IgA Vasculitis Immunoglobulin A Vasculitis (Henoch-Schönlein Nephritis, PSH)
DISEASE DEFINITION
Immunoglobulin A vasculitis, formerly known as Henoch-Schönlein purpura (HSP), is a type of vasculitis characterized by inflammation of small blood vessels (capillaries, arterioles, and venules) in the skin, joints, intestines, and kidneys. It is characterized by the deposition of IgA immune complexes in the blood vessel walls, leading to inflammation and damage. IgA vasculitis is most commonly seen in children but can also affect adults.
SYMPTOMS
Symptoms and features of IgA vasculitis may include:
Purpura (purple spots on the skin)
This is the hallmark sign of IgA vasculitis. Purpura refers to small, red or purple skin spots or bruises, typically on the lower legs and buttocks. These skin lesions may be palpable and often appear after an upper respiratory tract infection or other triggers.This occurs in nearly all children with the condition. This most often occurs over the buttocks, lower legs, and elbows.
Abdominal Pain
Gastrointestinal symptoms like abdominal pain, nausea, and vomiting may occur, and sometimes there can be gastrointestinal bleeding.
Joint Pain
Abnormal urine/kidney involvement
Arthralgia (joint pain) and arthritis (joint inflammation) are common, affecting the knees and ankles most often.
About one-third of cases involve the kidneys, leading to hematuria (blood in the urine) and proteinuria (protein in the urine). Severe kidney involvement can lead to glomerulonephritis.
Other Symptoms
Swelling and pain in the scrotum, headaches, involvement of other organs (such as the lungs and central nervous system)
DIAGNOSIS
Diagnosis is typically based on clinical symptoms, physical examination, laboratory tests and in some cases kidney biopsy. The tests may include:
- Urine analysis
- Blood examination
- Complete blood count
- complement factors
- immunoglobulin status
- to look for other causes of blood vessel inflammation (systemic lupus erythematosus, ANCA-associated vasculitis or hepatitis, C3 nephritis)
- Coagulation tests
- Skin biopsy
- Kidney biopsy
- in cases of impaired renal function and/or high proteinuria
- when immunosuppressive therapy is considered
- in the case of long-lasting small proteinuria
- Imaging of the abdomen
TREATMENT
The treatment of IgA vasculitis depends on the severity of symptoms and organ involvement.
In many cases, supportive care is sufficien including:
- rest
- pain relief
- management of gastrointestinal symptoms
In severe cases or when kidney involvement is present, immunosuppressive medications may be prescribed:
- corticosteroids or other immunosuppressants
- they reduce inflammation and protect the kidneys
It's important for individuals with IgA vasculitis to receive appropriate medical evaluation and follow-up, especially when kidney involvement is suspected, as early intervention can help prevent complications.
PROGNOSIS
Most children with IgA vasculitis recover fully without long-term complications. In adults, the course of the disease can be more variable, and some individuals may experience relapses. Kidney involvement can lead to long-term kidney damage in a minority of cases.