IgA Vasculitis

Authors: 

Vineel Kumar, Touro University - California, US

Tejwinder Sandhu, Touro University - California, US

What is IgA Vasculitis?

IgA vasculitis, formerly Henoch-Schonlein purpura, is the most common childhood systemic vasculitis (inflammation of small-sized blood vessels). The disease develops after IgA antibodies deposit in the walls of small blood vessels, which results in inflammation.

Symptoms depend on in which organ’s blood vessels the IgA antibodies are deposited. One common place is the skin's blood vessels on the buttocks and legs. In these cases, a palpable red-dotted rash called purpura develops. Abdominal pain and joint pain develop when the blood vessels of the intestines and joints are affected. Most children will completely recover from IgA vasculitis. Very rarely can the disease be severe, leading to kidney damage.

Who is at risk for IgA Vasculitis?

IgA vasculitis can affect anyone. However, it is most commonly seen in young children between 3 and 7 years and more commonly affects boys. Those with a family history of IgA vasculitis are more likely to develop it. Many children develop this disease after an upper respiratory tract infection.

What are the signs and symptoms of IgA Vasculitis?

Signs and symptoms of IgA vasculitis may include:

• Raised red/purple rash (purpura)

  • It is caused by leaking blood vessels in the skin. It usually appears on the legs and buttocks. Sometimes can also occur on the arms, trunk, and face. The rash is not itchy or painful.

• Abdominal pain

  • It can be mild to severe. Blood may be present in the stool. In rare cases, intussusception can occur, which means the bowel folds onto itself, causing a blockage.

• Joint pain and swelling

• Blood in the urine

• Swelling of the testicles in boys

• Seizures- rare

• Pneumonia-rare

• Rash, abdominal pain, and joint pain are the first symptoms, preceded by an upper respiratory tract infection weeks prior.

  
  

What tests will my doctor perform to diagnose IgA Vasculitis?

IgA vasculitis is diagnosed clinically after a thorough history, physical examination, and a tissue biopsy (skin or kidney biopsy). Your doctor will order some laboratory studies to help exclude other diagnoses and to evaluate kidney function. Tests may include:

• Blood tests

• Urine tests

• Gastrointestinal and kidney ultrasound scans

• Skin biopsy of the rash

• Kidney biopsy

  
  

Treatment of IgA Vasculitis

For the most part, IgA vasculitis is a self-limiting disease, meaning patients will recover independently within several weeks or less without treatment. To date, no therapy is known to shorten the duration of IgA vasculitis. Your doctor may recommend medications, such as anti-inflammatory medications, to treat and relieve symptoms such as abdominal pain, joint pain, and swelling. If the kidneys are involved, the treatment goal will be to prevent chronic kidney disease. In case of severe or recurrent disease, your doctor may prescribe medications to suppress the immune system.

Will I develop kidney failure if I have IgA Vasculitis?

The likelihood of developing kidney failure with IgA vasculitis is rare. Patients should have close follow up with their doctors for six months to look for signs of kidney disease. Adults are at a higher risk of developing kidney disease than children. In rare cases, kidney failure develops, requiring long-term hemodialysis until a kidney is available for transplantation.

Clinical trials for IgA Vasculitis

New treatments and therapies are being developed for kidney diseases. Ask your doctor if you want to participate in a clinical trial for IgA vasculitis. More information is available at: www.enrollmypatient.org