Materiały źródłowe

• #1 Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP) – PubMed

  https://pubmed.ncbi.nlm.nih.gov/26258874/

  Henoch-Schnlein purpura (HSP) is the most common vasculitis of childhood but may occur in adults. This small vessel vasculitis is characterised by palpable purpura, abdominal pain, arthritis or arthralgia and kidney involvement. […] To evaluate the benefits and harms of different agents (used singularly or in combination) compared with placebo, no treatment or any other agent for: (1) the prevention of severe kidney disease in patients with HSP without kidney disease at presentation; (2) the prevention of severe kidney disease in patients with HSP and minor kidney disease (microscopic haematuria, mild proteinuria) at presentation; (3) the treatment of established severe kidney disease (macroscopic haematuria, proteinuria, nephritic syndrome, nephrotic syndrome with or without acute kidney failure) in HSP; and (4) the prevention of recurrent episodes of HSP-associated kidney disease.

• #2 Henoch-Schönlein Purpura (HSP): Symptoms and Treatment

  https://patient.info/allergies-blood-immune/henoch-schonlein-purpura-leaflet

  Henoch-Schönlein purpura (HSP) is a rare condition that is caused by inflammation of blood vessels (vasculitis). It particularly affects children. Blood vessels throughout the body are affected but HSP most often causes a skin rash, tummy (abdominal) pain and joint pains. […] Depending on the symptoms that develop, someone with Henoch-Schönlein purpura (HSP) may be admitted to hospital for monitoring. For most people, HSP will get better on its own and so no specific treatment is needed. However, there are a number of things that can help with the symptoms. For example: […] Steroid medication may be suggested if there are signs that the kidneys are becoming affected. Sometimes steroids are also suggested if other symptoms are severe (such as joint pains or tummy (abdominal) pain), or if boys develop scrotal pain and swelling.

• #3

  https://www.singhealth.com.sg/patient-care/conditions-treatments/henoch-schonlein-purpura-hsp

  There is nothing that parents can do to prevent their children from getting HSP.

• #4 Vasculitis in Children (Henoch-Schönlein Purpura): Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/21086-vasculitis-in-children

  No, theres no known way to prevent IgA vasculitis. […] While IgAV is generally a rather mild illness that goes away on its own, it can also cause serious complications in the kidneys and bowels. Some children may need to take immunosuppressant medications to prevent these complications.

• #5 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  Early oral prednisone treatment at 1 to 2 mg per kg daily for two weeks reduces the intensity of joint and abdominal pain from Henoch-Schönlein purpura in children. […] Early prednisone treatment for Henoch-Schönlein purpura does not prevent renal disease, although it is effective in treating renal involvement and reduces the odds of developing persistent renal disease in children. […] Early prednisone treatment reduces the mean resolution time of abdominal pain from Henoch-Schönlein purpura in children. […] In patients with a normal initial urinalysis or with isolated hematuria, a monthly urinalysis should be continued for six months to detect signs of renal involvement. […] Because Henoch-Schönlein purpura spontaneously resolves in 94 percent of children and 89 percent of adults, supportive treatment is the primary intervention.

• #6 Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP) – PubMed

  https://pubmed.ncbi.nlm.nih.gov/26258874/

  Eight studies evaluated therapy to prevent persistent kidney disease in HSP. There was no significant difference in the risk of persistent kidney disease any time after treatment (5 studies, 746 children: RR 0.74, 95% CI 0.42 to 1.32), or at one, three, six and 12 months in children given prednisone for 14 to 28 days at presentation of HSP compared with placebo or supportive treatment. […] From generally low quality evidence, we found no evidence of benefit from RCTs for the use of prednisone or antiplatelet agents to prevent persistent kidney disease in children with HSP. Though heparin appeared effective, this potentially dangerous therapy is not justified to prevent serious kidney disease when fewer than 2% of children with HSP develop severe kidney disease. […] No evidence of benefit has been found for cyclophosphamide treatment in children or adults with HSP and severe kidney disease.

• #7 Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p229.html

  Systematic reviews have shown that steroids do not prevent complications and should not be used prophylactically. […] Do not prescribe prednisone or antiplatelet agents to prevent renal disease. […] A 2015 Cochrane review also concluded that steroids and cyclophosphamide do not help prevent renal disease. […] Randomized trials have shown that joint and abdominal symptoms resolve 1.2 days faster in children who receive prednisone, 1 to 2 mg per kg. […] Use steroids only in patients with severe pain who do not improve with supportive care and NSAIDs.

• #8 Henoch-Schönlein purpura in children: Use of corticosteroids for prevention and treatment of renal disease – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33334956/

  Henoch-Schnlein purpura is self-limiting in 94% of children, but permanent renal injury is reported in one-fifth of children with nephritic or nephrotic features. Corticosteroids have been considered as candidates for preventing and treating renal involvement in HSP. There is a moderate level of evidence to suggest corticosteroids are not effective in preventing renal involvement in HSP. […] However, based on low-level evidence and similarities with primary immunoglobulin A nephropathy, experts recommend corticosteroids in treating renal involvement in HSP to prevent long-term kidney injury. Dose and duration of therapy should be carefully considered in consultation with a pediatric nephrologist.

• #9 Management of IgA vasculitis nephritis (Henoch-Schonlein purpura nephritis) in Children

  https://www.chikd.org/journal/view.php?year=2020&vol=24&spage=1

  Recent studies have failed to prove the efficacy of early glucocorticoid administration to avoid renal injury in these patients. No trial identified in updated literature reviews supports the use of drugs to prevent nephritis in all children with IgAV. […] The prognosis of IgAVN is better in children than in adults. […] The aforementioned cohort studies indicated that pediatric IgAVN is no longer considered a mild disease. Proteinuria and hematuria may be associated with a 15% risk and a combination of nephritic-nephrotic syndrome may be associated with a 50% risk of progression to ESKD. These long-term studies highlight the importance of long-term follow-up in all patients during adulthood. Notably, all women with a history of IgAVN require close observation during and after pregnancy.

• #10 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  Hospitalization may be required when adequate outpatient monitoring is unavailable or if dehydration, hemorrhage, or pain control require inpatient management. […] Early steroid treatment is most appropriate for children with renal involvement or severe extrarenal symptoms. […] A meta-analysis found that corticosteroid use in children with Henoch-Schönlein purpura reduced the mean time to resolution of abdominal pain and decreased the odds of developing persistent renal disease. […] Early aggressive therapy is recommended for children and adults with severe renal involvement. […] A blood pressure measurement and urinalysis should be performed at the time Henoch-Schönlein purpura is diagnosed and at each return physician office visit. […] If the initial urinalysis is normal, or if there is isolated hematuria (without nephritic or nephrotic syndrome), a monthly urinalysis should be performed for the first six months after the diagnosis of Henoch-Schönlein purpura.

• #11 Henoch-Schönlein Purpura (HSP): Symptoms and Treatment

  https://patient.info/allergies-blood-immune/henoch-schonlein-purpura-leaflet

  In addition to this, if something is thought to have triggered HSP (for example, a specific medication that was being taken), this should be stopped. […] Other treatment will depend on whether complications develop. For example, if the kidneys become involved, referral to a kidney specialist for assessment and their advice about treatment may be advised. A kidney sample (biopsy) may be suggested to help give the specialist more information and guide them as to the best treatment. Treatment may include steroids and other medicines to help suppress the body’s defence (immune) system. Sometimes medication to lower blood pressure is needed if high blood pressure develops because of kidney involvement. […] Occasionally a process called plasma exchange is needed if the complications caused by HSP are very serious.

• #12 Management of IgA vasculitis nephritis (Henoch-Schonlein purpura nephritis) in Children

  https://www.chikd.org/journal/view.php?year=2020&vol=24&spage=1

  The Kidney Disease: Improving Global Outcomes (KDIGO) group systematically reviewed the available literature and reported that compared with no therapy or supportive therapy, early treatment with corticosteroids (CS) did not significantly contribute or contributed only minimally to severe kidney disease (nephrotic-range proteinuria, hypertension, or reduced kidney function). […] A study investigating adults with IgAVN reported that greater crescent deposition was associated with more severe renal manifestations and a poorer treatment response; however, whether the percentage of crescent deposits is associated with a higher risk of late renal insufficiency remains controversial. […] Institution of treatment only after renal biopsy leads to delayed management. Therefore, establishing less invasive and user-friendly laboratory tests are essential. Studies have investigated the role of genes, as well as blood and urinary biomarkers as predictors of progression of IgAVN to severe renal dysfunction.

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