Materiały źródłowe

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

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

  IgA vasculitis spontaneously resolves in 94% of children and 89% of adults, making supportive treatment the primary management strategy. […] Long-term prognosis depends on the extent of renal involvement. Six months of follow-up is prudent to assess for disease relapse or remission. […] Relapses most commonly involve the skin but can also involve the joints, kidneys, and gastrointestinal system; occur in 2% to 30% of children; and may occur up to 10 years later. […] Gastrointestinal symptoms at diagnosis are the best predictor of relapse in adults. […] Abnormal urinalysis in children on the day of diagnosis is predictive of severe renal involvement later. […] No renal involvement in the first six months predicts a low likelihood of chronic disease; 91% of renal involvement occurs within six weeks and 97% within six months.

• #2 Henoch–Schönlein purpura – Wikipedia

  https://en.wikipedia.org/wiki/Henoch%E2%80%93Sch%C3%B6nlein_purpura

  Overall prognosis is good in most patients, with one study showing recovery occurring in 94% and 89% of children and adults, respectively (some having needed treatment). […] In children under ten, the condition recurs in about a third of all cases, usually within the four months of the initial attack. Recurrence is more common in older children and adults. […] In adults, kidney involvement progresses to end-stage kidney disease (ESKD) more often than in children. In a UK series of 37 patients, 10 (27%) developed advanced kidney disease. Proteinuria, hypertension at presentation, and pathology features (crescentic changes, interstitial fibrosis and tubular atrophy) predicted progression. […] About 20% of children that exhibit nephrotic or nephritic features experience long permanent renal impairment.

• #3 Henoch-Schönlein Purpura in adults: outcome and prognostic factors – PubMed

  https://pubmed.ncbi.nlm.nih.gov/11961015/

  Henoch-Schnlein Purpura nephritis (HSPN) has been extensively studied in children but, its natural history in adults is much less known. […] At the end of follow-up, patient survival was only 74%. […] The data indicate that clinical presentation of HSPN in adults is severe and its outcome relatively poor, worse than in children. […] Identification of clinical and histologic prognostic factors may permit the design of appropriate therapeutic prospective studies.

• #4 Renal Prognosis and Related Risk Factors for Henoch-Schönlein Purpura Nephritis: A Chinese Adult Patient Cohort | Scientific Reports

  https://www.nature.com/articles/s41598-018-23638-2

  This study investigated the clinicopathological characteristics of Henoch-Schnlein purpura nephritis (HSPN) in Chinese adult patients and analyzed the renal outcomes and prognostic risk factors for progression to end-stage renal disease (ESRD). […] During a median follow-up of 54.0 months, 32 patients (4.6%) progressed to ESRD. The 5- and 10-year cumulative renal survival rates from ESRD were 96.4% and 88.6%, respectively. […] Baseline urinary protein, renal insufficiency, glomerular sclerosis and tubular atrophy/interstitial fibrosis were independent predictors of renal outcomes. […] The patients with a time-average proteinuria 0.4g/day had the lowest rates of ESRD or a 50% decline in renal function. […] The optimal goal of therapy for HSPN patients may be to lower proteinuria to 0.4g/day and control hypertension to achieve an ideal renal outcome.

• #5 Renal Prognosis and Related Risk Factors for Henoch-Schönlein Purpura Nephritis: A Chinese Adult Patient Cohort | Scientific Reports

  https://www.nature.com/articles/s41598-018-23638-2

  Hypertension, urinary protein 1.0g/day, an eGFR 60mL/min/1.73m2, and hypoproteinemia predicted an increased risk of ESRD in the univariate analysis. […] This model confirmed that urinary protein 1.0g/day [hazard ratio (HR) 2.486, P=0.031], an eGFR 60mL/min/1.73m2 (HR 5.344, P0.001), glomerular sclerosis 10% (HR 2.397, P=0.044) and moderate and severe tubular atrophy/interstitial fibrosis (HR 5.239, P0.001) were independent predictors of renal outcomes. […] The multivariate Cox analysis also revealed that interval increase of 100mg/day in TA-P increased the risk of ESRD by 1.082 (P0.001). […] The optimal goal of anti-proteinuric therapy for Chinese adult HSPN patients is to lower proteinuria 0.4g/day during follow-up. […] In conclusion, identifying of clinical and histological prognostic factors may permit the prediction of renal outcomes. The optimal goal of therapy for patients may be to lower proteinuria to 0.4g/day and to control hypertension to predict a good renal prognosis for HSPN patients.

• #6 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/984105-overview

  IgAV is generally a benign disease with an excellent prognosis. Spontaneous resolution is usual: Most patients experience complete resolution of symptoms within 8 weeks, and probably fewer than 5% experience chronic symptoms. However, initial attacks of IgAV can last several months, and relapses are possible. IgAV is fatal only in the rarest of cases. […] A clinical course with complete resolution of the disease usually occurs in patients with the following: mild kidney involvement, no neurologic complications, disease that lasts less than 4-6 weeks initially. […] Recurrences occur in as many as 50% of patients within 6 weeks but can happen as late as 7 years after the initial disease. […] Although IgAV generally resolves without permanent consequences, serious GI and renal complications may occur, and the higher the number of recurrences, the higher the likelihood of permanent kidney damage.

• #7 Henoch-Schönlein Purpura | AAFP

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

  Henoch-Schönlein purpura is a self-limited illness that demonstrates no clinical sequelae in most patients without renal involvement. Most patients recover fully within four weeks. […] Long-term prognosis depends on the severity of renal involvement; end-stage renal disease occurs in 1 to 5 percent of patients. One systematic review revealed that the onset of renal disease in patients with Henoch-Schönlein purpura developed within four weeks in 85 percent of patients, six weeks in 91 percent of patients, and six months in 97 percent of patients. […] A blood pressure measurement and urinalysis should be performed at the time Henoch-Schönlein purpura is diagnosed and at each return physician office visit. Serum blood urea nitrogen and creatinine determination are needed if hematuria or proteinuria are identified. 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. This will detect the first signs of renal involvement, and facilitate early nephrology consultation and the initiation of steroids.

• #8 Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-018-0247-8

  The recurrence rate of Henoch-Schnlein purpura (HSP) is 2.7%30%, with varied average intervals between the first and second episodes. […] The prognosis of HSP is generally good, but recurrence is common among children (recurrence rate, 2.7%66.2%). […] Various predictors for recurrence, including greater joint and gastrointestinal involvement at diagnosis, history of infection, elevated erythrocyte sedimentation rate, steroid treatment, and renal manifestations, have been identified but they are inconsistent. […] The annual incidence of recurrent HSP was low. However, children who had underlying allergic rhinitis, presented with renal involvement, and received steroid treatment for 10 days should be notified regarding the possibility of recurrence. […] Our results revealed that renal involvement, underlying allergic rhinitis, and steroid treatment for 10 days were risk factors for HSP recurrence in children, regardless of age, sex, and income levels.

• #9 Henoch-Schonlein Purpura | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/henoch-schonlein-purpura

  Henoch-Schönlein purpura (HSP) is a form of vasculitis, a condition that involves inflammation of the blood vessels. It can affect any organ of the body. […] Most children with Henoch-Schönlein purpura will recover completely without long-term consequences. […] About a third of children with HSP will experience a recurrence of symptoms within the first year after disease onset. In most cases, the recurrence is less severe and lasts a shorter period of time than the initial onset of symptoms. […] In rare cases, HSP can lead to permanent damage to the kidneys, which may require life-long management by a nephrologist.

• #10 Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-018-0247-8

  Our finding that allergic rhinitis was an independent risk factor for HSP was in line with studies reporting an increased risk of HSP in atopic children. […] HSP patients with underlying allergic rhinitis may be more susceptible to chronic inflammatory status leading to a more intense immune response to causative antigens, thereby predisposing the recurrence of HSP. […] Our study showed a significantly increased association between steroid use for 10 days regardless of early initiation and the risk of recurrence. […] The duration of follow-up in children with HSP for the early identification of possible recurrence is an important issue. […] For the early detection of recurrence, it may be worth considering following up patients with the first HSP episode for at least 9.2 months and those with a second HSP episode for another 6.4 months.

• #11 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/984105-overview

  Kidney damage related to IgAV is the primary cause of morbidity and mortality. As many as 15% of patients may have long-term kidney insufficiency, but no more than 1-2% will have ESKD. […] Predictors of serious nephropathy or end-stage kidney disease (ESKD) include bloody stools and persistent rash. […] The long-term prognosis for patients with IgAV nephritis is determined by the development of CKD, which sometimes is difficult to predict from the initial clinical and histologic presentation. CKD can develop long-term even after apparent complete recovery from IgAV nephritis.

• #12 Henoch Schonlein Purpura – A 5-Year Review and Proposed Pathway

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3250434/

  Henoch Schonlein Purpura (HSP) is the commonest systemic vasculitis of childhood typically presenting with a palpable purpuric rash and frequently involving the renal system. […] Generally the prognosis is good, with the exception of those with significant renal involvement. […] The principal aim of HSP follow up is detecting persistent renal inflammation, which undiagnosed, could progress to permanent renal damage. […] The long term risk of permanent renal impairment in patients with minor urine abnormalities is low (1.6% reported previously). However, this rises to 19.5% in children with nephrotic or nephritic features. […] A meta-analysis on the use of early corticosteroids in this condition suggested some effect on the renal outcome and immunosuppressive treatments are used in severe cases, as they are believed to preserve renal function, despite a paucity of randomised controlled trials.

• #13 Henoch Schonlein Purpura – A 5-Year Review and Proposed Pathway

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3250434/

  Our data, together with other studies, demonstrates an overall low risk of HSP associated nephritis in an unselected cohort of patients presenting with features of HSP, 91% had a self-limiting disease course in our series. […] There have now been several cohorts describing this, together with the finding that patients with HSP who present with or develop proteinuria are at the greatest risk of renal complications. […] The revised pathway ensures patients with a normal urinalysis receive a less intensive follow up, but with a safeguard to convert onto the more intensive monitoring if proteinuria develops. […] The presence of proteinuria is a very important exit criterion as it is the marker most commonly correlated with the renal prognosis. […] Reduced glomerular filtration rate is linked to a worse long-term renal prognosis. […] In summary, unlike all other complications of HSP, nephritis is typically asymptomatic and requires active screening.

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

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

  Nephritis at disease onset raises the risk of hypertension or urine abnormalities for up to eight years, and the risk of proteinuria for five years in children. […] Any baseline renal disease before IgA nephritis increases the risk of progression to end-stage renal disease; however, less is known about the predictors of renal progression in adults. Adults have an 11% risk of end-stage renal disease and a 13% risk of severe renal failure.

• #15 Determination of Risk Factors in Children Diagnosed With Henoch-Schönlein Purpura | Volume 33 – Issue 4 – December 2018 | Archives of Rheumatology

  https://archivesofrheumatology.org/full-text/949

  Logistic regression analysis revealed that the risk of gastrointestinal system involvement was significantly higher in patients with skin lesions in the upper extremities (p=0.002, odds ratio [OR]=3.2). […] The risk of joint involvement was significantly higher in girls (p=0.024, OR=2.18), in patients with soft tissue swelling (p=0.005, OR=2.63), and with low mean platelet volume levels (p=0.008, OR=4.07). […] The risk of renal involvement was significantly higher in girls (p=0.047, OR=2.7), in patients 10 years (p=0.001, OR=1.4), and in patients with elevated C-reactive protein levels (p=0.007, OR=6.57). […] Renal involvement in Henoch-Schnlein purpura is independent of gastrointestinal system or joint involvement and very close follow-up is required in the acute period particularly in girls 10 years with high C-reactive protein levels.

• #16 Determination of Risk Factors in Children Diagnosed With Henoch-Schönlein Purpura | Volume 33 – Issue 4 – December 2018 | Archives of Rheumatology

  https://archivesofrheumatology.org/full-text/949

  In conclusion, our study did not demonstrate any relationship between GIS involvement and low MPV levels as mentioned before; however, skin lesions on upper extremities were found to increase the risk of GIS involvement. In addition, low MPV levels, female sex, and soft tissue edema were found to increase the risk for joint involvement. Only female sex, being 10 years of age, and high CRP levels were associated with renal involvement and most of our patients had renal problems within the first three months, particularly in the first month. We may conclude that the renal involvement in HSP is independent of GIS or joint involvement and very close follow-up is required in the acute period particularly in girls 10 years with high CRP levels.

• #17 Henoch–Schönlein purpura – Wikipedia

  https://en.wikipedia.org/wiki/Henoch%E2%80%93Sch%C3%B6nlein_purpura

  The findings on renal biopsy correlate with the severity of symptoms: those with asymptomatic hematuria may only have focal mesangial proliferation while those with proteinuria may have marked cellular proliferation or even crescent formation. The number of crescentic glomeruli is an important prognostic factor in determining whether the patient will develop chronic renal disease. […] In ESKD, some eventually need hemodialysis or equivalent renal replacement therapy (RRT). If a kidney transplant is found for a patient on RRT, the disease will recur in the graft (transplanted kidney) in about 35% of cases, and in 11%, the graft will fail completely (requiring resumption of the RRT and a further transplant).

• #18 Henoch Schonlein Purpura – A 5-Year Review and Proposed Pathway | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0029512

  A stratified approach will allow focus on these individuals. […] The revised pathway ensures patients with a normal urinalysis receive a less intensive follow up, but with a safeguard to convert onto the more intensive monitoring if proteinuria develops. […] The presence of proteinuria is a very important exit criterion as it is the marker most commonly correlated with the renal prognosis. […] The defined cut off values have been based on previous cohort data which identified patients with moderate to severe proteinuria at greater risk of poor renal outcome. […] In summary, unlike all other complications of HSP, nephritis is typically asymptomatic and requires active screening. This study provides important follow up data on the natural course of HSP associated nephritis.

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