common in GPA and include nose bleeds, deafness, recurrent sinusitis and nasal crusting (over time there can be collapse of the nose).

Respiratory symptoms such as haemoptysis and cavitating lesions on x-ray are frequently seen.

Nasal cartilege collapse

Question 13

GPA/ wegeners investigations

Answer 13

CXR in GPA (Wegener’s) showing cavitating opacities

GPA is associated with cANCA and PR3 (proteinase-3)

Question 14

Eosinophilic granulomatosis with polyangiitis (EGPA)

AKA Churg strauss syndrome

Answer 14

this condition is characterised by late onset asthma, rhinitis and a raised peripheral blood eosinophil count.

Neurological symptoms such as mono neuritis multiplex are common.

Question 15

ANCA - conditions