Vasculitis

Question 1

GCA
a) Classic presentation
b) Diagnostic findings
c) Management
d) If steroids cannot be tapered at 12 months, what is most appropriate management?

Answer 1

a) - Aged over 50 (more common in women)
- Unilateral headache of subacute onset
- Scalp tenderness, jaw claudication, vision loss
- Systemic features - fever, weight loss, depression
- PMR in 40% - morning pain + stiffness in shoulder/pelvis
- Other vasculitic features e.g. mononeuritis, skin rash

O/E
- Scalp tenderness (particularly temporal region)
- Reduced or absent temporal artery pulsation

b) - ESR >50 is significant
- Temporal artery US - ‘halo’ sign
- Temporal artery biopsy: Marked intimal thickening, transmural inflammation, T-cell and histiocyte infiltration, multinucleated giant cells present
(note: biopsy may be normal due to skip lesions)

c) Visual symptoms:
- Urgent ophthalmology review and inpatient admission
- 60-100 mg oral prednisolone or 0.5-1g IV methylprednisolone for 3/7 followed by oral pred

No visual symptoms:
- Urgent rheumatology review, probable outpatient treatment
- 40-60mg oral prednisolone daily

Further management:
- Monitoring of clinical situation, and of steroid SEs (hypertension, hyperglycaemia, etc.)
- PPI and bone prophylaxis often given
- Steroid dose gradually tapered down over 12-18 months

d) Continue steroids, consider addition of methotrexate or IL-6 inhibitor (tocilizumab) and then gradually taper steroids

Question 2

Polyarteritis nodosa
a) Aneurysm appearance
b) Vessel size affected
c) Disease associated
d) Presentation
e) Lab findings
f) Management

Answer 2

a) Rosary bead
Microaneurysms common in angiography

b) Medium sized vessels have aneurysms (classical for PAN)

c) Hep B

d) Skin and neuro commonly
- Livedo reticularis, ulceration
- Mononeuritis multiplex
Fever, myalgia, wt loss, arthralgia, etc.
Renal involvement may cause renal hypertension and AKI but not GN
Does not affect the lungs
Can cause non-atheromatous coronary and gut infarction

e) Neutrophilia
Not ANCA-associated
HBsAg in 30%

f) Steroids
- If Hep B positive, give Interferon

Question 3

GPA vs EGPA vs MPA
a) Clinical features
b) Histology
c) Antibodies
d) Management

Answer 3

GPA
- Upper resp (sinusitis, nasal), lower resp (SOB, haemoptysis), skin (vasculitis) and renal (RPGN). Not usually history of asthma or eosinophils
- Necrotising granulomatous inflammation
- c-ANCA (PR-3) positive in 90%, MPO positive in 10%
- High dose steroids + cyclophosphamide*. PLEX considered if RPGN or pulmonary haemorrhage

*avoid in women of childbearing age as reduces fertility- substitute with MMF

EGPA:
- Lower resp predominant (asthma), upper resp also (sinusitis), renal involvement in 50% (20% GN), skin, mononeuritis, eosinophilia
- Necrotising granulomatous inflammation
- p-ANCA (Anti-MPO) in 40%, ANCA-negative commonly, very rarely anti-PR3
- 80% respond to steroids, 20% need cytotoxic meds

MPA:
- Smaller vessel disease, affecting kidneys causing GN (90%), nerves (mononeuritis), skin (petechiae, purpurae) commonly.
- Less commonly affects the lungs (10%), rarely affects the upper airways/sinuses
- Non-granulomatous
- Anti-MPO common, less commonly anti-PR3

Question 4

Takayasu arteritis
a) Epidemiology
b) Arteries affected commonly
c) Clinical features
d) Management
e) Prognosis

Answer 4

a) - Rare
- Most common in Asian (esp Japanese) women 20-40

b) Large vessels:
- Affects aorta and PA, and major branches commonly
- Also known as ‘pulseless disease’

c) - Causes discrepancy in BP between left and right arms, and between UL + LLs
- Causes ischaemic and claudication symptoms
- Causes weight loss, fever, malaise
- Bruits commonly heard

d) - Steroids + other immunosuppressants
- Statins + aspirin may be used
- Surgery e.g. angioplasty/ revascularisation

e) - 98% survival >10 years with treatment, most have monophasic illness and do not recur when steroids tapered over 2-5 years
- Complications include MI, CVA, heart failure, aortic aneurysm rupture

Question 5

ESR
a) Normal range for men and for women
b) Significant value for GCA

Answer 5

a) Men: age / 2
Women: (age + 10) / 2

i.e.
70 year old man - normal ESR up to 35
70 year old woman: normal ESR up to 40

b) >50 (but may be lower if strong suspicion, or if on steroids/other immunosuppression)

Question 6

Churg-Strauss diagnostic criteria - HAPPEN

Answer 6

4 out of 6:

Histological evidence of extravascular eosinophils
Asthma
Pulmonary infiltrates
Paranasal sinus abnormalities
Eosinophils >10% WCC differential
Neuropathy - poly or mono