Henoch-Schönlein Purpura - Een Diepgaande Gids

Henoch Schonlein Purpura (IgA Vasculitis)

Henoch-Schönlein purpura (HSP) is a disease that inflames small blood vessels. The inflammation causes blood vessels in the skin, intestines, kidneys, and joints to start leaking. The main symptom is a raised rash with many small bruises on your legs or buttocks.

HSP got its name from two doctors, Eduard Henoch and Johann Lukas Schönlein, who researched this condition in the late 1800s. In 2012, a group of scientists renamed the disease immunoglobulin A (IgA) vasculitis because of the discovery that the antibody IgA causes small blood vessels to leak.

Although people of any age can get HSP, the disease most often affects children ages 3 to 10. It is more common in boys than girls. Adults with HSP tend to have more severe disease than children do.

Usually, IgA vasculitis goes away on its own after 4 weeks. Within 6 months, the disease comes back in about 1/3 of people, but it usually doesn’t cause long-term problems. Rarely, it causes kidney disease. It’s important to have regular follow-up visits with a doctor if you or your child has IgA vasculitis to prevent serious complications.

Het syndroom van Henoch-Schönlein / Een allergische ontsteking van bloedvaatjes

dossier Het syndroom van Henoch-Schönlein, ook Henoch-Schönlein purpura (HSP) genoemd, is een vrij zeldzame vorm van bloedvatontsteking (vasculitis) die veroorzaakt wordt door een verstoorde werking van het afweersysteem.

Het gaat om een ontsteking van de kleine bloedvaatjes (vasculitis) in de huid, de nieren, de darm… Deze bloedvaatjes laten daardoor veel vocht en eiwitten door. Die ontstekingsreactie wordt uitgelokt door lichaamseigen antistoffen van het IgA type die zich aan de vaatwand hechten.

Bij het syndroom van Henoch-Schönlein hebt u kleine bloeduitstortingen (purpura) op de onderste ledematen. Bijbehorende klachten zijn buikpijn, pijnlijke en dikke gewrichten en nierfunctiestoornissen.

De ziekte is vrij zeldzaam en komt vooral bij kinderen voor tussen de leeftijd van 4 en 11 jaar. De piekleeftijd van ontstaan is 5 jaar. Op kinderleeftijd zijn er twee keer zo veel jongens dan meisjes aangedaan. Op volwassen leeftijd krijgen vrouwen echter vaker de aandoening.

Complications

IgAV can involve nearly every organ system. Reported complications include the following:

Hematuria, usually microscopic, can be accompanied by mild-to-moderate proteinuria ( < 2 g/day). Oliguria, hypertension, and azotemia are rarely present. Nephrotic syndrome (urinary protein excretion >40 mg/m 2 /hr or protein:creatinine ratio ≥200 mg/mmol in an early morning spot urine) can also occur. In most cases, histologic examination of the kidneys reveals mesangial proliferation that can be diffuse or focal and segmental. Resolution of the kidney involvement is the focus in these patients.

A study reported that even patients with mild forms of IgAV nephritis are at risk for significant long-term proteinuria. The study also added that very early introduction of angiotensin-converting enzyme inhibitors/angiotensin receptor blockers may improve the long-term outcome independent of histological lesions. [111]

GI complications include hydrops of the gallbladder, pancreatitis, and GI bleeding. Surgical complications include intussusception, bowel infarction, and perforation.

Overall, 5% of patients develop ESKD. Urinary complications include bladder-wall hematoma, calcified ureter, hydronephrosis, and urethritis.

A retrospective study by Lee et al of of 212 pediatric patients with IgAV admitted to a tertiary care hospital in Korea found that the incidence of kidney involvement and nephrotic syndrome was significantly higher in patients with severe GI symptoms and those over age 7 years. [112]

References

Purpuric papules and plaques of the lower extremity characteristic of IgA vasculitis (Henoch-Schönlein purpura).

Hemorrhagic macules, papules, and patches on the ankle and foot of a child with IgA vasculitis (Henoch-Schönlein purpura).

Older lesions of IgA vasculitis (Henoch-Schönlein purpura) demonstrating increased extravasation with ecchymoses on dorsal foot and ankle.

Henoch-Schönlein Purpura Causes

Doctors don’t know exactly what causes HSP. They think it’s an autoimmune disease, meaning the body attacks itself. IgA is an antibody that normally fights infections. In HSP, it causes the blood vessels to become inflamed.

An abnormal immune response to an infection may be a factor in many cases. In 3 out of 4 people with IgA vasculitis, symptoms appear after an upper respiratory tract infection like a cold or the flu. It also affects children who’ve had infections like Epstein-Barr and chickenpox. Certain genes may increase the risk.

Other possible causes include:

• Reactions to certain foods
• Insect bites
• Medicines
• Certain vaccines
• Exposure to cold weather

HSP and COVID-19

Viruses are a likely cause of HSP. Often this condition appears after a virus like group A strep. A few people have developed HSP after a COVID infection or the COVID-19 vaccine.

Researchers don’t know the exact link between IgA vasculitis and COVID. It’s possible that the COVID virus directly damages blood vessels. Or the immune system might overreact to the virus and inflame the blood vessels. Getting IgA vasculitis after a COVID infection or vaccine is very rare, and scientists still need to learn more about the possible connection.

Clinical Presentation

The onset of purpura, abdominal pain, and arthritis may be in any sequence, although abdominal pain and arthritis are not universally present. The timing of symptoms may be within days or insidious over a period of weeks. 5 Henoch-Schönlein purpura usually follows an upper respiratory infection. 11 , 16 Fatigue and low-grade fever are also common.

A nonmigratory arthritis occurs in 75 percent of patients with Henoch-Schönlein purpura. 13 , 16 The knees and ankles are more commonly involved than small joints. The arthritis symptoms include swelling, warmth, and tenderness. The symptoms are transient, leave no deformity, and may precede the purpuric rash in 15 to 25 percent of patients. 2 , 3

Renal disease is the most serious sequela of Henoch-Schönlein purpura, occurring in 40 to 50 percent of patients. 18 Although death from Henoch-Schönlein purpura is rare, renal disease is the leading cause of death in these patients. 18 Risk of renal disease is greatest in persons older than 10 years with persistent purpura, severe abdominal pain, or relapsing episodes. 8 , 19 Unlike abdominal pain or arthritis that may precede the rash, renal disease is a late sequela. It usually starts within the first month and rarely occurs more than six months after the illness begins. 16 Signs of Henoch-Schönlein purpura-associated renal disease are microscopic hematuria, red cell casts, and proteinuria. Renal disease will spontaneously remit in most patients. However, progressive glomerulonephritis may develop, patients with persistent proteinuria are at the highest risk of this complication. 18