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Henoch–Schönlein purpura (HSP), also known as anaphylactoid purpura or purpura rheumatica, is an autoimmune disease of the skin and mucous membranes. It is a severe inflammation of the blood vessels, caused by immunoglobulin A (IgA) immune complexes deposits in small vessels. Vast majority of the HSP cases involve children under the age of 10 years. However, this condition may also be seen in adults and infants. The disease usually affects several different organs at the same time, and it is characterized by rash, abdominal pain and joint pain.
Henoch–Schönlein purpura characteristics
Henoch–Schönlein purpura is a severe systemic vasculitis, characterized by the deposition of Immunoglobuline A (IgA) and complement component 3 (C3) in the blood vessels. The disease can affect skin, joints, kidney and even gastrointestinal tract. Up to 90% of the cases involves children under the age of ten years, and almost half of those are affected before the age of five years. Moreover, the HSP can also occur in infants or even in adults. Course of the disease in infants is usually mild, but in adults it can be more severe. HSP potential complictions include renal, gastrointestinal and pulmonary disorders. The most frequent consequnce of Henoch–Schönlein purpura is kidney damage, which in extreme cases may lead to death.
Causes and symptoms related to Henoch–Schönlein purpura
Although the cause is still unclear, Henoch–Schönlein purpura may occur as a consequence of bacteria or virus infection, resulting in abnormal reaction of the immune system. In addition, it may be triggered by some types of drugs and vaccinations. Genetic background may also be a determining factor of this disease.
Henoch–Schönlein purpura is characterized by few main symptoms, including:
- Purpura, which is similar to rash
- Abdominal pain
- Kidney involvement
Purpura occurs in all cases. It appears usually on the legs and buttock, but it can affect any other part of the human body. It spreads symmetrically and escalates in the areas exposed to pressure. Swollen and sore joints appear in 8 out of 10 cases. It usually involves knees, ankles and elbows. This symptom does not cause any permanent damage to the body. Another main symptom is abdominal pain, which occurs in over half of the cases. It is usually colicky in nature and can get worse after meals. Diarrhea, nausea, vomiting and constipation may also appear along with abdominal pain. Henoch–Schönlein purpura can also affect the kidney, resulting in hematuria or proteinuria. Those last mentioned symptoms may seem dangerous, since they last for a long period of time. Chronic kidney disease affects less than 1% of the HSP patients.
Diagnosis and treatment
Henoch–Schönlein purpura can be diagnosed with ease. The combination of the symptoms is fairly unique, and currently there are not many diseases bearing the same hallmarks. Occasionally, HSP may be mistaken with other autoimmune diseases, for instance with juvenile rheumatoid arthritis. If necessary, blood test and urinalysis can be run in order to determine the existence of the disease. In some cases, it is recommended to perform the biopsy of the skin or kidney, which will definitely secure the diagnosis.
Majority of the patients do not need any specific treatment, apart from controlling the symptoms, since HSP resolves spontaneously in over 90% of the cases. Symptomatic treatment include pain killers for joints and abdominal pain. In some cases oral steroids may be used to reduce gastrointestinal and renal disorders. Combination of steroids and immunosuppressive drugs can be given, if there are no benefits of using steroids alone.