What is Henoch-schönlein purpura (HSP)?
It is a vasculitis that usually develops after an upper respiratory tract infection and progresses with the involvement of small vessels. Vasculitis means inflammation of the vessel wall. In HSP, especially skin and joint findings are prominent. In a minority of cases, HSP can cause severe kidney or bowel disease.
HSP is more common in children than adults, but can be more severe when it occurs in adults.
Who gets Henoch-Schonlein Purpura?
Usually, HSP develops after an upper respiratory tract infection. It is the most common form of vasculitis in children. Male/female=1.5. It is more common in the seasons, especially in the spring. It is common in children between the ages of 4-7. frequency; 1.4 / 10 000. In adults, it is more common in young-adults (in their 20s); however, it should not be forgotten that it can develop at any age.
What is the cause of Henoch-Schonlein Purpura?
The cause of this abnormality in the immune system is not known exactly. The onset of the disease in two-thirds of the cases, on average ten days after an upper respiratory tract infection, suggests the contribution of an infection as environmental exposure in the genetically predisposed individual. In addition, environmental factors such as food, insect bites, vaccination are thought to be responsible.
What are the symptoms of Henoch-Schonlein Purpura?
Most pediatric patients have joint pains or arthritis (arthritis) with palpable purpura (red bleeding foci under the skin). With the involvement of the gastrointestinal tract in 70% of pediatric patients; abdominal pain, nausea, vomiting, diarrhea or constipation. There is less blood and mucus (slimy appearance) in the stool. More rarely, intussusception may occur. Renal involvement is mild in 10-50% of patients. Very rarely, progressive kidney disease develops. More than 90% of cases are children. The disease usually resolves within a few weeks. However, the situation is somewhat different in adults. Skin manifestations are more variable in adults and sometimes the symptoms last longer in adults. Permanent kidney damage in adults is more common than in children. However, less than 5% of patients with HSP develop progressive renal failure. Therefore, renal involvement in adults should be taken more seriously; Treatment should be given in the early period and should be followed closely.
HSP in adults can be confused with other forms of systemic vasculitis. Wegener’s granulomatosis and microscopic polyangiitis also progress with purpura, arthritis and kidney inflammation. In the course of these diseases, there is both more serious involvement in other organs (for example, lung, eye and nerve involvement) and more serious kidney involvement. Therefore, it can be differentiated by careful evaluation with blood tests, urinalysis, lung imaging and biopsy. HSP vasculitis, when direct immunofluorescence (DIF) test on skin biopsy and IgA cannot be detected (such as sampling from old rash, incorrect sampling); may be mistakenly diagnosed as hypersensitivity vasculitis.
How is Henoch-Schönlein Purpura diagnosed?
The presence of palpable purpura (platelet count is normal) due to inflammation of the blood vessels of the skin, along with other symptoms such as joint findings, abdominal pain and kidney involvement findings suggest HSP. Except for the skin rash, all other symptoms may not be present at the same time. The diagnosis of HSP was made in the (microscopically marked) skin biopsy; can be confirmed by demonstrating the deposition of IgA and C3 (complement 3) in the walls of small blood vessels. However, this can be shown in skin biopsies taken within the first 48 hours of the rash. It may be lost if taken later.
How is Henoch-Schonlein Purpura treated?
Skin rash usually in high gravity; develops in the hips, ankles, and legs. For this reason, patients should not stay too long; It is recommended that they stretch their legs as much as possible. Joint symptoms regress with non-steroidal anti-inflammatory drugs (NSAIDs, such as naproxine, diclofenac, indomethacin); however, it may worsen gastrointestinal (gastrointestinal) symptoms and should be avoided in patients with kidney disease. Steroids in the treatment of HSP; used according to the clinical findings of the patient. It is not used in the early period only in cases with skin rash; because it usually goes away on its own. If the skin findings do not regress or continue to increase; low-to-moderate doses of steroids can be used for a short time. Since most patients have joint and gastrointestinal symptoms, steroids are also used to alleviate them. In those with severe gastrointestinal bleeding or kidney involvement, higher doses of steroids are used. In those with progressive kidney involvement; In addition to steroids, immunosuppressive (immunosuppressive) drugs can be added to the treatment. In one third of the patients, the findings may recur within the first 6 months.
