Vasculitis in Children

Overview

What is vasculitis?

Vasculitis is the inflammation (swelling) of the body’s blood vessels. The condition occurs if your immune system attacks your blood vessels by mistake. Vasculitis can affect very small blood vessels (capillaries), medium-size blood vessels, or large blood vessels such as the aorta (the main blood vessel that leaves the heart).

What happens to the blood vessel in vasculitis?

When the blood vessel is inflamed, it may become weakened and result in structural disruption. Blood flow is impaired and blood clots may form in the inflamed vessels. Together with the swelling of the vascular walls, this effect may contribute to vessel narrowing or occlusion (blockage). Rarely, the vessels also may become thin and stretch in size, which can lead to aneurysms and rupture. This can result in bleeding into the tissue. The vessel wall itself becomes more "leaky," allowing the fluid from within the blood vessels to enter the surrounding tissues and causing swelling. These effects are both responsible for the various types of rashes and skin changes seen in this group of diseases. Involvement of the vessels supplying vital organs like the brain, kidneys, lungs or heart can compromise organ function and be a very serious condition.

What are the types of vasculitis? How is vasculitis classified?

Vasculitis classification in children is based on the size of the blood vessel involved.

  • Large vessel vasculitis, affects the aorta and its major branches.
  • Medium vessel vasculitis, affects arteries supplying the kidneys, bowels, brain or heart
  • Small vessel vasculitis, affects smaller blood vessels including capillaries in various organs

Some types of vasculitis diseases are quite common in children (e.g. Henoch-Schönlein purpura and Kawasaki disease), while the others are rare and their exact frequency is unknown.

Symptoms and Causes

What are the main symptoms of vasculitis?

Disease symptoms vary according to the overall number of inflamed blood vessels (widespread or just a few sites) and their location (vital organs like brain or heart versus skin or muscle) as well as the degree of blood supply compromise. This can vary from a transient minor decrease of blood flow to complete occlusion with subsequent changes to the unsupplied tissue caused by the lack of oxygen and nutrient supply. This can eventually lead to tissue damage with subsequent scarring.

Symptoms based on blood supply of organ involvement can include:

  • Brain : Alteration of consciousness, severe headache, stroke-like symptoms (paralysis)
  • Eyes: Blurred vision, seeing floaters, sensitive to light
  • Skin: Rash, chronic ulcer, bruise
  • Bowel: Abdominal pain, diarrhea, blood in stool
  • Lungs: Shortness of breath, coughing up blood
  • Kidneys: Kidney failure, decrease amount of urine, blood and protein leakage from kidney

Diagnosis and Tests

How is vasculitis diagnosed?

Diagnosis of vasculitis can be challenging. The symptoms resemble other various, more common pediatric illnesses. The diagnosis is based on an expert evaluation of clinical symptoms, together with the results of blood and urine tests and imaging studies (ultrasonography, X-rays, CT and MRI scans, angiography). Where appropriate, diagnosis is confirmed by biopsies (samples) taken from the involved and most accessible tissues or organs.

Management and Treatment

How is vasculitis treated?

The treatment for chronic vasculitis is long-term and complex. The main goal is to stop inflammation (swelling) and get the disease under control as soon as possible (induction therapy), and to maintain control over a long period of time (maintenance therapy), while avoiding drug side effects where possible.

Treatments are chosen strictly on an individual basis according to the patient´s age and the disease severity.

Various treatments can be used to suppress the activated immune system and fight inflammation. Medications can be used individually or in combination of corticosteroids, cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil, rituximab and anti-TNF agents.

Outlook / Prognosis

What is the long-term prognosis (outlook) for vasculitis?

Prognosis of vasculitis is highly individual. It depends not only on the type and extent of vessel involvement and the organ involved, but also on the interval between disease onset and the start of treatment. The individual response to treatment can also be a factor. The risk of organ damage is related to how long the disease is active. Damage to the vital organs can have life-long consequences.

With proper treatment, clinical remission is often achieved within the first six to 12 months. The remission can be life-long, but long-term maintenance therapy is often needed. Periods of disease remission may be interrupted by disease relapses requiring more intensive therapy. Untreated disease has relatively high risk of permanent organ damage and death. It is important to follow your doctor’s recommendation and compliance with follow-up appointments to monitor the disease.

Living With

Living with vasculitis?

Recommendations might vary according to the possible presence of organ functional impairment. Children with this condition should live life as normally as possible. Once the disease is reasonably controlled, patients are encourage to go back to school, play sports, and participate in extra-curricular and family activities. In general, there are no specific limitations to physical activity that children want to do (as long as it is safe). Exercise does not harm vasculitis and in fact, it helps prevent deconditioning, increases muscle strength and muscle endurance.

There is no special diet that has been shown to be effective for vasculitis or worsen the condition. Therefore, children with vasculitis should eat a standard, well-balanced, healthy diet.

Resources

Is there a supporting group for vasculitis?

Vasculitis Foundation is a leading non-profit organization that is dedicated to addressing the needs of adults and children who live with vasculitis in the United States. Many local and national events are organized to educate and connect patients/families together.

For children, more information can be found at http://www.vasculitisfoundation.org.

Last reviewed by a Cleveland Clinic medical professional on 02/08/2019.

References

  • Weiss P. Pediatric Vasculitis. Pediatric Clinic North America. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3348547/) 2012 April, 59(2): 407-423. Accessed 3/22/2019.
  • Schmidt W. Vasculitis. (https://www.sciencedirect.com/science/article/pii/B978143770127210022X) Essential Applications of Musculoskeletal Ultrasound in Rheumatology. 2010, 279-292. Accessed 3/22/2019.
  • Benseler S, Silverman E, Aviv R. Primary central nervous system vasculitis in children. (https://onlinelibrary.wiley.com/doi/full/10.1002/art.21766) Arthritis & Rheumatology. 30 March 2006. Accessed 3/22/2019.
  • Merck Manual Professional Version. Overview of Vasculitis. (https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/overview-of-vasculitis) Accessed 3/22/2019.

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