Vasculitis, Systemic vasculitis in children (an overview) 4/5 (2)

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Vasculitis in children with Kawasaki disease. Kim DS - Yonsei Med. J. (2006). CC BY-NC 3.0


Vasculitis is Rheumatic (autoimmune) inflammation (not infection) in blood vessels. There are several types of vasculitis among children. The different types of vasculitis diseases of can damage different blood vessels and organs. They are also distinguished due to various symptoms and typical changes in blood samples or tissue samples (biopsy).


Disease sensation with fatigue (tiredness)night sweats and some fever are common. Symptoms otherwise depend on which blood vessels and organs are attacked.

Medical examinations

Fever and blood tests show signs of inflammation (CRP and sedimentation rate, ESR), but no infection. If small blood vessels are attacked on the skin, a rash with marked small red spots often occurs (petechiae). Vasculitis in major blood vessels causes signs of decreased blood circulation in larger areas. If kidneys are affected, urine test will show egg white (proteins) and traces of blood. In the case of vasculitis in the lungs, a cough can produce blood stained expectorate (hemoptysis) and CT images show lung opacities. Some vasculitis diseases have abnormality in special blood samples (Antibodies) that are typical for the specific diagnosis. Sometimes tissue samples from small blood vessels useful for diagnosis.

Some vascular diseases in children:

Henoch-Schönleins purpura (IgA vasculitis)

Keywords are:

  • Small-spotted rash (petechiae), especially on the legs
  • Abdominal pain
  • Renal inflammation (protein and blood urine)
  • Arthritis (arthritis)
  • Tissue sample (biopsy) shows a catchment of IgA in skin

Behcet's disease

Granulomatosis with polyangiitis (GPA) (Wegener's Granulomatosis)

Keywords are

Microscopic Polyangiitis (MPA)

Keywords are

  • Skin abnormalities
  • Nephritis
  • Pulmonary abnormalities
  • Joint and / or muscle pain
  • Eye Inflammation
  • Blood samples with abnormal ANCA and MPO subgroup

Kawasaki syndrome (mucocutaneous lymph node syndrome)

Keywords are

  • Fever for at least 5 days
  • Red, inflammed eyes
  • Sore, dry cracked lips
  • Red dots (purpura), mostly on legs and arms
  • Red tongue (strawberry tongue)
  • Svolen lymph nodes, mostly on the neck

Polyarteritis nodosa (PAN) in children and DADA2

Keywords are

  • Protracted fever
  • Muscle pain
  • Cold, discolored fingers
  • Marble-like pattern in skin
  • Knots beneath the skin
  • Stroke
  • Recently a gene defect (2014) has been found as a cause of the disease in children (DADA2)

Takayasu arteritis


  • Night sweats, fever, inflammation (CRP and sedimentation rate, ESR) without effect of antibiotics
  • Neck pain (carotid artery, carotid artery)
  • Chest pain (vasculodynia)
  • Claudication
  • Blood pressure difference between right and left arm
  • Reduced radial pulse

Differential diagnosis

It is always difficult to diagnose early in the course of the disease. Infections, other systemic rheumatic diseases, autoinflammatory fever syndromes and cancer disease must also be considered as possible alternatives with similar symptoms.


Children with rheumatic disease,

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