Vasculitis

Question 1

Henoch - Schonlein purpura criteria

Answer 1

Need palpable purpura and one or more of:

• renal inv
• arthritis
• GI pain
• any bx demonstrating IgA deposition perivascularly

Question 2

Causes of HSP

Answer 2

Preceding respiratory infection - 20-50% have positive ASOT, no Causal role for Group B haemolytic strep

Question 3

Natural hx of HSP

Answer 3

Usually self limited disease taking weeks to months to resolve
Need to monitor urine red cell casts and protein for 6 months post episode
In adults, monitor for solid organ malignancy lung

Question 4

Acute haemorrhagic oedema of infancy - causes

Answer 4

75% have associated infection, drug exposure of immunisation
2/3 have viral prodrome (URTI) 80%, diarrhoea illness 12%, UTI 6%

Question 5

AHOOI presentation

Answer 5

Usually occurs in children 4-24 mo of age
Cutaneous lesions begin as plaques with variable degree of haemorrhage
Favours head and extremities
Subsequently become oedematous and targetoid in appearance

Question 6

T/F infants with AHOOI are unwell-appearing

Answer 6

F appear well

Question 7

T/F infants with AHOOI develop tender non pitting oedema of the face, nears, and extremities including neck and feet

Answer 7

T

Question 8

List triggers of AHOOI

Answer 8

Infections:

• adenovirus
• coxsackievirus
• CMV
• EBV
• HSV
• Hep A
• measles
• rotavirus
• VZV
• campylobacter
• E. coli

Medications:

• paracetamol
• penicillins
• cephalosporins
• triemethoprim - sulfamethoxazole
• NSAIDs

Question 9

Natural hx of AHOOI

Answer 9

Benign with spontaneous and complete resolution without sequelae in 1-3 weeks

Question 10

ASSOCIATIONS OF URTICARIAL VASCULITIS

Answer 10

MICE
Medications - NSAIDs, MTX, cimetidine
Infection
CT disorders - lupus, sjogren, cryoglobulinaemia
Eek cancer - haem malignancies> solid organ malignancies (colon/renal_)

Question 11

Criteria for hypocomplementaemic urticarial vasculitis

Answer 11

6 mo hx vasculitis

C1q (positive C1q precipitin test with low C1q level) 
Glomerulonephritis 
UVEITIS 
Arthralgias
Vasculitis on skin biopsy 
Abdo cramping

Question 12

What is the most common extracutaneous feature of hypocomplementaemic urticarial vasculitis

Answer 12

MSK involvement is the most common - arthralgias of hands, elbows and knees
20% have pulmonary symptoms
30% have GI manifestations

Question 13

Erythema elevatum diutinum is associated with which monoclonal gammopathy

Answer 13

IgA paraproteinaemia

Question 14

Associations of erythema elevatum diutinum

Answer 14

SRR Wegener has IBD, HIV and coeliac 
- Sjogren 
- relapsing polychondritis 
- RA
- wegener’s granulomatosis 
-IBD
- hiv
- coeliac disease
-

Question 15

Prognosis of EED

Answer 15

Prognosis of EED
- chronic relapsing/remitting course
- most spontaneously resolve over 5-10 y but disease can last up to 40 y
`

Question 16

Microscopic polyangiitis extracutaneous features

Answer 16

PRN

• pulmonary - haemorrhage, capillaritis, asthma
• renal - glomerulonephritis
• neuro - mononeuritis multiplex - painful sensory and motor peripheral neuropathy

Question 17

Granulomatosis with polyangiitis extracutaneous manifestations

Answer 17

Upper respiratory - nasal ulcers, saddles nose, otitis , chronic sinusitis, mastoiditis
Pulmonary - nodules, fixed infiltrates
Renal - glomerulonephritis
Ocular - proptosis, scleritis

Less often neuro, GI, cardiac

Question 18

Extracutaneous manifestations of EGPA

Answer 18

CR PHUN

• cardiac
• renal
• pulmonary
• haem - IgE, eosinophilia
• Upper resp
• neuro

Question 19

Polyarteritis nodosa associations/causes

Answer 19

Infections - Strep esp in children, parvoB19, HIV, Hep B in 7% of pt with classic PAN
- HBV could represent a clinical subtypes with higher GI, neurological and renal involvement

• med: minocycline
• inflammatory conditions: IBD, SLE, familial Mediterranean fever
• malignancy - hairy cell leukaemia

Question 20

Describe features of systemic PAN

Answer 20

Livedo reticularis
Ulcers “punched out”
Palpable purpura - may be pustular or ulcerative
Inflammatory retiform purpura
MULTIORGAN INVOLVEMENT - except lungs usually spared
- MSK : arthralgias , yalgias
- NEURO: paraesthesia, mononeuritis multiplex, cerebral infarcts rare in classic PAN
- GI: abdo pain, mesenteric ischaemia confers a poor prognosis with 1 y survival <50%
- CVS - dyspnoea due to CCF
- renal: kidney involvement occurs in the interlobular arteries NOT A GLOMERULONEPHRITIS . Need MRA kidney show microaneurysm of renal/mesenteric or coeliac arteries highly suggestive of classic PAN