Rare but Serious – Understanding Childhood Systemic Vasculitis

Vasculitis means inflammation of the blood vessels. While some types—like Henoch–Schönlein purpura—are common and usually mild in children, others are much rarer and more serious. These forms are called primary systemic vasculitis, and they require careful diagnosis and long-term treatment.

The most well-known types in children include:

• Polyarteritis nodosa (PAN)

• Takayasu arteritis

• Granulomatosis with polyangiitis (GPA, formerly Wegener’s)

• Microscopic polyangiitis (MPA)

• Other rare forms, including eosinophilic granulomatosis with polyangiitis (EGPA)

⚠️ Important: Systemic vasculitis can lead to serious complications if not recognized early. If your child has persistent fever, unusual fatigue, severe abdominal pain, unexplained high blood pressure, or signs of organ involvement, seek medical evaluation urgently. Early diagnosis and treatment can be life-saving.

What Do These Diseases Have in Common?

All of these conditions involve inflammation in blood vessel walls, which can reduce or block blood flow to important organs. Depending on which organs are affected, symptoms can vary widely—and sometimes appear suddenly.

Common features may include:

• Fever, fatigue, and weight loss

• Skin rash or nodules

• Muscle and joint pain

• High blood pressure

• Abdominal pain

• Nerve problems (numbness, weakness)

• Kidney or lung involvement (in some forms)

Because they can affect multiple organs at once, early recognition is essential.

When Should You Suspect It?

These forms of vasculitis are rare, but should be considered when a child has:

• Persistent unexplained fever

• Elevated inflammatory markers (like ESR, CRP)

• Organ symptoms that don’t respond to usual treatments

• High blood pressure or kidney abnormalities in an otherwise healthy child

Pediatric rheumatologists work with nephrologists, cardiologists, neurologists and others to make a precise diagnosis.

How Are They Diagnosed?

There’s no single test for vasculitis, so diagnosis involves:

• Blood and urine tests

• Imaging (ultrasound, CT, MRI, angiography) to detect vessel inflammation or narrowing

• Biopsy of affected tissue (if safe and needed)

It can take time and collaboration across specialties to confirm the diagnosis.

What Treatments Are Used?

Treatment usually begins with corticosteroids to quickly control inflammation, often followed by immunosuppressive medications such as:

• Cyclophosphamide

• Azathioprine

• Methotrexate

• Biologic agents in resistant or relapsing cases (e.g. rituximab)

Because these diseases can affect vital organs, prompt and aggressive treatment is essential—but the goal is always remission and long-term stability.

What Is the Outlook?

Thanks to advances in medicine, most children can achieve remission and return to normal activities. Long-term monitoring is necessary, especially if organs like the kidneys, lungs, or heart were affected.

Family support, open communication with healthcare providers, and regular follow-up are key to managing life with a rare vasculitis.

Learn More

The PRINTO leaflet on systemic vasculitis explains the different types, symptoms, treatments, and what families should expect.

🔗 Read more on PRINTO:

Vasculitis – PRINTO Patient Leaflet

All rights and content remain with PRINTO, and the original resources can be found at:

https://www.printo.it/pediatric-rheumatology/GB/info/GB