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The “other” vasculitis syndromes and kidney involvement

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Abstract

There are a number of vasculitides that are not confined to a specific vessel size, do not have characteristic features, and/or are not secondary to another disease. Most of these vasculitides are rare in childhood. Behçet disease is representative of this group as it involves vessels of any size on both the arterial and venous side. In addition to renal vascular involvement, Behçet disease may involve the kidney through glomerulonephritis, secondary amyloidosis and, rarely, tubulointerstital involvement. Vasculitis secondary to infections, malignancy, and drugs are not common among children. However, vasculitis may be associated with a number of rheumatic diseases in childhood and the auto-inflammatory syndromes (periodic fever syndromes). Auto-inflammatory syndromes are diseases characterized by periodic attacks of clinical and laboratory inflammation. Studies carried out during the past decade have provided valuable information on the mechanism of inflammation and innate immunity in general. This group of vasculitides is associated with secondary amyloidosis of the kidney if not treated. Hypocomplementemic urticarial vasculitis is an interesting vasculitic disease with frequent kidney involvement. Here, we introduce the reader to the wide scope of these diseases; although rare, such diseases represent a challenge to the nephrologist.

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Authors and Affiliations

  1. Pediatrics, Hacettepe University Faculty of Medicine, Ankara, 06100, Turkey

    Seza Ozen

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  1. Seza Ozen
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Correspondence to Seza Ozen.

Additional information

Answers

1. d

2. e

3. a

4. c

5. e

Questions

Questions

(Answers appear following the reference list)

  1. 1.

    Which is true for Behçet disease?

    1. a.

      It is never seen in children

    2. b.

      It is a short lasting mucositis

    3. c.

      Only mid-size arteries are affected

    4. d.

      Both the veins and arteries may be affected

    5. e.

      CNS is not involved

  2. 2.

    What kind of kidney involvement is seen in Behçet disease?

    1. a.

      Glomerulopathies

    2. b.

      Secondary amyloidosis

    3. c.

      Tubulointerstitial disease

    4. d.

      Renal artery aneurysm

    5. e.

      All of the above

  3. 3.

    Autoinflammatory diseases lack autoantibodies. Is this statement:

    1. a.

      True

    2. b.

      False

  4. 4.

    In FMF patients who are not sufficiently treated what kind of kidney disease develops?

    1. a.

      Tubulointerstitial nephritis due to colchicine

    2. b.

      Microscopic polyarteritis/polyangiitis

    3. c.

      Secondary amyloidosis

    4. d.

      An immune complex nephritis

    5. e.

      IgA nephropathy

  5. 5.

    In hypocomplementemic urticarial vasculitis syndrome which is false?

    1. a.

      There is hypocomplementemia

    2. b.

      There is persistent urticaria

    3. c.

      Anti C1q antibodies are detected

    4. d.

      Half develop glomerulonephritis

    5. e.

      Patients have cryoglobulins

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Ozen, S. The “other” vasculitis syndromes and kidney involvement. Pediatr Nephrol 25, 1633–1639 (2010). https://doi.org/10.1007/s00467-009-1327-2

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