Objectives
Henoch–Schönlein purpura (HSP) is the most common vasculitis of children. The aim of this study is to evaluate the demographic and clinic findings of patients with HSP and also to ...determine predictive factors for assessing the development of gastrointestinal system (GIS) and renal involvement.
Methods
This study was performed prospectively among children with HSP who are under 18 years of age and being followed-up in the Pediatric Rheumatology Unit of Health Sciences University Kanuni Sultan Süleyman Training and Research Hospital between January 2016 and January 2018.
Results
A total of 265 patients, 137 boys (51.7%) and 128 girls (48.3%), were involved to the study. The mean ± standard deviation of age at the diagnosis was 7.5 ± 3.2. The most common disease onset season was spring (31.7%). The rate of arthritis, GIS involvement, and renal involvement were 54%, 51.3%, and 29.1%, respectively. GIS bleeding was more frequent in males than females (
p
= 0.007). Boys over 7 years of age had significantly more common GIS bleeding (
p
= 0.04). Intussusception, relapse, and serious GIS involvement requiring hospitalization and steroid treatment were highly associated with severe renal involvement.
Conclusions
We demonstrated that patients suffering intussusception, relapse, and serious GIS involvement or requiring hospitalization and steroid treatment had tendency to present with severe renal involvement. Therefore, these patients should be followed up carefully for not overlooking renal involvement of HSP.
Aim
Henoch‐Schönlein purpura (HSP), a primary vasculitis, characterized by purpura, abdominal pain, arthritis and renal involvement, is predominantly a disease of childhood. However, rarely it can ...occur in adults in whom it is believed to be a more severe form with poor renal outcomes. We aimed to answer if the age of onset affected the clinical spectrum and renal outcomes of the disease in a north Indian population. Hence, we studied the differences in clinical spectrum and renal outcomes between adult‐onset HSP and childhood‐onset HSP.
Methods
Case records of all adult patients diagnosed with HSP (onset ≥ 18 years) over the last 25 years in our department (1992–2017) were retrieved. Data on clinical features, lab abnormalities and outcomes were extracted and compared with that in pediatric HSP patients (onset < 18 years) seen during the same period.
Results
A total of 87 patients, including 30 adults and 57 children, were seen during this period. Compared to children, most of the adults had purpura as the first clinical manifestation (86.7% vs. 56.1%, P < 0.01) whereas abdominal pain was only rarely the initial symptom in adults (10.0% vs. 36.8%, P < 0.02). During the disease course, adults had a higher frequency of joint involvement as compared to children (90.0% vs. 43.9%, P < 0.001). There was no significant difference in the frequency of renal involvement (60.0% vs. 50.9%, P = NS) and gastrointestinal involvement (66.7% vs. 84.2%, P = NS) between adults and children. Outcomes were good in both groups and most adults and children achieved complete recovery (83.3% and 86.0%, respectively). There was no difference in the frequency of immunosuppressive treatment required by the two groups. None of the patients developed renal insufficiency on follow‐up.
Conclusion
Compared to children, adults with HSP seldom have abdominal pain as the first clinical manifestation while joint involvement is seen more commonly in them during the disease course.
The recurrence rate of Henoch-Schönlein purpura (HSP) is 2.7%-30%, with varied average intervals between the first and second episodes. Few studies have explored the incidence and risk factors for ...recurrent HSP.
We used a 16-year nationwide database to analyze the incidence of recurrent HSP. Patients with HSP were identified, and risk factors for recurrent HSP were explored. Kaplan-Meier and Cox regression model analyses were performed, and covariates were adjusted in the multivariate model.
From January 1, 1997 to December 31, 2012, among 2,886,836 individuals in the National Health Insurance Research Database, 1002 HSP patients aged < 18 years were identified. Among them, 164 had ≥2 HSP episodes (recurrence rate, 16.4%; incidence of recurrent HSP, 7.05 per 100 person-years); 83.6% patients with one HSP episode remained free of secondary HSP. The average time intervals between the first and second and second and third HSP episodes were 9.2 and 6.4 months, respectively. After adjusting for demographic parameters, comorbidities, and socioeconomic status, recurrent HSP was found to occur more frequently in patients who had renal involvement (adjusted hazard ratio, 2.41; 95% confidence interval CI, 1.64-3.54; p < 0.001), were receiving steroid therapy for > 10 days (adjusted hazard ratio, 8.13; 95%CI, 2.51-26.36; p < 0.001), and had allergic rhinitis (adjusted hazard ratio, 1.63; 95%CI, 1.06-2.50; p = 0.026).
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.
Abstract Henoch–Schönlein purpura (HSP) is a common childhood systemic vasculitis with clinical characteristics of cutaneous palpable purpura, arthralgia/arthritis, bowel angina, and ...hematuria/proteinuria. HSP is identified mainly based on the above presentations. Combined with pathohistological findings of leukocytoclastic vasculitis (LCV) and IgA-immune deposits in vessel walls and/or glomeruli increase the diagnostic sensitivity and specificity. However, considering the accessibility of biopsy and some patients with atypical presentations, there are still medical unmet needs in HSP diagnosis. This article reviews the diagnosis of HSP including the aspects of classification criteria, differential diagnosis, and some laboratory findings as the biomarkers with diagnostic potential.
Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting ...factors related to outcome among a large cohort of pediatric HSP patients.
We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses.
The study included 420 HSP patients, of which the mean age at diagnosis was 7.68 ± 3.15 years. Clinical manifestations were arthralgia and/or arthritis (
= 244, 58.1%), abdominal pain (
= 235, 56%), subcutaneous edema (
= 163, 38.8%), and renal involvement (
= 125, 29.8%). Disease recurred for at least once, in 69 (16.4%) patients and colchicine treatment yielded a favorable response in 11 of 12 relapsing patients, who did not respond to ibuprofen or steroids. Frequencies of renal involvement and biopsy-proven nephritis were higher in patients with severe GI involvement. Besides, patients with biopsy-proven nephritis had higher rates of abdominal pain, intussusception, severe GI involvement, and systemic steroid administration.
We speculate that renal involvement, biopsy-proven nephritis and severe GI involvement can be related to each other. Colchicine may be effective in patients with relapsing disease.
Background
Henoch–Schönlein purpura (HSP) mainly affects children, but age is also thought to be an important prognostic factor. Kidney involvement is a major cause of mortality in HSP patients. The ...purpose of this study was to analyze the clinicopathological correlations between adults and children.
Methods
A total of 208 children and 75 adult patients with HSP nephritis (HSPN) were evaluated. All patients underwent a renal biopsy.
Results
Extra-renal symptoms (arthritis and abdominal pain) were more common in the pediatric patient group than in the adult group (
P
< 0.05), but renal symptoms (edema and hypertension) were relatively rare (
P
< 0.05). A significant positive correlation was noted between pathological type and clinical type (
P
< 0.01). Pathological activity was positively related to renal failure, abdominal pain, microscopic hematuria, hypertension, and proteinuria (
P
< 0.05). Pathological chronicity was positively associated with age, duration of follow-up since the onset of palpable purpura, renal failure, lower extremity edema, hypertension, and proteinuria (
P
< 0.05).
Conclusions
Various clinicopathological differences exist between children and adults with HSPN. Massive proteinuria, renal failure, and abdominal pain usually correlated with severe pathology. Renal biopsy should be performed in both pediatric and adult HSPN patients with repeated hematuria and/or consistent minimal proteinuria.
Chronic kidney diseases have become a major issue worldwide. The spectrum of biopsy proven renal diseases differs between locations and changes over time. It is therefore essential to describe the ...local epidemiological trends and the prevalence of renal biopsy in various regions to shine new light on the pathogenesis of various renal diseases and provide a basis for further hypothesis-driven research. We retrospectively analyzed 34,630 hospitalized patients undergoing native renal biopsy between January 1, 2009 and December 31, 2018. Indications for renal biopsy and histological diagnosis were analyzed to describe the prevalence of renal biopsy, and changing prevalence between period 1 (2009-2013) and period 2 (2014-2018) were further analyzed. Nephrotic syndrome (NS) was the most common indication for biopsy. Membranous nephropathy (MN, 24.96%) and IgA nephropathy (IgAN, 24.09%) were the most common primary glomerulonephritis (PGN). MN was most common in adults, with IgAN more prevalent in children. Lupus nephritis (LN) was the most common secondary glomerulonephritis (SGN) in adults, while Henöch-Schönlein purpura nephritis (HSPN) in children. The prevalence of MN increased significantly and nearly doubled from period 1 (15.98%) to period 2 (30.81%) (P = 0.0004). The same trend appeared with membranoproliferative glomerulonephritis (MPGN), diabetic nephropathy (DN) and obesity-related glomerulopathy (ORG), while the frequencies of minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), LN and hepatitis B associated glomerulonephritis (HBV-GN) significantly decreased between the two intervals. NS was the most common indication for biopsy across all age groups and genders. MN has overtaken IgAN to become the most common PGN in adults, while IgAN was the most common PGN in children. LN was the most common SGN in adults, and HSPN the most common in children.
Background: The duration of follow up to assess the risk of long term renal impairment in Henoch-Schönlein purpura (HSP) without nephritic or nephrotic syndrome or renal failure on diagnosis remains ...undetermined. Aims: To undertake a systematic review of the literature to assess whether the risk of long term renal impairment without renal involvement on diagnosis could be estimated and to determine the time period when renal involvement is very unlikely after the diagnosis of HSP. Methods: Search of studies of unselected children with HSP, and available information on urinary findings, renal involvement, and long term renal function follow up. Studies of selected children with HSP nephropathy at diagnosis were excluded. Results: Twelve studies of 1133 children were reviewed. The follow up period ranged from 6 weeks to 36 years. Proteinuria and/or haematuria, which occurred in 34.2%, of which only one fifth were in association with nephritic or nephrotic syndrome, developed in 85% of cases within 4 weeks of the diagnosis of HSP, in 91% within 6 weeks, and in 97% within 6 months. Permanent renal impairment never developed after normal urinalysis; it occurred in 1.6% of those with isolated urinary abnormalities, and in 19.5% of those who developed nephritic or nephrotic syndrome. Conclusion: No long term renal impairment occurred after normal urinalysis. Even if urinalysis is normal at presentation, the testing should be continued for six months. There is no need to follow up after the first six months those whose urinalysis remains normal.
No consensus exists among general pediatricians or pediatric rheumatologists regarding whether corticosteroid therapy ameliorates the acute manifestations of Henoch-Schönlein purpura or mitigates ...renal injury. Therefore, we sought to synthesize the reported experimental and observational data regarding corticosteroid use.
We performed a meta-analysis based on a comprehensive review of the literature in the Medline database (1956 to January 2007) and the Cochrane Controlled Trials Register. On the basis of reported outcomes among patients with Henoch-Schönlein purpura who were treated at diagnosis with corticosteroids compared with patients treated with supportive care only, we calculated odds ratios for the resolution of abdominal pain, the need for surgical intervention secondary to severe pain or intussusception, the likelihood of Henoch-Schönlein purpura recurrence, and the development of transient or persistent renal disease.
Of 201 articles retrieved from the initial literature search, 15 were eligible for inclusion. Corticosteroid treatment did not reduce the median time to resolution of abdominal pain but did significantly reduce the mean resolution time and increased the odds of resolution within 24 hours. Early corticosteroid treatment significantly reduced the odds of developing persistent renal disease. In addition, although the results were not statistically significant, the prospective data suggest reduced odds of both surgical intervention and recurrence.
Corticosteroids, given early in the course of illness, seem to produce consistent benefits for several major clinically relevant Henoch-Schönlein purpura outcomes.
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