Abstract
Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis.
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Answers:
1. d
2. b
3. c
4. a
5. c
Multiple-choice questions: (Answers appear following the reference list)
Multiple-choice questions: (Answers appear following the reference list)
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1.
Which of the following is true in relation to childhood PAN?
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a.
Crescentic glomerulonephritis is a well-recognised complication
-
b.
There is often a discrepancy between the ESR and the CRP results
-
c.
Cytoplasmic ANCA is usually detectable
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d.
Livido reticularis is a characteristic feature
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2.
In childhood PAN, which of the following statements is correct?
-
a.
Most cases have an infective aetiology
-
b.
Renal and hepatic angiography is a valuable investigative tool
-
c.
The aorta is frequently affected
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d.
Cyclophosphamide is contraindicated because of side effects
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3.
In relation to cutaneous childhood PAN, which of the following is true?
-
a.
Pyoderma gangrenosum is a severe manifestation
-
b.
Leukocytoclastic vasculitis is a characteristic histological finding
-
c.
The condition rarely progresses to systemic PAN
-
d.
It is the commonest cause of vasculitis involving the skin in children
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4.
Regarding KD, which of the following is correct?
-
a.
IV-Ig can be effective if administered later than 10 days after disease onset
-
b.
Only coronary arteries are affected
-
c.
Acute glomerulonephritis is the usual renal manifestation
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d.
The disease is unlikely unless all criteria are met
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5.
For KD, which of the following is false?
-
a.
There is evidence for genetically determined susceptibility to the disease
-
b.
Long-term endothelial dysfunction has been demonstrated after the acute phase
-
c.
Female patients with cardiac sequelae have a higher mortality rate than male patients
-
d.
There are data supporting an aetiological role for superantigens
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Dillon, M.J., Eleftheriou, D. & Brogan, P.A. Medium-size-vessel vasculitis. Pediatr Nephrol 25, 1641–1652 (2010). https://doi.org/10.1007/s00467-009-1336-1
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DOI: https://doi.org/10.1007/s00467-009-1336-1