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Small vessel vasculitis

Pediatric Nephrology volume 25, pages 1025–1035 (2010)Cite this article

Abstract

The pediatric small vessel vasculitides reviewed in this article are Henoch–Schönlein purpura (HSP) and the anti-neutrophil cytoplasmic antibody-associated vasculitides (AAV). The new classification criteria for HSP and Wegener’s granulomatosis are now validated and will facilitate the conduct of future epidemiological studies and clinical trials. The clinical manifestations of small vessel vasculitis in children are described, and current therapies discussed. There is a lack of good clinical trial data on which to base therapy for HSP. Similarly, data based on randomized controlled trials (RCTs) for pediatric AAV are lacking, although children with AAV are for the first time now included in a RCT of mycophenolate mofetil versus cyclophosphamide. Significant challenges remain in the field of pediatric small vessel vasculitis, including the development of validated disease outcome measures and biomarkers to be used in clinical trials. Lastly, long-term outcome data are lacking in survivors of pediatric small vessel vasculitis.

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Author information

Authors and Affiliations

  1. Department of Rheumatology, Institute of Child Health, 30 Guilford St, London, WC1N1EH, UK

    Paul Brogan & Despina Eleftheriou

  2. Department of Nephro-urology, Institute of Child Health, 30 Guilford St, London, WC1N1EH, UK

    Michael Dillon

Authors
  1. Paul Brogan
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  2. Despina Eleftheriou
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  3. Michael Dillon
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Corresponding author

Correspondence to Paul Brogan.

Questions:

Questions:

(answers appear at the end of the list of questions)

  1. 1.

    Regarding HSP, which of the following statements is TRUE?

  1. a.

    HSP never recurs in renal allografts.

  2. b.

    IgA is always found on immunostaining of biopsies of lesional tissue.

  3. c.

    HSP rarely occurs after infections.

  4. d.

    IgA may be elevated in sera.

  1. 2.

    Regarding Wegener’s granulomatosis in children, which of the following is TRUE?

  1. a.

    The diagnosis can be excluded in children if ANCA are negative.

  2. b.

    Serum ANCA levels correlate well with disease activity.

  3. c.

    Despite lack of RCT evidence, there is still a role for plasma exchange in those with severe disease.

  4. d.

    New therapies such as rituximab have replaced standard toxic therapies such as cyclophosphamide.

  1. 3.

    Regarding ANCA associated vasculitides, which of the following is TRUE?

  1. a.

    Plasma exchange has proven efficacy for the treatment of WG in adults.

  2. b.

    CSS in children only occurs as a result of therapy with leukotriene inhibitors.

  3. c.

    The majority of children with CSS are ANCA positive.

  4. d.

    Etanercept has proven efficacy for the treatment of WG in adults.

  1. 4.

    Regarding ANCA associated vasculitides, which of the following statements is FALSE?

  1. a.

    Wegener’s granulomatosis never affects the aorta.

  2. b.

    Intravenous cyclophosphamide is preferable to oral cyclophosphamide because it has a lower toxicity profile.

  3. c.

    Intravenous cyclophosphamide can be given to patients with MESNA hypersensitivity.

  4. d.

    Septrin (co-trimoxazole) may reduce relapses for patients with WG and respiratory tract involvement.

  1. 5.

    Regarding the following clinical trials of small vessel vasculitis, which of the following statements is TRUE?

  1. a.

    Trials of novel therapies for small vessel vasculitis should always be conducted in adults before children.

  2. b.

    Lack of good RCT data precludes the use of rituximab in the treatment of pediatric AAV.

  3. c.

    RCT data suggest that early use of corticosteroids helps prevent the onset of HSP nephritis.

  4. d.

    The Birmingham vasculitis activity score can be helpfully used in RCTs of therapy for small vessel vasculitis.

Answers:

  1. 1.

    d

  2. 2.

    c

  3. 3.

    a

  4. 4.

    a

  5. 5.

    d

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Brogan, P., Eleftheriou, D. & Dillon, M. Small vessel vasculitis. Pediatr Nephrol 25, 1025–1035 (2010). https://doi.org/10.1007/s00467-009-1317-4

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