Henoch-Schönlein purpura

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Classification according to ICD-10
D69.0 Anaphylactoid purpura
ICD-10 online (WHO version 2019)

The Purpura Henoch , and Henoch-Schonlein purpura (synonyms: rheumatoid purpura , Immunkomplexpurpura , purpura , anaphylactoid purpura , formerly peliosis rheumatica ) is an immunologically mediated inflammation of the small blood vessels ( vasculitis of capillaries and pre- and postcapillary vessels) of unknown Etiology , which, as a multi-system disease, preferably affects the skin, joints, intestines and kidneys. It occurs most frequently in preschool and school age after a previous respiratory tract infection or other triggers (e.g. medication), begins acutely and often takes the form of several attacks. In the great majority of cases, the disease heals without consequences. It is named after the German doctors Johann Lukas Schönlein (1793–1864) and Eduard Heinrich Henoch (1820–1910). Henoch-Schönlein purpura was renamed IgA vasculitis at the 2012 Chapel Hill Conference .

Epidemiology

Henoch-Schönlein purpura is the most common childhood vasculitis, affecting 15 to 25 out of 100,000 children and adolescents each year. The mean age at the onset of the disease is six to seven years; the disease is rare in the first two years of life. It occurs more frequently in the cold season and affects boys more often than girls.

Pathogenesis

The purpura Schönlein-Henoch is probably an immunopathological reaction on a genetic basis, which is caused by different triggers: The confrontation with an unknown antigen leads to the proliferation of an increased rate of circulating IgA- secreting B- lymphocytes . Make it multiplies IgA - immune complexes , which are reflected in the small vessels, complement activate granulocytes attract and other white blood cells, and finally to an aseptic inflammation of the vascular bed in the skin, gut and Nierenglomerula lead. Because of this pathogenesis , Henoch-Schönlein purpura is classified as belonging to the group of type III hypersensitivity reactions ( Arthus reaction ) according to the classification by Coombs and Gell .

Symptoms

Guiding symptoms

Typical purpura on the lower leg
Extremely pronounced purpura on the inside of the thigh
Extremely pronounced purpura on the back of the thighs
  • Skin (up to 100%): The ultimately typical palpable purpura is only an initial symptom in 50% of cases. Rather, for hydrostatic reasons, it arises in dependent areas as a hemorrhage on a wheal . Are then formed with a glass spatula no longer blanchable, reddish-brown, slightly raised and therefore palpable, some still quaddelige ( urticarial ) skin lesions with different sizes, partly confluent petechiae symmetrically preferred on the extensor surfaces of the legs and occur on the buttocks.
  • Gastrointestinal tract (30-75%): Colic-like abdominal pain, blood in the stool, vomiting and, as rare complications, an ulcer , intussusception (intertwining of the intestinal loops), ileus (interrupted transport of the intestinal contents), ischemia (interruption of the Blood flow in a section of the intestine with tissue destruction) and perforation (hole in the intestinal wall). Bleeding can lead to shock due to a decrease in the amount of blood remaining.
  • Joints (40–85%): Mostly there is a fleeting inflammation of the ankle and / or knee joints, often with fleeting swelling of the feet and / or hands.
  • Kidney (6–60%): blood in the urine, protein in the urine up to nephrotic syndrome , decreased kidney function (measurable by creatinine clearance ) up to terminal kidney failure and high blood pressure are possible. Kidney involvement is the most important prognostic manifestation; it can manifest itself a few weeks after the acute phase of purpura. However, there is little data in Germany on the frequency of kidney involvement.
  • Testicles (rare): painfulness and swelling of the testicles. Henoch-Schönlein purpura is rarely accompanied by testicular torsion , which should then be ruled out quickly, if necessary with a trial exposure, in order to avoid permanent damage that occurs after four to ten hours.
  • Central nervous system (rare): Involvement occurs within two weeks of the onset of the disease and manifests itself in headache, seizures, hemiplegia, minor chorea , temporary blindness or blurred vision, aphasia , ataxia , mental disorders, and cerebral hemorrhage.

Duration

The appearance of the purpura is often preceded by uncharacteristic pain conditions and a slight fever. The acute phase lasts (3) - 12 - (60) days. Relapses are possible; one speaks of one after a> 4-week symptom-free interval. The disease is rarely chronic . It can also drag on for 2 years.

diagnosis

In the case of Henoch-Schönlein purpura, there are no conclusive laboratory parameters or similar, the diagnosis is made clinically, i.e. based on the symptoms. In the differential diagnosis , other possible diseases must first be ruled out: Depending on the symptoms, idiopathic thrombocytopenia , sepsis , leukemia , other vasculitis or hemolytic-uremic syndrome . According to the classification criteria of the American Rheumatism Society (ACR), the diagnosis can be made with a sensitivity of 87% and a specificity of 88% if two of the following four criteria are met:

  • palpable purpura , (see under symptoms).
  • Age of onset <20 years.
  • Angina abdominalis (the presence of diffuse abdominal pain and blood in the stool is often considered sufficient to meet this criterion).
  • Histological evidence of granulocytes in the vessel wall of arterioles or venules . (A skin biopsy is rarely required).

A modification of these criteria stipulates that at least one further criterion must be met in addition to the palpable purpura if the platelet count is normal . Although joint involvement or abdominal symptoms can precede the skin symptoms, a diagnosis is usually not possible without skin symptoms already present. In the laboratory tests, CRP and the sedimentation rate are usually only moderately increased, ANA are negative, as are autoantibodies against antigens of neutrophil granulocytes ( ANCA ) of the IgG class.

Further diagnosis is symptom-related and serves to identify complications; it may be carried out repeatedly if symptoms change, in uncomplicated cases it is carried out on an outpatient basis by the pediatrician and in complicated cases as an inpatient treatment in a children's clinic, possibly in consultation with the appropriate specialists:

  • Abdominal ultrasonography or x-ray to check for blood in your stool.
  • Urine examination to assess kidney involvement ( a kidney biopsy is often recommended for proteinuria significantly> 100 mg / gCrea).
  • Exclusion of brain involvement through an examination with magnetic resonance imaging .
  • Exclusion of testicular torsion by color-coded Doppler sonography .

treatment

There is no official therapy guideline for children. Bed rest or physical rest during an acute episode is advisable; Avoid tight clothing, local cooling for joint swelling.

Treatment is extended based on symptoms:

  • For severe joint pain: non-steroidal anti-inflammatory drugs ,
  • For abdominal involvement: oral steroids in a dosage of 1 to 2 mg methylprednisolone per kg body weight and day; steroid pulse therapy for severe forms. Ranitidine also shortens the duration and severity of abdominal symptoms.
  • With kidney involvement: The available studies do not allow a clear recommendation. Preventive steroid therapy may not have any effect on the longer prognosis. ACE inhibitors such as enalapril appear to have a protective effect on the kidneys in proteinuria. Even fish oil (2x1g) to improve proteinuria. In severe kidney involvement with nephrotic syndrome or depending on the biopsy findings, a combined immunosuppressive therapy with steroids and cyclophosphamide (e.g. 2 mg per kg body weight and day for a total of 2–3 months) or azathioprine is often used. Dialysis or kidney transplantation is rarely necessary.
  • For high blood pressure: antihypertensive therapy
  • If the brain is involved: steroid pulse therapy, possibly plasmapheresis or intravenous immunoglobulins .
  • In the case of intussusception: everting the intestinal wall with a sonographically controlled enema, possibly surgery
Henoch-Schönlein purpura of adulthood

In adults, however, the rate and severity of individual complications is significantly higher and is associated with a significant deterioration in the prognosis (Tancrede-Bohin 1997, Blanco 1997, Garcia-Porrua 2002). The frequency and severity of complications are also higher in Henoch-Schönlein purpura in adults than in non-IgA-associated leukocytoclastic vasculitis with systemic involvement (so-called hypersensitivity vasculitis) (Michel 1992). Arthritis of the knee and ankle joints is common (61%). If kidney involvement has already occurred initially, the chances of long-term remission are only 20% (Pillebout 2002). Almost 50% of adults suffer from abdominal complaints and intestinal bleeding in 25%, e.g. Sometimes with fatal outcome. Up to 25% of affected adults have to dialyze ten years after diagnosis.

literature

Individual evidence

  1. ↑ Cord Sunderkötter: Leukocytoclastic Vasculitis.
  2. ↑ Sebastian Stach: Therapy-dependent outcome of IgA nephropathy and Henoch-Schönlein purpura nephritis in childhood. Dissertation .