Small vessel Vasculitis ***

Question 1

Classification used?

Answer 1

ANCA-associated vasculitis

Immune complex associated vasculitis

Question 2

ANCA associated vasculitis:

3 types?

Answer 2

ANCA associated vasculitis:

(1) GPA (Granulomatosis with polyangiitis) (WEGENER’s)
(2) Churg Strauss Syndrome (Eosinophilic granulomatosis with polyangiitis (EGPA)
(3) Microscopic Polyangiitis (MPA)

Question 3

Immune Complex associated:

2 types?

Answer 3

Henoch-Schonlein purpura (HSP) - IgA vasculitis

Anti-GBM - Goodpasture’s Syndrome

Question 4

GPA (Granulomatosis with polyangiitis) (WEGENER’s)

Systemic (organ threatening) or Localised (Non-organ threatening)

Features?

What is one distinguishing feature of a small vessel?

Also leads to progressive glomerulonephritis (GN) (either crescentic/sclerosing).

Localised - What can happen to their nose?

Answer 4

Alveolar haemorrhage*** - haemoptysis

Glomerulonephritis
Scleritis
Mononeuritis multiplex
Systemic symptoms

Saddle nose deformity

Question 5

Microscopic Polyangiitis (MPA)

pANCA**

Symptoms same

Answer 5

Microscopic Polyangiitis (MPA)

pANCA**

Symptoms same

Question 6

Constitutional symptoms (anorexia / weight loss & fever myalgia and arthralgia)

RENAL - glomerulonephritis

SKIN - maculopapular purpura - lower limb

NEUROPATHY - mononeuritis multiplex

PULMONARY - haemorrhage, infiltrates & effusions

GI tract - abdominal pain, bleeding, ischaemia, ulceration

Answer 6

Constitutional symptoms (anorexia / weight loss & fever myalgia and arthralgia)

RENAL - glomerulonephritis

SKIN - maculopapular purpura - lower limb

NEUROPATHY - mononeuritis multiplex

PULMONARY - haemorrhage, infiltrates & effusions

GI tract - abdominal pain, bleeding, ischaemia, ulceration

Question 7

Churg Strauss Syndrome (Eosinophilic granulomatosis with polyangiitis (EGPA):

Long prodromal period

What may a patient develop later in life which should trigger investigations for vasculitis?

What is seen on biopsy?

Rest of symptoms the same

Answer 7

Late-onset Asthma - atypical asthma symptoms

Eosinophils/granulomas

Question 8

Henoch-Schonlein purpura (HSP) - IgA vasculitis:

What antibody is negative in this?

Typical trigger?

Where does the purpuric rash tend to occur?

What is needed for diagnosis?

What is the treatment?

Answer 8

ANCA negative - so ANCA a good way of differentiating the different types

Streptococcal infection

Rash - extensor surfaces

Biopsy - renal + dermis

SELF-limiting (analgesia only)