Schönlein

Schönlein-Henoch Purpura (SHP), also known as IgA vasculitis, is a form of vasculitis that affects small blood vessels, primarily characterized by the combination of purpura, arthritis, abdominal pain, and kidney disease. It is more common in children than in adults, but it can affect individuals at any age. The condition is named after Johann Lukas Schönlein and Eduard Heinrich Henoch, who were the first to describe the disease in the 19th century.

Causes and Pathophysiology

Schönlein-Henoch Purpura is primarily caused by an abnormal immune response. The exact trigger is unknown, but the condition often follows an upper respiratory tract infection. It is associated with the deposition of Immunoglobulin A (IgA) antibodies in the walls of small blood vessels, leading to inflammation and bleeding.

Symptoms

The hallmark symptom of SHP is a purpuric rash, which is a rash characterized by small red or purple spots on the skin that do not blanch when pressed. This rash is most commonly found on the lower extremities. Other symptoms include:

• Arthritis or joint pain, typically in the knees and ankles
• Abdominal pain, which can be severe and is caused by inflammation in the gastrointestinal tract
• Kidney disease, manifesting as hematuria (blood in urine) or proteinuria (protein in urine), which can progress to more severe kidney damage in some cases
• Rarely, SHP can affect other organs, including the lungs and brain

Diagnosis

Diagnosis of Schönlein-Henoch Purpura is primarily based on clinical presentation. Laboratory tests may show elevated levels of IgA. A skin biopsy or kidney biopsy can confirm the presence of IgA deposits in the vessel walls.

Treatment

There is no specific cure for SHP, and treatment focuses on managing symptoms and preventing complications. Mild cases may not require treatment beyond symptom management. In more severe cases, medications such as corticosteroids may be used to reduce inflammation. In cases with significant kidney involvement, more aggressive treatments, including immunosuppressive drugs, may be necessary.

Prognosis

The prognosis for individuals with Schönlein-Henoch Purpura is generally good, especially for children. Most symptoms resolve without long-term damage within a few weeks to months. However, kidney involvement can lead to chronic kidney disease in a small percentage of cases.

Epidemiology

Schönlein-Henoch Purpura is the most common form of vasculitis in children. It has a peak incidence in the age group of 4 to 6 years. The condition is less common in adults, and the prognosis can be worse with more severe kidney involvement.

History

The disease was first described by Johann Lukas Schönlein in 1837, with further characterization of the gastrointestinal and renal involvement by Eduard Heinrich Henoch in the latter half of the 19th century.

See Also

• Vasculitis
• Immunoglobulin A
• Purpura
• Kidney disease