Vasculitis In Children Causes And Treatment

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Introduction to paediatric vasculitis

Vasculitis is a complex group of disorders characterised by inflammation of blood vessels and can affect individuals of all ages. However, it presents unique challenges that demand specialised care when children are affected. This article aims to provide a comprehensive guide to vasculitis in children, honing in on its treatment options and management methods. The article will cover the causes, clinical presentations, diagnosis, various types of paediatric vasculitis, and treatment approaches. By the end of this article, you will have an increased understanding of this condition and how healthcare regulations influence its treatment.

Vasculitis in children: An overview

Definition and Overview

Vasculitis is a condition characterised by inflammation of blood vessels. In children, it can affect the body's arteries, veins, or capillaries. These inflamed vessels can lead to a variety of symptoms and complications, making early diagnosis and appropriate treatment critical.1

Prevalence and age distribution

While vasculitis is rare in children compared to adults, it can still occur. The prevalence varies among different types of vasculitis, and age distribution often plays a role in diagnosis and management.1

Types of vasculitis in children

There are several types of vasculitis that can affect children, including Kawasaki disease, Henoch-Schoenlein purpura, polyarteritis nodosa, and Takayasu arteritis. Each type has distinct characteristics and requires tailored approaches to treatment.2

Understanding the causes and risk factors3

Immunological factors

Vasculitis often involves the body’s immune system mistakenly attacking its own blood vessels. Autoimmune factors play a significant role in the development of vasculitis in both children and adults.

Genetic predisposition

Genetic factors can contribute to a child's susceptibility to vasculitis. Understanding the disease's genetic basis can aid in diagnosis and treatment decisions.

Environmental triggers

Environmental factors, such as infections, toxins, or certain medications, can trigger vasculitis in children. Identifying and avoiding these triggers is crucial for managing the condition effectively.

Recognising clinical presentation4

Common symptoms

Vasculitis in children can manifest in a variety of ways, including fever, skin rashes, joint pain, abdominal pain, and organ-specific symptoms. Recognising these signs is vital for early intervention.

Differential diagnosis

Given the diverse symptoms of vasculitis, healthcare professionals often need to rule out other conditions that may present similarly. This step is key for accurate diagnosis and appropriate treatment planning.

The importance of early diagnosis

Early diagnosis of vasculitis in children is associated with better outcomes and reduced risk of complications. Delayed diagnosis can lead to severe organ damage, underscoring the importance of timely medical attention.

Diagnostic methods4

Medical history and physical examination

A thorough medical history and physical examination are the initial steps in diagnosing vasculitis. The information gathered during this process helps to guide further diagnostic testing.

Laboratory tests

Blood tests, including a complete blood count and inflammatory markers, can provide valuable information about the presence and extent of inflammation. Specific antibodies or markers may be indicative of certain types of vasculitis.

Imaging studies

Imaging techniques such as ultrasounds, computed tomography scans, and angiography can visualise blood vessels and identify areas of inflammation or damage.

Biopsy procedures

In some cases, a biopsy of affected tissue, typically skin or an affected organ, may be necessary to confirm the diagnosis and determine the type and extent of vasculitis.

Classification of paediatric vasculitis5

Primary vasculitis

Primary vasculitides are conditions where vasculitis is the main problem. These include Kawasaki disease, Henoch-Schoenlein purpura, and childhood polyarteritis nodosa.

Secondary vasculitis

Secondary vasculitides are associated with other underlying conditions, such as connective tissue disorders or infections. Identifying and treating the underlying cause is essential for managing secondary vasculitis.

Systemic vs organ-specific vasculitis

Vasculitis can affect multiple organ systems (systemic) or target specific organs (organ-specific). Treatment strategies may differ based on this classification.

Treatment approaches

General principles of management

The management of paediatric vasculitis involves a multi-faceted approach that includes controlling inflammation, addressing symptoms, and preventing complications. Regular follow-up with a paediatric rheumatologist or specialist is needed.

Medication options

Corticosteroids6

Prednisone: It is a commonly prescribed corticosteroid which is used to reduce inflammation in vasculitis. It is often administered orally and can be tapered based on the child’s response and the type of vasculitis.

Methylprednisolone

In severe cases or when rapid control of inflammation is necessary, intravenous methylprednisolone may be given as a pulse therapy.

Prednisolone

This medication, often preferred in paediatric patients due to its relatively lower risk of side effects, can also be used to manage vasculitis.

Corticosteroids are effective in reducing inflammation, but long-term use can lead to side effects such as weight gain, mood swings, and osteoporosis. Careful monitoring and dose adjustments are essential.

Immunosuppressive agents6

In more severe or refractory cases of paediatric vasculitis, immunosuppressive drugs may be introduced to modulate the immune response and prevent further damage. These agents include:

Methotrexate

Methotrexate is an immunosuppressive drug that may be used as a steroid-sparing agent in certain types of vasculitis.

Cyclophosphamide

Cyclophosphamide is a potent immunosuppressant and is reserved for severe cases when other treatment methods have failed.

Azathioprine

Azathioprine may be used as a maintenance therapy to reduce the need for higher doses of corticosteroids.

Biologic therapies

Biologic therapies have revolutionised the treatment of vasculitis by targeting specific molecules involved in the inflammatory process. Examples of biologics used in paediatric vasculitis treatment include:

Rituximab

Rituximab targets beta cells, part of the immune system, and has shown promise in treating certain types of vasculitis, particularly in cases resistant to conventional therapies.

Tocilizumab

This biologic agent blocks the action of interleukin-6, a pro-inflammatory cytokine, and has been used in refractory cases of vasculitis.

Biologic therapies are administered via intravenous infusion and may be associated with a reduced risk of long-term side effects compared to high-dose corticosteroids.

Supportive care

Supportive care measures an integral part of paediatric vasculitis management and can include:

Pain management

Over-the-counter or prescription pain relievers may be recommended to alleviate joint or abdominal pain.

Physical therapy

Physical therapy can help children regain strength and mobility, especially if vasculitis has caused joint or muscle problems.

Dietary modifications:

Depending on the child’s specific symptoms, dietary changes may be recommended, such as a low-sodium diet for managing hypertension associated with vasculitis.

Age-specific challenges

The treatment of paediatric vasculitis must consider the child’s age, growth, and development. Medications and treatment plans may need adjustment as the child grows, and regular monitoring of growth and development milestones is essential.

Adverse effects of treatment

Many medications used to treat vasculitis can have side effects, especially when used over an extended period of time. Paediatric patients require close monitoring to manage and alleviate these potential risks. Healthcare providers carefully weigh the benefits of treatment against the potential side effects and adjust treatment plans accordingly.

Quality of life and psychosocial support 7

Caring for a child with vasculitis can be emotionally challenging, both for the child and their family. Support from healthcare professionals, including paediatric psychologists or social workers, can help children and their families cope with the emotional and psychological aspects of living with a chronic illness. Ensuring a child’s quality of life and overall well-being is a top priority in paediatric vasculitis management.

Prognosis and outcomes

Disease course variability7

The course of vasculitis in children can vary greatly, depending on the type, severity, and response to treatment. Some children may experience remission and go on to lead relatively normal lives, while others may require ongoing management to control their condition. Close monitoring and regular follow-ups with a healthcare team are key to adapting treatment strategies as required.

Factors influencing prognosis

Several factors can influence the long-term prognosis of paediatric vasculitis, including the type of vasculitis, the child’s age at diagnosis, the effectiveness of treatment, and the presence of complications. Early diagnosis and prompt treatment are important factors associated with better outcomes.

Potential complications

If left untreated or undertreated, vasculitis can lead to severe complications, such as organ damage, growth delays, and long-term health issues. It is essential for physicians and families to work together to manage the condition effectively and minimise these risks. Regular checkups and ongoing communication with the healthcare team are vital.

Conclusion

In conclusion, paediatric vasculitis is a complex condition that requires a comprehensive approach to diagnosis and treatment. The management of vasculitis in children involves a combination of medications, supportive care, and close monitoring to achieve the best possible outcomes. As ongoing research continues to advance our understanding of vasculitis and improve treatment options, there is hope for a brighter future for children living with these challenging conditions. By staying informed and working collaboratively with healthcare providers, families can provide the best possible care and support for their children with vasculitis.

FAQs on Paediatric Vasculitis

  1. What is the most common cause of vasculitis in children?

Kawasaki disease is one of the most common forms of vasculitis in children. It primarily affects young children and can lead to inflammation of the blood vessels, especially in the coronary arteries.

  1. What causes vasculitis in children?

The exact cause of vasculitis in children is not always clear. It often involves the immune system mistakenly attacking blood vessels. Various factors, including genetics, environmental triggers, and infections, can contribute to the development of vasculitis.

  1. Is vasculitis common in children?

Vasculitis is relatively rare in children compared to adults. However, it can occur, and the prevalence may vary depending on the specific type of vasculitis.

  1. Which vasculitis is common in childhood?

Apart from Kawasaki disease, Henoch-Schoenlein purpura (HSP) is another common type of vasculitis seen in childhood. HSP often presents with skin rashes, joint pain, and abdominal symptoms.

  1. How urgent is vasculitis?

Vasculitis can be a serious condition, and the urgency of treatment depends on the type and severity. Some forms of vasculitis, like Kawasaki disease, require immediate medical attention due to the risk of complications, especially involving the heart.

  1. Why are doctors worried about vasculitis?

Doctors are concerned about vasculitis because it can lead to inflammation and damage in blood vessels, affecting various organs and systems. Prompt analysis and treatment are fundamental to prevent complications.

  1. Is vasculitis something to worry about?

While not all cases of vasculitis are severe, it is a condition that should be taken seriously. Timely diagnosis and appropriate treatment are essential to manage vasculitis effectively and reduce the risk of complications.

  1. What is the average age for vasculitis?

The age at which vasculitis occurs can vary depending on the type. Kawasaki disease typically affects young children, while other forms of vasculitis may occur in older children or adolescents.

  1. What test confirms vasculitis?

A definitive diagnosis of vasculitis often requires a biopsy of affected tissue, such as skin or an affected organ. Blood tests, imaging studies, and a thorough medical history and physical examination help confirm the diagnosis and determine the type and extent of vasculitis.

References

  1. Eleftheriou D, Brogan PA. Vasculitis in children. Paediatrics and Child Health [Internet]. 2018 Feb [cited 2023 Sep 20];28(2):57–63. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1751722217302378
  2. Vasculitis | Boston Children's Hospital [Internet]. [cited 2023 Sep 20]. Available from: https://www.childrenshospital.org/conditions/vasculitis#:~:text=The%20two%20most%20common%20forms%20of%20vasculitis%20in,coronary%20arteries%20%28blood%20vessels%20around%20the%20heart%29.%20
  3. Vasculitis - causes and risk factors | nhlbi, nih [Internet]. 2023 [cited 2023 Sep 20]. Available from: https://www.nhlbi.nih.gov/health/vasculitis/causes
  4. Philadelphia TCH of. Vasculitis [Internet]. 2014 [cited 2023 Sep 20]. Available from: https://www.chop.edu/conditions-diseases/vasculitis
  5. https://www.vasculitis.org.uk/wp-content/uploads/2019/08/paediatric-vasculitis.pdf [Internet]. 04-Foster-04.indd; 2012. The classification of paediatric vasculitis, Chapter 4.
  6. GOSH Hospital site [Internet]. [cited 2023 Sep 20]. Vasculitis. Available from: https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/vasculitis/
  7. Cleveland Clinic [Internet]. [cited 2023 Sep 20]. Iga vasculitis in children: what causes it? Available from: https://my.clevelandclinic.org/health/diseases/21086-vasculitis-in-children

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This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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