IgA-associated vasculitis - causes, symptoms, complications and treatment

IgA-associated vasculitis (formerly Henoch-Schonlein disease or Henoch-Schonlein purpura) affects children in 90% of cases. Most often it develops suddenly in healthy children. The first case of Henoch-Schonlein purpura was described in 1802, but the disease still poses a serious diagnostic problem. This the most commonly diagnosed form of acute inflammation of small vessels in children Inflammation primarily affects arterioles and capillaries, but can also affect other blood vessels. The peak incidence occurs at 6 years of age, but the risk group includes children 3-15 years old, as well as adults - 10% of diagnosed cases occur in adults.

During the study, it was shown that the disease can be inherited, which for scientists is proof that it is genetically determined. Some studies indicate that boys are more susceptible to the disease, but there is no clear evidence of an association between acute vasculitis and gender. Henoch-Schonlein purpura is associated with an immune response to certain infections and medications; vaccinations, insect bites, and hypothermia have also triggered an immune response in some children.

Currently, Henoch-Schonlein purpura is diagnosed in 10-20 children per 100,000 population per year. This disease is associated with a high risk of organ complications, including those in the gastrointestinal tract and kidneys. Inflammation caused by IgA antibodies deposited in the walls of blood vessels can spread to other organs, leading to necrosis.

In adults, Henoch-Schonlein disease is diagnosed 100 times less frequently than in children. It applies equally to women and men.

Causes of IgA-Associated Vasculitis

Henoch-Schonlein purpura develops for unknown reasons, but certain factors have been identified that are involved in the development of inflammation of small blood vessels. Immunological mechanisms primarily lead to excessive deposition of IgA antibodies in the walls of blood vessels.

In the development of Henoch-Schonlein purpura, an important role is played by IgA antibodies, which are present not only in the blood of diagnosed patients, but also accumulate in the form of deposits in the walls of blood vessels. Deposits of IgA antibodies are also present in organs affected by purpura.

IgA-associated vasculitis is thought to be caused by an inappropriate response of the immune system to certain antigens. The disease often develops as a result of infectious agents - in about 90% of young patients, the symptoms of Henoch-Schonlein purpura were preceded by an upper respiratory tract infection that developed within 3 weeks before the onset of the disease.

A high risk of infection is associated with sinusitis and dental diseases caused by bacteria. Henoch-Schonlein purpura can be associated with infection of the body by various pathogens, such as Helicobacter pylori, parvoviruses and parasites.

Other possible causes of purpura are, for example, food allergens, drugs, incl. antibiotics, excessive cooling of the body, insect bites the body’s reaction to protective vaccinations. In adults, Henoch-Schonlein purpura may indicate, among other things: for paraneoplastic syndrome.

How does Henoch-Schonlein purpura manifest itself?

IgA-associated vasculitis can cause not only inflammation of small vessels, but also soft tissue edema, which leads to organ complications. It is associated with the blood supply to all organs of the body.

The result of the development of Henoch-Schonlein purpura can be kidney disease - most often it causes changes in the glomeruli. Other common complications include inflammation and digestive disorders such as dull abdominal pain, as well as joint pain and arthritis. Rarely, this disorder causes central nervous system symptoms such as headaches.

The main, but not always the first symptom indicating Henoch-Schonlein purpura is skin changes. During the course of the disease, urticaria, erythema, or a papular rash may initially appear, which eventually transforms into petechiae and subcutaneous effusions. Skin lesions rise above the surface of the skin. Half of the children diagnosed with Schonlein-Henoch disease before the rash developed had gastrointestinal or joint symptoms.

The classic “set” of symptoms of purpura:

• petechiae without thrombocytopenia and coagulopathy,

• joint pain or arthritis

• gastrointestinal symptoms (abdominal pain, vomiting, bloody diarrhea),

• renal symptoms (IgA nephropathy, glomerulonephritis).

The disease may be accompanied by swelling of the soft tissues, such as swelling of the hands, swelling of the joints of the lower extremities, swelling of the forehead and swelling of the testicles.

Complications of Henoch-Schonlein disease

Henoch-Schonlein disease causes symptoms of varying severity; in younger children it is less pronounced.

Unfortunately, sometimes this causes serious complications. They concern individual organs in which deposits of IgA antibodies have accumulated. Serious complications resulting from vasculitis include:

• kidney failure,

• severe bleeding into the gastrointestinal tract,

• bleeding into the lungs

• pneumonia,

• hemorrhage in the brain

• convulsions

• intestinal obstruction,

• inflammation of the pancreas.

Inflammation of small vessels requires appropriate treatment.

How is Henoch-Schonlein purpura treated?

Treatment of Henoch-Schonlein disease depends primarily on the severity of the symptoms and their type. It is necessary to exclude bacterial and viral causes of the disease. During the illness, non-steroidal anti-inflammatory drugs, vascular homeostatic drugs and steroid drugs. In case of joint pain, rest and limitation of physical activity is recommended.

After the symptoms disappear, blood pressure measurement and more frequent urinalysis may be required. Women who had Henoch-Schn disease as children are at an increased risk of high blood pressure during pregnancy.