Henoch-Schönlein Purpura Children Research Articles

Alteration of gut microbiota in Henoch-Schönlein purpura children with gastrointestinal involvement.

The compositional and structural changes of gut microbiota were closely related to the status of Henoch-Schönlein purpura (HSP). To investigate if clinical indicators and gut microbiota differ between HSP patients with or without gastrointestinal (GI) involvement and to explore the alterations of fecal microbiota in HSP children with and without GI symptoms. A total of 22 children with HSP were enrolled in the study. Fecal microbiota composition was analyzed by 16S rRNA sequencing. Clinical indicators, fecal microbial diversity, and compositions were compared between the two groups. Respectively, 9 patients with GI involvement (HSP-A) and 13 patients without GI involvement (HSP-N) were enrolled. Prealbumin (PA) and the ratio of immunoglobulin A (IgA) / complement (C)3 were significantly decreased in the HSP-A group and an elevated D-dimer was found in the HSP-N group. The relative abundances of Blautia, Lachnospira, and Haemophilus were significantly higher in the HSP-A group compared to HSP-N. Lower levels of unidentified Prevotellaceae, Parabacteroides, and Romboutsia were found in HSP-A patients. The linear discriminant analysis effect size (LEfSe) showed that the biomarkers for the HSP-A group included Blautia, Anaerostipes, Veillonella, Lachnospira, and Haemophilus. For the HSP-N group, unidentified Prevotellaceae, Intestinibacter, Romboutsia, and Akkermansia were the prominent biomarkers at the genus level. Additionally, the ratio of IgA/C3 exhibited a negative correlation with the genus Blautia. Meanwhile, PA showed negatively correlation with Veillonella. These results provide a broader understanding for future microbial-based therapies to decrease the development of GI involvement and improve the clinical outcome of HSP in children.

Gut Microbiota Dysbiosis in Childhood Vasculitis: A Perspective Comparative Pilot Study.

Kawasaki disease (KD) and Henoch–Schönlein purpura (HSP) are the most frequent vasculitis in childhood. For both, a multifactorial mechanism has been hypothesised, with an abnormal immune response in genetically predisposed children. Gut microbiota (GM) alterations might trigger the hyperimmune reaction. Our aim was to explore the GM in KD and compare it with the GM of HSP and febrile children. Children diagnosed with KD, HSP and non-KD febrile illness (F) were enrolled. GM was profiled by 16S rRNA gene sequencing and compared with the profiles of healthy children from previous studies. We enrolled 13 KD, 10 HSP and 12 F children. Their GM significantly differed from controls, with an overall reduction in the relative abundance of beneficial taxa belonging to the Ruminococcaceae and Lachnospiraceae families. Potential KD and HSP signatures were identified, including smaller amounts of Dialister in the former, and Clostridium and Akkermansia in the latter. Notably, the GM structures of KD, HSP and F patients stratified by abdominal involvement, with more severe dysbiosis in those suffering from intestinal symptoms. This is the first study analysing GM in a mostly Caucasian cohort of KD and HSP children. Our data could open up new opportunities for childhood vasculitis treatment.

Serum immunoglobulin profiles in Chinese children with Henoch-Schönlein purpura.

The present study focuses on the associations of serum immunoglobulin with disease activity, relapse/recurrence and renal involvement in Henoch-Schönlein purpura (HSP) children. 1683 Chinese children with HSP were recruited from January 2015 to January 2021. Laboratory data of blood samples and urine tests were collected. Renal biopsy was performed by the percutaneous technique. Patients' histories associated with HSP onset were obtained by interviews and questionnaires. (1) IgA and IgE were dramatically elevated in HSP patients as compared to their normal values and subject to a 1.75-3.09 fold and a 1.97-2.61 fold increase, respectively. (2) No significant correlation of the disease activity with IgA and IgE was determined, respectively. (3) The serum IgA levels were significantly lower in patients with relapse/recurrence than that in patients without relapse/recurrence, which may be attributed to the transmission of IgA-mediated immune complexes from blood to vessel walls. (4) No significant correlation was found between serum IgA, IgE levels and the pathological classification. HSP children have marked disorders of serum immunoglobulin profiles, characterized by significant increases in IgA and IgE. The detection of serum IgA may be applicable to predict relapse/recurrence of HSP whereas not associated with disease activity and renal involvement.

Diagnostic value of nailfold capillaroscopy changes in Henoch–Schönlein purpura children with gastrointestinal or renal involvement

Aim of the work: Capillaroscopy is known as a non-invasive, safe, and valuable diagnostic tool to assess the small vessels of the microcirculation in the nailfold. This study aimed to determine the diagnostic value of capillaroscopy in children with Henoch-Schönlein purpura (HSP) and major organ (gastrointestinal or renal) involvement which could increase the risk of complications. Patients and method: This study was conducted on 32 HSP children referred to the rheumatology ward of Children's Medical Center in Tehran, Iran, from 2019 to 2020, as well as 30 healthy controls. The vascular changes of the nailfold were assessed through capillaroscopy. Results: The mean age of the patients was 6.4 ± 2.2 years (3–11 years), and the female/male ratio was 1:1.7 (12 females and 20 males). The main finding of nail-fold capillaroscopy was abnormal morphology in 56.2 % of patients (vs. 6.7% control). A significant difference was seen between patients and control in terms of capillary morphology and architecture (p < 0.05). Abnormal capillary density and diameter tended to be more common in patients than in control (p > 0.05). Seven patients had gastrointestinal or renal involvement (21.9%). A high negative predictive value and low positive predictive value of density, architecture, and diameter of capillaries, were noted between patients with and without gastrointestinal and renal involvement. Conclusion: The role of microvascular involvement in children with HSP is emphasized. Changes in these vessels could be an image of this small vessel vasculitis to reflect major organ invovlement and thus avoid future complications of the disease.

Epidemiology and Clinical Characteristics of Henoch–Schönlein Purpura Associated with Streptococcal Infection in 217 Children in Hubei Province, China

Objectives The objectives of present study were to analyze the association of the streptococcal infection with childhood Henoch–Schönlein purpura (HSP) in China. Methods: We performed a case-control study over a period of five years to evaluate the epidemiology and clinical characteristics of group A β-hemolytic streptococcal (GABHS) triggered HSP. Results: 1. The frequency of GABHS-triggered HSP was 15.1%, while that of GABHS infection developing HSP in children was 4.7%. 2.The epidemiological characteristics of HSP with streptococcal infection were similar to those of HSP alone. 3. The GABHS-triggered HSP cases had a significantly higher frequency of renal involvement than the noninfectious group. 4. IgA and IgG were significantly increased in the streptococcal infection group than in the noninfectious group, while the levels of C3 and C4 decreased significantly. Conclusions: GABHS infection is the most frequent agent in HSP children, and may aggravate the immune dysfunction and prolong the course of HSP.

Abnormalities of Serum Fatty Acids in Children With Henoch-Schönlein Purpura by GC-MS Analysis.

Purpose: The objectives of this work were to test the levels of serum medium- and long- chain fatty acids (MLCFAs) in children and to discover their possible relationship with Henoch-Schönlein Purpura (HSP), also known as Immunoglobulin A vasculitis.Methods: A total of 57 children with HSP (HSP group) and 28 healthy children (CON group) were recruited for this study. Serum specimens were collected to detect the compositions and contents of MLCFAs by gas chromatography with mass spectrometry (GC-MS) analysis.Results: The contents of all detected 37 MLCFAs in the HSP group were higher than the healthy group. Thirty-one species of MLCFAs were discovered to have a significant difference (p < 0.05) in two groups. Comparing to healthy controls, there were 31, 31, 18 fatty acids showed a statistical difference in the untreated group, regular treated group, and withdrawal group of HSP, respectively. The trend of fatty acids in the three HSP groups was similar to the healthy controls, as well as the untreated group and regular treated group changed more obviously than the withdrawal group. Almitate (C16:0) and 18 carbon atoms (C18) of fatty acids were abundant in all three HSP groups, divided according to the treatment of glucocorticoid. Some fatty acids were found having considerable differences (p < 0.05) in three groups. Monounsaturated fatty acids (MUFAs), including elaidate (C18:1T), cis-11,14,17-eicosatrienoic acid ester (C20:1), and cis-15-tetracosenoate (C24:1), were distinctly higher in HSP children with renal damage.Conclusion: Our study revealed that the abnormalities in MLCFA may be associated with the development of HSP. Another interesting finding was that fatty acids contents were changing during the glucocorticoid treatment. Meanwhile, long-chain MUFAs may have an impact on renal damage in HSP patients. Further studies need to be carried out in order to explore the specific mechanism of fatty acids in the course of HSP.

&lt;p&gt;&lt;strong&gt;Correlation between serum cystatin C, thrombomodulin and T lymphocyte subsets in children with Henoch-Schonlein purpura&lt;/strong&gt;&lt;/p&gt;

Henoch-Schönlein Purpura (HSP) is a systemic small vessel, leucocytoclastic vasculitis disease affecting children, and the abdominal pain and joint pain are its classic triad. Here, we studied the correlation between serum cystatin C (CysC), thrombomodulin (TM), and T lymphocyte subsets in HSP cases, and the diagnostic values of these indices. A total of 120 HSP children treated at The 940th Hospital of Joint Logistics Support Force of the Chinese People's Liberation Army from January 2019 to May 2020 participated in this study. Another 64 healthy children receiving routine physical examination in the same time range were enrolled as a control group. In the early morning of the next day after admission, the cubital venous blood was collected. The serum levels of CysC were measured by immunoturbidimetry, the TM was detected using ELISA, while the T lymphocyte subsets were detected by flow cytometry. Univariate and multivariate logistic regression analyses were performed to determine the susceptibility factors. The receiver operating characteristic (ROC) curves were plotted to evaluate the predictive values of CysC, TM and T lymphocyte subset alone and their combination for HSP. The serum levels of CysC, TM, and cluster of differentiation 8 (CD8 + ) of HSP children significantly increased and their CD3 + , CD4 + levels and CD4 + /CD8 + ratio significantly decreased compared with those of control group ( P &lt;0.05). The serum levels of CysC and TM were significantly correlated negatively with CD3 + , CD4 + levels and CD4 + /CD8 + , whereas positively with CD8 + level ( P &lt;0.05). Diet, infection, drugs, exercise-induced tiredness, air pollution, family environment, family inheritance, age, winter and spring were the susceptibility factors for children with HSP. The diagnosis using CysC, TM and T lymphocyte subsets had an AUC of 0.901, sensitivity of 93.1%, and specificity of 90.2%. In conclusion, the combined monitoring of serum CysC, TM, and T lymphocyte subsets in children with HSP can raise the accurate diagnosis rate.

Predicting the Severity of Henoch–Schönlein Purpura: The Role of the Functional Platelet Indices

Objecive: Our aim is to measure the discriminative competency of the qualitative platelet indices obtained at the time of diagnosis on the severity and outcome of Henoch-Schönlein purpura (HSP).Materials and Methods: Electronic data of consecutive HSP children were extracted. The severity of the disease was estimated by an adopted clinical scoring system. Platelet count (PLT), mean platelet volume (MPV), plateletcrit (PCT), and platelet distribution width (PDW) indices at the time of diagnosis were recorded.Results: Overall, 76 HSP patients (mean age: 7.4±2.5 years, 56.5% male) were evaluated. HSP was classified as mild in 59 (77.6%) cases and severe in 17 (22.4%). MPV was significantly lower in severe HSP patients (7.6±1 vs. 8.44±1.1, p&amp;lt;0.01), however, there was no significant difference for PCT and PDW parameters. Relapse occurred in 14 patients (18.4%, 5 mild HSP and 9 severe HSP patients) during follow up. Severe HSP cases were more prone to relapse (p&amp;lt;0.01) and relapsing HSP patients had lower PDW (13.2±3.1 vs. 14.8±2.4 p&amp;lt;0.05).Conclusions: MPV level obtained at the time of first presentation of the HSP disease has a discriminative value for mild and severe HSP. Also, lower PDW might be predictive to suspect the HSP patients susceptive to relapse.

Henoch-Schönlein Purpura in Children: An Updated Review.

Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic smallvessel vasculitis with a predilection for the skin, gastrointestinal tract, joints, and kidneys. It is the most common form of systemic vasculitis in children. The study aimed to familiarize physicians with the etiopathogenesis, clinical manifestations, evaluation, and management of children with Henoch-Schönlein purpura. A PubMed search was conducted in January 2020 in Clinical Queries using the key terms "Henoch-Schönlein purpura" OR "IgA vasculitis" OR "anaphylactoid purpura". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. This paper is based on, but not limited to, the search results. Globally, the incidence of HSP is 10 to 20 cases per 100, 000 children per year. Approximately 90% of cases occur in children between 2 and 10 years of age, with a peak incidence at 4 to 7 years. The diagnosis should be based on the finding of palpable purpura in the presence of at least one of the following criteria, namely, diffuse abdominal pain, arthritis or arthralgia, renal involvement (hematuria and/or proteinuria), and a biopsy showing predominant IgA deposition. Most cases are self-limited. The average duration of the disease is 4 weeks. Long-term complications are rare and include persistent hypertension and end-stage kidney disease. Therapy consists of general and supportive measures as well as treatment of the sequelae of the vasculitis. Current evidence does not support the universal treatment of HSP patients with corticosteroids. Oral corticosteroids may be considered for HSP patients with severe gastrointestinal pain and gastrointestinal hemorrhage. Most cases of HSP have an excellent outcome, with renal involvement being the most important prognostic factor in determining morbidity and mortality. Unfortunately, early steroid treatment does not reduce the incidence and severity of nephropathy in children with HSP. In HSP children who have severe nephritis or renal involvement with proteinuria of greater than 3 months, an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker should be considered in addition to corticosteroids to prevent and/or limit secondary glomerular injury.

Predictive role of neutrophil to lymphocyte ratio and mean platelet volume in Henoch-Schönlein purpura related gastrointestinal and renal involvement.

Henoch-Schönlein purpura (HSP) is a systemic small-vessel vasculitis that occurs mainly in children. The aim was to evaluate the blood neutrophil-to-lymphocyte ratio (NLR) and mean platelet volume (MPV) in patients with HSP and to investigate the relationship with gastrointestinal and renal involvement. Children with HSP and healthy individuals as controls were included. Hemoglobin level, white blood cell count, platelet count, NLR, MPV erythrocyte sedimentation rate and C-reactive protein were evaluated. There were 71 HSP children and 74 controls. NLR was significantly higher in HSP patients with gastrointestinal bleeding than without gastrointestinal bleeding (p < 0,001). The optimal cutoff value of NLR for predicting gastrointestinal bleeding was 2.05, with 93 % sensitivity and 62 % specificity. MPV was significantly higher in HSP patients with renal involvement than without renal involvement (p = 0,027). Blood NLR and MPV may be useful markers to identify gastrointestinal and renal involvement in HSP patients.

Analysis of relevant risk factors to Henoch-Schönlein purpura in Tibetan children

To analyze probable risk factors to Henoch-Schönlein purpura (HSP) in Tibetan children so as to bring evidences for correct identification of high-risk children in plateau areas. 140 high-altitude Tibetan children with HSP admitted to Shannan People's Hospital of Tibet Autonomous Region from October 2015 to October 2018 were enrolled, and 140 high-altitude Tibetan healthy children and 140 plain area HSP children were selected as the control. Gender, age, family history, allergy, past history (rheumatic disease, autoimmune disease, asthma), clinical phenotype, biochemical markers (antibody positive rate, platelet count and hemoglobin), clinical efficacy and recurrence were retrospective analyzed. The risk factors of HSP in the high-altitude Tibetan children were analyzed by univariate and multivariate Logistic regression analysis. It was shown by univariate analysis that the proportion of allergic history and past history of high-altitude HSP children was higher than those of high-altitude healthy children (allergic history: 35.7% vs. 11.4%, past history: 21.4% vs. 5.7%, both P < 0.05). Compared with plain area HSP children, the age of high-altitude HSP children was increased (years old: 6.5±2.3 vs. 5.3±2.2), the clinical phenotype was more complex (37.9% vs. 57.1% for simple skin and limb type, 21.4% vs. 14.3% for abdominal type, 28.6% vs. 21.4% for renal type, 7.1% vs. 5.0% for brain or lung type, 5.0% vs. 2.2% for complex type), the positive rate of antibody was increased (64.3% vs. 50.0%), platelet count was decreased (×109/L: 116.2±12.3 vs. 176.8±35.4), hemoglobin level was increased (g/L: 125.6±15.7 vs. 113.8±10.9), recurrence rate was lower (4.3% vs. 10.7%), and the difference was statistically significant (all P < 0.05). It was shown by multivariate Logistic regression analysis that age, allergic history and past history were independent risk factors for HSP in high-altitude Tibetan children [age: odds ratio (OR) = 1.263, 95% confidence interval (95%CI) = 1.063-1.968; allergic history: OR = 1.765, 95%CI = 1.326-2.452, past history: OR = 1.421, 95%CI = 1.102-2.232, all P < 0.05]. Clinical phenotypic and biochemical indexes were important risk factors affecting the clinical efficacy of high-altitude Tibetan HSP children (non-simple skin and limb type: OR = 2.123, 95%CI = 1.623-2.869; antibody positive: OR = 1.865, 95%CI = 1.502-2.768; both P < 0.05). It is different of HSP occurrence in Tibetan children from plateau and plain areas. Attention should be paid to screening age, allergy history, past history, clinical phenotype, antibody positive and other high risk children. Early and effective intervention can improve clinical curative effect and reduce recurrence.

Gut microbiota dysbiosis is associated with Henoch-Schönlein Purpura in children

BackgroundAlterations in the intestinal microbiota have been associated with the development of allergic diseases, such as asthma and food allergies. However, there is no report detailing the role of microbiota alterations in Henoch-Schönlein Purpura (HSP) development. MethodA total of 85 children with HSP and 70 healthy children were recruited for this study. Intestinal microbiota composition was analyzed by 16S rRNA gene-based pyrosequencing. Fecal microbial diversity and composition were compared. ResultWe compared the gut microbiota of 155 subjects and found that children with HSP exhibited gut microbial dysbiosis. Lower microbial diversity and richness were found in HSP patients when compared to the control group. Based on an analysis of similarities, the composition of the microbiota in HSP patients was also different from that of the control group (r = 0.306, P = 0.001). The relative abundance of the bacterial genera Dialister (P < 0.0001), Roseburia (P < 0.0001), and Parasutterella (P < 0.0001) was significantly decreased in HSP children, while the relative abundance of Parabacteroides (P < 0.006) and Enterococcus (P < 0.0001) in these children was significantly increased. Based on Spearman correlation analysis, the LOS showed a significant negative (P < 0.05) correlation with the genera Paraprevotella and Roseburia. Meanwhile, IgA levels exhibited a significant negative (P < 0.01) correlation with the genus Bifidobacterium. ConclusionsOur results indicate that HSP is associated with significant compositional and structural changes in the gut microbiota. These results enhance the potential for future microbial-based therapies to improve the clinical outcome of HSP in children.

Association of ACE, VEGF and CCL2 gene polymorphisms with Henoch-Schönlein purpura and an evaluation of the possible interaction effects of these loci in HSP patients.

Henoch-Schönlein purpura (HSP) is a multisystem, small vessel, leucocytoclastic vasculitis. It is predominantly a childhood vasculitis, rarely reported in adults. Studies have shown that several different genetic factors such as genes involved in inflammatory system and renin-angiotensin system (RAS) are important in the pathogenesis of Henoch-Schönlein purpura. The purpose of this study was to evaluate the independent effect of 3 gene polymorphisms including CCL2-2518 C/T, VEGF-634G/C and ACE(I/D) with HSP disease and their possible joint interactions in developing the disease. In this case-control study 47 HSP cases and 74 unrelated healthy controls were enrolled for evaluation. All individuals were genotyped for CCL2-2518C/T, VEGF-634G/C and ACE(I/D) gene polymorphisms. The possible association of these polymorphisms with susceptibility to develop HSP disease independently and in different joint combinations was evaluated. The frequencies of TT genotype and T allele of CCL2-2518C/T gene polymorphism and CC genotype and C allele of VEGF-634G/C gene polymorphism were significantly high in HSP children (p-values = 0.005 and = 0.007 respectively). Interestingly, studying the joint interaction of these 2 genotypes (CC genotype of VEGF G-634C and TT genotype of CCL2 C-2518T) in this cohort showed a more significant effect in the development of the disease (p < 0.000, OR = 6.009). The frequency of TT genotype of CCL2 gene when combined with II genotype of ACE gene in HSP children was significantly higher (p < 0.000, OR = 4.213). The results of this pilot study provide evidence of the possible gene-gene interaction effects of CCL2, VEGF and ACE genes in developing HSP disease.

Expansion of Circulating T Follicular Helper Cells in Children with Acute Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP) is a common systemic small vessel vasculitis in children with disorder autoimmune responses. T follicular helper (TFH) cells play crucial roles in regulating immune responses. The aim of our study was to investigate the probable role of TFH cells in the pathogenesis of children with HSP. In this study, the frequency of circulating CXCR5+CD4+TFH cells with inducible costimulator (ICOS) expression in the children with acute HSP was significantly higher than that in healthy controls (HCs) but not CXCR5+CD4+TFH cells with programmed death-1 (PD-1) expression. Moreover, serum levels of IL-21 and IL-6 cytokines, IgA, and C3 in HSP children were also significantly higher than those in HCs. A positive correlation was observed between the frequencies of circulating ICOS+CXCR5+CD4+TFH cells and the serum IL-21 or IgA levels of acute HSP children, respectively. Additionally, the mRNA expression levels of interleukin- (IL-) 21, IL-6, and transcriptional factors (B-cell lymphoma-6, Bcl-6) were also significantly increased in peripheral blood from acute HSP children compared to HCs. Taken together, these findings suggest that TFH cells and associated molecules might play critical roles in the pathogenesis of HSP, which are possible therapeutic targets in HSP children.

An Analysis of the Levels of the Soluble Form of the Endothelial Protein C Receptor in Children with Henoch–Schönlein Purpura

The pathogenesis of Henoch–Schönlein Purpura (HSP) has not been clearly defined. Inflammatory cytokines have been associated with HSP but there are only a few reports that have focused on coagulation. The endothelial protein C receptor (EPCR), which has anticoagulant and antiinflammatory activity, is the key component of the protein C pathway. Recent studies have implicated the soluble form of EPCR (sEPCR) in Wegener's granulomatosis, Behçet's disease, and systemic lupus erythematosus. The aim of this study was to evaluate the levels of sEPCR in HSP children. Twenty-two children with HSP and 17 healthy children were included. We found no significant differences (P > .05) between patient and control groups in the levels of von Willebrand factor and thrombomodulin. The median sEPCR values in the HSP group were lower than the control group (79 vs. 102 ng/mL, respectively) (P > .05). The mean sEPCR value in HSP patients with severe abdominal pain was lower than without (88.8 ± 54.9 vs. 108.2 ± 66.3 ng/mL, respectively) (P > .05). In addition, the mean IL-6 serum levels were significantly elevated in HSP patients during the acute stage of HSP (2.1 ± 1.7 vs. 1.5 ± 1.2 pg/mL, P = .038). We also observed a slight negative correlation between the levels of sEPCR and IL-6 (R = −.135, P > .05). To our knowledge, this was the first study to analyze sEPCR levels in HSP. Our results did not conclusively identify a direct role of sEPCR in HSP, but our findings warrant further investigations, especially in severe HSP cases characterized by gastrointestinal bleeding or renal involvement.

Intravenous dexamethasone followed by oral prednisolone versus oral prednisolone in the treatment of childhood Henoch–Schönlein purpura

The aim of this study was to evaluate the effectiveness of intravenous corticosteroid therapy when Henoch-Schönlein purpura (HSP) patients are unable to tolerate oral medications due to abdominal pain. We retrospectively analyzed 111 children with a diagnosis of HSP (mean age 6.9 ± 2.3 years, male:female = 54:57) from the years 2000 to 2007. They were divided into two groups: 49 patients received only oral prednisolone (PL group) and 62 patients received oral prednisolone after intravenous dexamethasone (Dexa + PL group). Palpable purpura was seen in all 111 patients (100%), abdominal pain in 55 (50%), and arthralgia in 65 (59%). Dexa + PL group had significantly longer duration of fasting than PL group (0.7 ± 1.2 vs. 0.02 ± 0.1 days, P < 0.01) due to more severe and frequent abdominal pain (68 vs. 27%, P < 0.01). Intravenous dexamethasone resulted in the rapid resolution of abdominal pain or arthralgia in all patients without major complications. However, the development of nephritis (21% in PL group versus 32% in Dexa + PL group, P = 0.098), the number of relapse (4 vs. 11%, P = 0.167), and persistent nephritis at last follow-up (12 vs. 16%, P = 0.563) were not different between the two groups despite more severe symptoms in Dexa + PL group. Intravenous dexamethasone followed by oral prednisolone may be a useful and effective therapeutic strategy in HSP children who cannot tolerate oral medications due to severe abdominal pain.

Henoch Schonlein Purpura in Childhood: Epidemiological and Clinical Analysis of 150 Cases Over a 5-year Period and Review of Literature

To examine epidemiological, clinical, and outcome in Italian children affected with Henoch Schönlein purpura (HSP). Retrospective study of children discharged with a diagnosis of HSP from the Meyer Children's Hospital, between 1998 and 2002. Epidemiological, clinical, laboratory data, treatment, and outcome were collected by reviewing medical charts. One year after data collection, the children's parents were interviewed by telephone about the outcome. 150 children entered the study: M:F=1.8:1; mean age 6.1+/-2.7 years. At onset, purpura was present in all cases, arthritis/arthralgias in 74%, abdominal involvement in 51%, scrotal edema in 13%, renal involvement in 54%, severe nephropathy in 7%, acute renal insufficiency in 2%, and intussusception in 0.6%. Purpura was the presenting symptom in 74%, arthritis in 15%, and abdominal pain in 12%. The most frequent laboratory abnormalities were high-erythrocyte sedimentation rate (ESR) (57%), hyper-IgA (37%), and proteinuria (42%). All patients recovered within 2 months. Recurrences, verified in 35%, were correlated with high ESR values and corticosteroid (CS) treatment, independently from other variables. After a mean 2.5-years follow-up, 2 patients had hematuria with normal renal function. Epidemiological and clinical findings in our cohort are similar to those in the literature, even though the mean disease duration was shorter than previously reported. Relapses occurred significantly more frequently in children treated with CS. This finding supports the recommendation to limit the use of steroids to a carefully selected group of HSP children. The prognosis was excellent; although severe nephropathy was found in a small percentage of the children, at follow-up all had normal renal function. Thus, our study confirms the benignity of HSP in Italian children, especially regarding renal outcome.