Henoch - Schöenlein Purpura (HSP)
The most common vasculitis in the childhood age group is a vasculitic disease called HSP, and its incidence increases in autumn and spring. In this article, I aimed to briefly inform you about the disease in the form of questions and answers.
What is HSP?
Henoch-Shöenlein purpura (HSP) It is a disease caused by inflammation of small blood vessels (capillaries). This inflammation is called vasculitis and usually affects small blood vessels in the skin, intestines, and kidneys. These inflamed blood vessels can bleed into the skin, causing a dark red or purple rash we call purpura. It can also cause bleeding into the small intestines or kidneys, causing blood in the stool and urine (hematuria).
Dr. Henoch and Dr. Schoenlein described the disease separately more than a hundred years ago.
How common is it?
Although HSP is not a common disease of childhood, it is the most common systemic vasculitis in children between the ages of 5 and 15. It is more common in boys than in girls (2:1). There is no preferred ethnic group or geographical distribution of the disease. The majority of cases seen in Europe or the northern hemisphere occur in winter, but some cases can also be seen in autumn and spring.
What are the causes of the disease?
HSP is unknown. Germs (such as viruses and bacteria) are thought to be an important triggering factor for the disease, as it often occurs following an upper respiratory tract infection. However, HSP can also occur after taking medications, insect bites, exposure to cold, chemical toxins and some food allergens. For these reasons, the term 'allergic purpura' was previously used, considering that HSP was an allergic reaction to all these agents. In some countries, it is also called 'rheumatoid purpura' due to its symptoms related to joints and muscles. The accumulation of some specific products, such as immunoglobulin A, seen in HSP lesions, causes an abnormal immune system response in the skin, joints, gastric tissues. It supports that it attacks the small blood vessels in the trointestinal system, kidneys and rarely the central nervous system and causes disease.
Is it hereditary? Is it contagious? Can it be prevented?
HSP is not a hereditary disease. It is not contagious and cannot be prevented.
What are the main symptoms?
The most common symptom is the characteristic skin rash seen in all HSP patients. . The rashes are usually small, red, pinhead-like, slightly raised from the skin, and sometimes hives-like. The rash gradually turns purple. These rashes are called "palpable purpura". Although purpura is usually seen in the lower extremities and hips, it may also occur in other parts of the body (arms and trunk).
In the majority of patients (65%), Painful joint (arthralgia)or painful and swollen joint (arthritis) with limited movement are observed in the knees, ankles, wrists, elbows and fingers. Arthralgia and/or arthritis occur with soft tissue swelling and tenderness around or near the joint. Soft tissue swelling on the hands, feet, forehead and scrotum (the sac that covers the testicles) may occur in the early stages of the disease, especially in young children. Joint symptoms are temporary and disappear within a few days.
When the intestinal vessels become inflamed, more than 60% of patients experience intermittent abdominal pain around the navel, sometimes accompanied by mild or severe indigestion. It may be accompanied by bleeding from the canal (hemorrhage). Rarely, a condition of intestinal folding in the abdomen, which we call intussusception, may occur, which may cause intestinal obstruction and require surgery.
When the renal vessels become inflamed, 20-35%may cause bleeding in the patient, ranging from mild to severe. Varying degrees of hematuria and proteinuria (presence of protein in the urine)may be observed. Usually kidney problems are not serious. In rare cases, kidney disease can last for months or years and turn into kidney failure (1-5%). In such cases, a nephrologist (kidney specialist) should be consulted and the patient should be consulted by a doctor. It is necessary to cooperate with the patient.
The symptoms described above usually last 4-6 weeks. Rarely, they can be seen a few days before the skin rash appears. They may occur simultaneously or consecutively. Symptoms due to inflammation of the vessels in these organs, such as seizures, brain or lung bleeding, and swelling in the testicles, may rarely be observed.
Is the disease the same in every child? ?
Although the disease is more or less the same in every child, the duration and severity of skin and organ involvement varies greatly from patient to patient. HSP may occur in the form of a single attack or may progress with recurrences that recur five to six times.
Is the disease seen in children different from the disease in adults?
The disease in children is different from the disease in adults. It is no different from the disease, but the disease is less common in adults.
How is it diagnosed?
The diagnosis of HSP is mainly based on classical purpuric symptoms, which are often limited to the lower extremities and hips. It is placed based on the rash and the clinic. Whether or not it is accompanied by abdominal pain, joint involvement and hematuria is not diagnostic. Other diseases that cause a similar picture should be excluded.
Which laboratory tests are useful?
There is no specific test to diagnose HSP. The erythrocyte sedimentation rate or C-reactive protein (a measure of systemic inflammation) may be normal or elevated. Fecal occult blood may be positive due to bleeding in the small intestines. Urine analysis should be performed to detect renal involvement during the course of the disease. Low-grade hematuria is common and improves over time.
If kidney involvement is severe (kidney failure and severe proteinuria), a kidney biopsy is required.
Can it be treated?
Most HSPpatients recover without the need for any medication.
Treatment usually consists of paracetamol or simple analgesics such as ibuprofen and naproxen when joint complaints are evident. It is a supportive treatment using (painkillers). The use of steroids (prednisone) is recommended in patients with severe gastrointestinal symptoms and bleeding and in patients with other organs (e.g. s)It is suitable for rare cases with severe complaints. If kidney disease is severe, a kidney biopsy should be performed and, if necessary, a treatment including immunosuppressant drugs and steroids will be initiated.
What are the side effects of drug treatment?
As is the case in most cases. If drug treatment is unnecessary or used for short periods of time, no serious side effects are expected. Drug side effects may be a problem in rare cases of severe kidney disease that requires long-term use of prednisone and immunosuppressant drugs (see Treatment section).
How long does the disease last?
The total duration of the disease is approximately 4-6 weeks. Half of the children have at least one relapse within a six-week period, which is shorter and milder than the first. Relapses rarely last long. The majority of patients recover completely.
What kind of follow-up examinations are required?
Urine samples are taken 6-7 times during the disease and after recovery to detect kidney problems. should be taken and examined; So much so that, in some cases, kidney involvement may occur weeks after the onset of the disease.
What are the long-term consequences of the disease?
In most children, the disease is self-limiting and It does not cause problems in the long run. Permanent or severe kidney disease is observed in a small percentage of patients and a progressive course resulting in possible kidney failure may occur.
Can he/she continue school/sports?
Physical activity is often restricted during the acute illness, but once the child recovers, he or she can go back to school and lead a normal life. Vaccinations should also be postponed and the time of the missed vaccine should be determined by the child's doctor
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