Small to Medium Vessel Vasculitis

Question 1

what groups can these be divided into

Answer 1

ANCA positive and ANCA negative conditions

Question 2

what are similar presenting features of both groups of ANCA positive and negative

Answer 2

• fever
• weight loss
• raised, non-blanching purpuric rash
• arthralgia/arthritis
• mononeuritis multiplex
• glomerulonephritis
• lung opacities on x-ray

Question 3

what are the 4 vasculitis’ associated with ANCA

Answer 3

granulomatosis with polyangiitis (wegners), microscopic polyangiitis (MPA), renal limited vasculitis (RLV), Churg-Strauss syndrome (CSS)

Question 4

what symptoms are common with granulomatosis with polyangiitis (GPA or wegners)

Answer 4

ENT symptoms
-nose bleeds
-deafness
-recurrent sinusitis 
-nasal crusting (over time collapse of the nose)
Respiratory systems
-haemoptysis 
-cavitating lesions on X-ray

Question 5

what genetic marker is GPA associated with

Answer 5

cANCA and PR3 (proteinase-3)

Question 6

what is eosinophilic granulomatosis with polyangiitis (EGPA) characterised by

Answer 6

• late onset asthma
• rhinitis
• raised peripheral blood eosinophilic
• neurological symptoms such as mono neuritis multiplex are common

Question 7

what is the most common complication of microscopic polyangiitis

Answer 7

glomerulonephritis (occurs up to 90%)

Question 8

what are investigations

Answer 8

• raised ESR, PV and CRP
• anaemia of chronic disease is common
• U+E for renal involvement
• ANCA
• urinalysis (renal vasculitis)
• CXR
• biopsy of affected area

Question 9

what is the management

Answer 9

ANCA associated=IV steroids and cyclophosphamide due to aggressive course
some situations would call for other treatments e.g GPA localised to nose

Question 10

what is Henoch-Schonlein Purpura

Answer 10

acute immunoglobulin A (IgA) mediated disorder characterised by a general vasculitis involving small vessels of the skin, gi tract, kidneys, joints and rarely lungs and CNS

Question 11

who does Henoch-Schonlein Purpura commonly affect

Answer 11

children

Question 12

what is the common history of Henoch-Schonlein Purpura

Answer 12

URTI predates the symptoms by a few weeks

Question 13

what are common symptoms of Henoch-Schonlein Purpura

Answer 13

purpuric rash over buttocks and lower limbs, abdominal pain and vomiting and joint pain

Question 14

what is the treatment for Henoch-Schonlein Purpura

Answer 14

self limiting
doesn’t need specific treatment
usually settles over course of weeks to months