Henoch-Schonlein purpura is a systemic autoimmune disease characterized by the appearance of a hemorrhagic rash, most often on the skin and mucous membranes, as well as damage to the joints and kidneys. This disease is most often observed in children and can be associated with previous infections, such as streptococcal infection. It can also develop in response to vaccination or taking certain medications. The pathogenesis of purpura includes disorders associated with the deposition of immune complexes in the vascular walls, which leads to inflammation and hemorrhagic syndrome. This disease requires a careful approach to diagnosis and treatment, since, despite its relatively favorable prognostic assessment, it can lead to serious complications, especially with kidney damage.
History of the disease and interesting historical facts
Henoch-Schonlein purpura was first described in the 19th century, namely in 1806, but the name became known only in 1901, when Helmut Schonlein and Heinrich Henoch independently described the clinical picture of the disease. Over the next decades, reports of various epidemics of this disease appeared in the medical literature, which served as the basis for studying the influence of infectious agents on its development. Interestingly, at the beginning of the 20th century, there was an opinion about a possible connection between purpura and vaccination against diphtheria, which significantly increased attention to immune mechanisms.
Epidemiology
Henoch-Schonlein purpura is a disease that occurs with a frequency of 10-20 cases per 100,000 children per year. Children aged 2 to 6 years are most susceptible to this disease, with boys getting sick more often than girls (ratio 2:1). Epidemiological studies show that the disease most often occurs in the autumn-winter period, which may be associated with an increased incidence of respiratory infections at this time of year. In some cases, especially in the presence of concomitant factors such as an allergy history, diseases of the immune system, the course of purpura may be more severe.
Genetic predisposition to this disease
Although Henoch-Schonlein purpura has a number of components that indicate an autoimmune nature, genetic research shows that certain genetic factors may increase susceptibility to the disease. For example, polymorphisms in the genes encoding the HLA-DRB1 and HLA-DQB1 molecules have been identified that may influence the development of the disease. A link with other autoimmune diseases has also been found, which implies a common predisposition, probably related to the body's immunological response.
Risk factors for the development of this disease
There are a number of risk factors that may contribute to the development of Henoch-Schonlein purpura. Physical factors include:
- Viral infections, especially streptococcal infections (eg, sore throat)
- Respiratory viral infections
- Vaccination against various infections (in rare cases)
Chemical factors can also play a significant role:
- Diseases caused by certain medications (antibiotics, nonsteroidal anti-inflammatory drugs)
- Application of X-ray diagnostic contrast medium
Other factors include:
- Genetic predisposition
- General weakness of the immune system
- History of allergic diseases
Diagnosis of this disease
Diagnosis of Henoch-Schonlein purpura is based on clinical findings and laboratory tests. The main symptoms include:
- Hemorrhagic rash, usually on the buttocks, extremities, mucous membranes
- Joint pain, often symmetrical
- Kidney problems such as hematemesis or proteinuria
Laboratory tests help confirm the diagnosis. The following are usually performed:
- Complete blood count with platelet count
- Biochemical blood test to rule out renal pathology
- Immunological tests for the detection of antibodies
Radiological examination may include ultrasound diagnostics to evaluate the kidneys and joints. It is also important to conduct a differential diagnosis with other diseases, such as idiopathic thrombocytopenic purpura and vasculitis.
Treatment
Treatment of Henoch-Schonlein purpura involves a comprehensive approach. Depending on the severity of symptoms, various therapeutic methods may be used:
- General treatment: rest, sufficient fluid intake
- Pharmacological treatment: use of nonsteroidal anti-inflammatory drugs (NSAIDs) to relieve pain
- Glucocorticoids may be prescribed in case of severe course of the disease, as well as to prevent complications
- In the presence of renal impairment, the use of immunosuppressants is possible.
Additionally, in severe cases, surgical treatment may be considered, especially if the disease is complicated by internal bleeding or other life-threatening conditions.
List of medications used to treat this disease
Henoch-Schonlein purpura requires an individual approach to the selection of medications, but the most commonly used are:
- Ibuprofen
- Paracetamol
- Prednisolone
- Methylprednisolone
- Azathioprine
- Mycophenolate mofetil
Disease monitoring
Monitoring the patient's condition includes regular follow-up examinations to track the dynamics of the disease and the effectiveness of therapy. The main control stages include:
- Complete blood count to assess platelet and hemoglobin status
- Biochemical tests to assess kidney function
- Clinical examination to assess joint and skin symptoms
The prognosis with adequate treatment is generally good, but there is a risk of serious complications such as chronic renal failure in severe cases of the disease.
Age-related features of the disease
Henoch-Schonlein purpura has age-related features of manifestation. In children, the disease is milder and has a more favorable prognosis. In elderly patients, the condition can be much more serious, and more intensive therapy is required to prevent the development of complications. Different age groups respond differently to treatment, which is also important to consider when monitoring the patient's condition.
Questions and Answers
- What are the main symptoms of Henoch-Schonlein purpura? The main symptoms are hemorrhagic rash, joint pain and possible kidney problems.
- How is this disease diagnosed? Diagnosis is based on clinical symptoms, laboratory tests and, if necessary, radiological examination.
- What is the prognosis for patients with Henoch-Schonlein purpura? The prognosis is usually favorable with adequate treatment, but complications may develop.
- What is the treatment for this disease? Treatment includes NSAIDs, glucocorticoids, possibly immunosuppressants for severe cases.
- Does the autoimmune nature of the disease influence its course? Yes, autoimmune aspects can determine the severity of the course and the choice of therapeutic strategy.
Advice from Dr. Oleg Korzhikov
It is important to remember that Henoch-Schonlein purpura is a disease that requires careful monitoring. I recommend paying attention to the following aspects:
- Have regular check-ups, even if your condition seems stable.
- Contact your doctor if new symptoms occur or if existing symptoms worsen.
- Follow the prescribed therapy, do not interrupt treatment on your own.
- Discuss vaccination and other preventive measures with your doctor.
- Maintain a healthy lifestyle, which can have a positive impact on your overall health.
