Vasculitis, Systemic vasculitis in children 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 blood vessels of different sizes and the organs that they are supposed to supply. Vasculitis in children is also distinguished on the basis of various symptoms and typical changes in blood samples or tissue samples (biopsy). The most common type in children is Henoch-Schönleins purpura (IgA vasculitis).

Symptoms of vasculitis

The child appears with fatigue (tiredness), night sweats and a slight fever are common. Symptoms otherwise depend on the diagnosis and which blood vessels and organs are attacked. Rash, joint and muscle pain may occur.

Medical examinations

Medical history charts current symptoms (see above), when they began and how the development has been. Pain over large blood vessels in the neck (Takayasu arteritis), in the abdominal area or over skin areas with reduced blood circulation may be typical. With vasculitis of the lungs, one may cough up some blood (hemoptysis).

Clinical Signs of decreased blood circulation in some cases can be seen on the hands and feet. If small blood vessels to the skin are attacked, a rash with marked small red dots often occurs (petechiae). Vasculitis in larger blood vessels can lead to reduced blood circulation in larger areas. Fever occurs. Blood pressure on both arms, assessment of heart, lungs, abdomen, nose, eyes, hearing, skin and joints are relevant.

Blood tests shows signs of inflammation with high CRP and blood lowering reaction (SR), but signs of no infection. ANCA vasculitis is rare but elevated PR3-ANCA or MPO-ANCA (Antibodies) is detected.

Urine. If kidneys are affected, urine sample will show egg whites (proteins) and traces of blood (hematuria).

Imaging. CT images can show lung opacities. Sometimes tissue samples from small blood vessels are useful for diagnosis.

Tissue samples (biopsy) may be necessary to make the correct diagnosis. The sample is taken from the attacked organ, provided that the risk of complications is not too great. Skin and kidneys are well suited for tissue testing when needed.


To prevent severe tissue damage, potent immunosuppressive drugs are usually needed for an extended period of time. These may include cortisone preparations such as prednisolone, DMARDs og Biological drugs. In general, one will first reduce the disease activity (induction treatment) and then keep the disease in check with milder maintenance treatment. In general, Hennoch-Schönlein purpura often has a relatively mild course among vasculitis diseases, so that one is restrained with powerful drugs. Regardless, the treatment is adapted to each individual case.

Some vasculitis diseases in children

Behcet's disease

Behcet's disease typically have recurrent ulcers (ulcers) in the mouth and abdomen, pimples (acne) -like skin changes, some get blood clots or eye inflammation. No special tests turn out, but CRP is often somewhat elevated during bad periods.

Granulomatosis with polyangiitis (GPA)

Granulomatosis with polyangiitis (GPA) (Wegener's Granulomatosis) may manifest as persistent tightness of the sinuses and / or ears, nosebleeds, red, inflamed eyes, joint pain, nephritis and some severe lung hemorrhage. Blood test with rash in ANCA and the subgroup PR3- ANCA.

Henoch-Schönleins purpura (IgA vasculitis)

Henoch-Schönleins purpura (IgA vasculitis). Some characteristics are small-spotted rash (petechiae), especially on the legs, abdominal pain, arthritis (arthritis) and nephritis (urine with proteins and blood). No special blood tests. Tissue sample (biopsy) shows evidence of IgA in skin or kidneys.

Kawasaki syndrome

Kawasaki syndrome (mucocutaneous lymph node syndrome) is a serious condition in which the child is obviously involved. Fever for at least 5 days, red, inflamed eyes, tender, dry chapped lips, red dots (purpura), mostly on legs and arms, red tongue (strawberry tongue) and swollen lymph nodes, mostly on the neck are hallmarks.

Microscopic Polyangiitis (MPA)

Microscopic Polyangiitis (MPA) most often have nephritis, and some show skin changes, lung changes, joint and / or muscle pain and eye inflammation. Blood tests with rash in ANCA and MPO subgroup.

Polyarteritis nodosa (PAN) in children and DADA

Polyarteritis nodosa (PAN) in children and DADA2 hscar fever over time, muscle aches, cold, discolored fingers, marble-like pattern in skin, nodules under the skin. A serious complication is a stroke (blood clot) at a young age, even among children. A gene defect (2014) can be detected as a cause of illness in children (DADA2)

Takayasu arteritis

Takayasu arteritis often causes night sweats, a slight fever, inflammation, pain in the neck and / or chest, pain in one arm during activity, especially hovering horizontal level (claudication). Blood pressure difference between right and left arm. Reduced radial pulse.

Similar Conditions / Differential Diagnoses

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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