Intestinal dysbiosis featuring abundance of Streptococcus associates with Henoch-Schönlein purpura nephritis (IgA vasculitis with nephritis) in adult.

BACKGROUND: The pathogenesis of Henoch-Schönlein purpura nephritis (HSPN) is closely associated with mucosal infection. But whether intestinal microbiota dysbiosis plays a role in it is not clear. METHODS: A total of 52 participants including 26 HSPN patients and 26 healthy controls were included. By using 16S ribosomal RNA gene sequencing, the intestinal microbiota composition between HSPN and healthy controls was compared. The diagnostic potency was evaluated by Receiver operating characteristic (ROC) with area under curves (AUC). Meanwhile, correlation analysis was also performed. RESULTS: The lower community richness and diversity of fecal microbiota was displayed in HSPN patients and the structure of gut microbiota was remarkedly different. A genus-level comparison indicated a significant increase in the proportions of g-Bacteroides, g-Escherichia-Shigella and g-Streptococcus, and a marked reduction of g-Prevotella_9 in HSPN patients, suggesting that the overrepresentation of potential pathogens and reduction of profitable strains were the main feature of the dysbiosis. The differential taxonomic abundance might make sense for distinguishing HSPN from healthy controls, with AUC of 0.86. The relative abundance of the differential bacteria was also concerned with clinical indices. Among them, Streptococcus spp. was positively associated with the severity of HSPN (P < 0.050). It was found that HSPN patients with higher level of Streptococcus spp. were more likely to suffering from hematuria and hypoalbuminemia (P < 0.050). CONCLUSIONS: The dysbiosis of gut microbiota was obvious in HSPN patients, and the intestinal mucosal streptococcal infection was distinctive, which was closely related to its severity.

A case of Henoch-Schönlein purpura associated with scrub typhus.

BACKGROUND: Henoch-Schönlein purpura (HSP) may be caused by several allergens. However, to date, HSP caused by Orientia tsutsugamushi has not been reported. Here, we report an unusual rash with features of HSP caused by Orientia tsutsugamushi. CASE PRESENTATION: A man visited a tertiary hospital with bilateral symmetrical purpura and fever. He presented with an eschar in the left popliteal fossa and proteinuria. He was diagnosed with tsutsugamushi disease by indirect fluorescent antibody and positive polymerase chain reaction tests. Purpura biopsy demonstrated a feature of leukocytoclastic vasculitis and IgA deposition in dermal vessels, indicative of HSP. CONCLUSIONS: When examining patients with unique rashes, such as in this case, we suggest investigating out-door activities and evidence of mite bites. Furthermore, differential diagnosis of tsutsugamushi disease should be considered when necessary.

A rare association of acute bacterial endocarditis with Henoch-Schönlein purpura (HSP) in an adult patient.

Henoch-Schönlein purpura (HSP) is a systemic, small vessel vasculitic disorder that mainly affects joint, skin, gastrointestinal tract and kidneys. It is primarily a disease of children that is typically self-limited, but 10 percent of cases occur in adults where features and outcomes may vary. The underlying pathogenesis of HSP remains unknown. We report a case of HSP that occurred with the onset of acute bacterial endocarditis (ABE) in an otherwise healthy 37-year-old Native American male. The patient presented with fevers, fatigue, abdominal pain and renal failure and was found to have acute left-sided staphylococcal endocarditis. He subsequently developed small bowel perforation and purpuric rash. Initially he was treated with broad spectrum antibiotics and small bowel resection. However, resolution of HSP and the associated signs and symptoms was only achieved after treatment with oral steroids and plasmapheresis.

Mendelian randomization reveals association of gut microbiota with Henoch-Schönlein purpura and immune thrombocytopenia.

Gut microbiota have been linked to immune thrombocytopenia (ITP) and Henoch-Schönlein purpura (HSP) in recent studies, but a cause-and-effect relationship is unclear. We used Mendelian randomization (MR) to assess causal relationships between gut microbiota and HSP/ITP using summary statistics from the GWAS dataset of the international MiBioGen and FinnGen consortium. The IVW method was used as the main evaluation indicator. MR analysis of 196 intestinal flora and HSP/ITP/sTP phenotypes showed that 12 flora were potentially causally associated with ITP, 6 with HSP, and 9 with sTP. The genes predicted that genus Coprococcus3 (p = 0.0264, OR = 2.05, 95% CI 1.09-3.88)and genus Gordonibacter (p = 0.0073, OR = 1.38; 95% CI 1.09-1.75) were linked to a higher likelihood of developing ITP. Additionally, family Actinomycetaceae (p = 0.02, OR = 0.51, 95% CI 0.28-0.90) and order Actinomycetales (p = 0.0199, OR = 0.50, 95% CI 0.28-0.90) linked to reduced HSP risk. Genus Ruminococcaceae UCG013 (p = 0.0426, OR = 0.44, 95% CI 0.20-0.97) negatively correlated with sTP risk. Our MR analyses offer evidence of a possible cause-and-effect connection between certain gut microbiota species and the likelihood of HSP/ITP.

Post-staphylococcal infection Henoch-Schönlein purpura nephritis: a case report and review of the literature.

A 37-year-old man developed Henoch--Schönlein purpura nephritis (HSPN) with nephrotic syndrome and rapidly progressive glomerulonephritis after otitis media and externa due to methicillin-resistant Staphylococcus aureus infection. Despite resolution of the infection and prednisolone therapy, his kidney disease worsened. However, the addition of cyclosporine A finally resulted in complete remission of the nephrotic syndrome. A review of similar cases with post-Staphylococcal infection HSPN revealed strong similarities between this entity and immunoglobulin A-dominant postinfectious glomerulonephritis (IgA-PIGN), an increasingly recognized form of PIGN typically associated with Staphylococcal infection, in both clinical and morphological features. Post-Staphylococcal infection HSPN may constitute a subgroup of IgA-PIGN.

Streptococcal infection in childhood Henoch-Schönlein purpura: a 5-year retrospective study from a single tertiary medical center in China, 2015-2019.

BACKGROUND: The present study focuses on the associations of streptococcal infection with the clinical phenotypes, relapse/recurrence and renal involvement in Henoch-Schönlein purpura (HSP) children. METHODS: Two thousand seventy-four Chinese children with HSP were recruited from January 2015 to December 2019. Patients' histories associated with HSP onset were obtained by interviews and questionnaires. Laboratory data of urine tests, blood sample and infectious agents were collected. Renal biopsy was performed by the percutaneous technique. RESULTS: (1) Streptococcal infection was identified in 393 (18.9%) HSP patients, and served as the most frequent infectious trigger. (2) Among the 393 cases with streptococcal infection, 43.0% of them had arthritis/arthralgia, 32.1% had abdominal pain and 29.3% had renal involvement. (3) 26.1% of HSP patients relapsed or recurred more than 1 time within a 5-year observational period, and the relapse/recurrence rate in streptococcal infectious group was subjected to a 0.4-fold decrease as compared with the non-infectious group. (4) No significant differences in renal pathological damage were identified among the streptococcal infectious group, the other infectious group and the non-infectious group. CONCLUSIONS: Streptococcal infection is the most frequent trigger for childhood HSP and does not aggravate renal pathological damage; the possible elimination of streptococcal infection helps relieve the relapse/recurrence of HSP.

Henoch-Schönlein purpura associated with Helicobacter pylori infection in a child.

Henoch-Schönlein purpura is an acute leukocytoclastic vasculitis that primarily affects children. Henoch-Schönlein purpura is often associated with an infection, and a wide variety of infectious agents have been implicated in the pathogenesis. We report a child with Henoch-Schönlein purpura associated with Helicobacter pylori infection. Treatment of the Helicobacter pylori infection was accompanied by prompt resolution of the Henoch-Schönlein purpura.

Stroke in Henoch-Schönlein purpura associated with methicillin-resistant Staphylococcus aureus septicemia: report of a case and review of the literature.

Neurological involvement in Henoch-SchOnlein purpura (HSP) such as stroke is uncommon manifestiation, particularly in association with Staphylococcus aureus (S. aureus). The authors reported a 17-year-old man who developed sudden onset of right hemiparesis while he was admitted in the hospital about his prolonged fever, palpable purpura and upper gastrointestinal bleeding. He also had evidence of MRSA septicemia before the onset of right hemiparesis. Skin biopsy was done and showed that there was leukocytoclastic vasculitis with IgA deposition. He had received completed course of antibiotics and then he was subsequently improved after steroid therapy in the next 2 weeks. Review of case reports from previous English literatures, discovered the association between MRSA infection and HSP which can cause several CNS manifestations including stroke symptoms from cerebral vasculitis.

Oral microbiota dysbiosis and its association with Henoch-Schönlein Purpura in children.

BACKGROUND: The pathogenesis of microbes in allergic diseases has been demonstrated and our previous research indicates that microbiota causing gut disorders in children is associated with Henoch-Schönlein Purpura. However, the role of oral microbiota in Henoch-Schönlein Purpura remains unknown. METHOD: A total of 164 children were enrolled, of which 98 were patients with HSP and 66 were healthy children. Oral swab samples were collected for DNA extraction and 16S rRNA gene sequencing, then analyzed for oral microbiota composition. RESULTS: Oral microbiota differed between healthy children and those with HSP. Children with HSP exhibited higher oral microbial diversity and richness than the controls. Firmicutes, Proteobacteria, and Bacteroidetes are the dominant phyla in children with HSP. We used linear discriminant analysis (LDA) effect size (LEfSe) algorithm and detected 21 bacterial taxonomic clades showing statistical differences (12 increased and 9 decreased) in children with HSP. The correlation analyses between clinical data and abundance in microbial community indicated that an abundance of Butyrivibrio sp. negatively correlated with the length of hospital stay (LOS). Haemophilus sp. negatively correlated to IgE and IgM but positively correlated to LOS, with decreasing significantly in patients with HSP. Prevotella positively correlated with IgM. Prevotella nanceiensis positively correlated with IgA, and were abundant in children with HSP. CONCLUSIONS: These results indicate that children with HSP have significantly different oral microbiota compared to healthy children. Although this study does not imply causality, it is helpful to identify the types and pathways of bacteria that can be used to prevent or treat HSP.

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

BACKGROUND: Alterations 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. METHOD: A 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. RESULT: We 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. CONCLUSIONS: Our 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.

Detection of Neisseria meningitidis DNA from skin lesion biopsy using real-time PCR: usefulness in the aetiological diagnosis of purpura fulminans.

OBJECTIVE: The present study evaluated the usefulness of a real-time polymerase chain reaction (rtPCR) assay for the detection of Neisseria meningitidis (Nm) and genogrouping on skin lesion biopsies in patients with purpura fulminans (PF). DESIGN: Retrospective single-centre study. SETTING: Adult and paediatric intensive care units at the University Hospital of Rouen. PATIENTS: All patients admitted between January 2000 and January 2006, with a final diagnosis of PF and for which a skin biopsy and blood cultures were performed, were included. INTERVENTIONS: Skin biopsy and blood cultures were used for culture and rtPCR. MEASUREMENTS AND MAIN RESULTS: Thirty-four patients fulfilled the criteria (27 children and 7 adults). Nm rtPCR performed on skin biopsy was positive in 100% (34/34) of cases, compared with only 14.7% (5/34) for skin culture (p=0.0001). rtPCR genogrouping on skin biopsy was positive in 58.8% (20/34) of the cases compared with 14.7% (5/34) for skin culture (p=0.0013). For patients (n=17) in whom rtPCR was performed both on blood and skin biopsy, skin biopsy gave a significantly higher rate of Nm detection [100% (17/17) vs. 58.8% (10/17); p=0.023] and genogroup characterisation [76.5% (13/17) vs. 35.3% (6/17); p=0.045] than blood. We encountered no specimen with culture-positive and rtPCR-negative results (negative predictive value of rtPCR 100%). CONCLUSION: In suspected PF cases, skin biopsy is more reliable to identify Nm and its genogroup than blood or, probably, CSF, especially when PCR methods are used. This could help the implementation of public health interventions, especially concerning a vaccination policy.

Severe myalgia of the lower extremities as the first clinical feature of meningococcal purpura fulminans.

In patients with meningococcal infection, devastating presentations, such as purpura fulminans, which can progress to extensive tissue necrosis of the limbs and digits, have a significant social impact. The case presented herein illustrates such a phenomenon in a patient who developed bilateral necrosis of the lower extremities as a result of infection with Neisseria meningitis. We emphasize that severe myalgia was the first clinical manifestation of meningococcal purpura fulminans in our case. However, myalgia has typically been overlooked and undervalued as an early clinical feature of meningococcal sepsis. Early recognition and prompt initial antibiotic therapy continue to be the cornerstones of the successful management of this dramatic disease, reducing morbidity and mortality.

Purpura fulminans in a child secondary to Panton-Valentine leukocidin-producing Staphylococcus aureus.

A case of purpura fulminans (PF) in a child secondary to infection with meticillin-sensitive Staphylococcus aureus (MSSA) encoding the Panton-Valentine leukocidin (PVL) toxin genes is presented. Occasional cases of PF have been documented secondary to S. aureus infection in adults, but, to the authors' knowledge, not in children. Here the first UK case of MSSA-PVL leading to PF is presented.

A case of henoch-schönlein purpura nephritis in pulmonary tuberculosis.

A 38-year-old man with pulmonary tuberculosis developed purpura over both lower extremities and renal disturbance after starting antituberculosis treatment. A renal biopsy and skin biopsy were performed to diagnose the new clinical manifestations, and leukocytoclastic vasculitis with IgA and C3 deposition were detected. Henoch-Schönlein purpura nephritis (HSPN) was diagnosed on the basis of the clinical and pathologic findings, and prednisolone therapy was added. The skin lesions disappeared in 7 days after starting steroid therapy, and renal function gradually improved. These results suggested that the pathogenesis of HSPN might be the consequence of the deposition of the circulating immune complexes. The treatment of HSPN has been not established yet. We should consider how to use steroid therapy for HSPN and call attention to the recurrences of renal disturbance and pulmonary tuberculosis. It is thus recommended to follow patients with HSPN in tuberculosis for long periods.

Successful treatment of septic shock with purpura fulminans caused by Trichosporon asahii in an immunocompetent patient.

Trichosporon asahii is an emerging mycosis characterized by high mortality rate in immunologically compromised patients. Only a few cases have been reported in immunocompetent subjects. We report a 46-yr-old man who had been healthy and who presented with septic shock and purpura fulminans caused by Trichosporon asahii. He responded well to antifungal therapy with amphotericin B and voriconazole.

[Distinction between bacterial and aseptic meningitis in children: refinement of a clinical decision rule]. / Distinction entre les méningites bactériennes et virales chez l'enfant: affinement d'une règle de décision clinique.

OBJECTIVES: To refine and to re-validate the best current tool (the Nigrovic rule: ''outpatient management may be considered for children without seizure, blood neutrophil count>or=10,000/mm(3), positive cerebrospinal fluid -CSF- Gram-staining, CSF protein>or=80 mg/dl, or CSF neutrophil count>or=1,000/mm(3)'') proposed to distinguish between aseptic meningitis (AM) and bacterial meningitis (BM) in the emergency department. METHODS: Children hospitalized for BM between 1995 and 2004, or AM between 2000 and 2004 were included, and randomly divided into derivation (111 children, 14 BM) and internal validation (57 children, 7 BM) sets. The Nigrovic rule was refined on the derivation set, introducing new variables (purpura, toxic appearance and high serum procalcitonin), changing variables thresholds (CSF protein) and withdrawing some variables (blood neutrophil count, CSF neutrophil count), according to previous results, with the aim to obtain 100% sensitivity user friendly tool. The refined rule was then applied on the internal validation set, stayed blinded during the derivation process. RESULTS: The refined rule was: start antibiotics in case of seizure, purpura, toxic appearance, procalcitonin>or=0.5 ng/ml, positive CSF Gram-staining, or CSF protein>or=50 mg/dl. The refined rule had 100% sensitivity on the derivation and the internal validation sets (95% confidence interval 78-100, and 65-100, respectively) with 62 and 51% specificity, respectively. CONCLUSION: The refined rule (called Meningitest) was a highly sensitive, specific and user friendly tool that could allow to safely avoid>50% a posteriori unuseful antibiotic treatments for patients with AM.

[Meningococcal purpura fulminans: treatment with recombinant protein C activator in 3 cases]. / Púrpura fulminante meningocócica tratada con proteína C activada. A propósito de tres casos.

Purpura fulminans is a serious disease associated with high rates of morbidity and mortality. It usually leads to disseminated intravascular coagulation and septic shock related to reduced levels of protein C. Recombinant protein C (rPC) activator has been used successfully to inhibit this process. Intracranial hemorrhages are the most important, life-threatening adverse effects of treatment with rPC activator. We report 3 cases of patients with meningococcal purpura fulminans who developed septic shock and multiorgan dysfunction. They were treated with the protocol for septic shock, antibiotics and rPC activator from the time of admission, and improvement in hemodynamic dysfunction was observed within hours in all patients. All received platelet replacement transfusions. Subarachnoid bleeding complications occurred in 2 patients. One patient died 5 days after admission and 2 were discharged from the intensive recovery care unit 28 days after admission.

Henoch-Schönlein purpura after postoperative Staphylococcus aureus infection with hepatic IgA nephropathy.

A 66-year-old man with a two-year history of hepatitis C viral liver cirrhosis, was diagnosed as having ascending colon cancer. Right hemicolectomy was performed, and a drain was fed down to the anastomosis. On post-operative day (POD) 9, and methicillin-sensitive Staphylococcus aureus (MSSA) was isolated from both drains. After POD 12, relapsing persistent diarrhea with some blood occurred. On POD 20, the temperature increased to 39 degrees C, with symmetrical purpura and swelling in the femurs, and knee arthralgia developed. HSP was suspected. Clinical follow-up showed slight spontaneous reduction of diarrhea and purpura on POD 26. However, despite the negative drain culture, the high fever was maintained on POD 27. Therefore, intravenous steroid pulse therapy was performed. The purpura subsequently disappeared, except for a slight pigmentation and the temperature returned to normal. A renal biopsy was performed 26 days after the appearance of purpura. Pathological views demonstrated acute focal segmental glomerulonephritis-like nephropathy in addition to cirrhotic nephropathy with a membranoproliferative glomerulonephritis (MPGN)-like pattern and the mesangial proliferative glomerulonephritis type. We describe a case of Henoch-Schönlein purpura (HSP) after postoperative Staphylococcus aureus infection of the intra-abdominal drain with IgA nephropathy associated with hepatitis C virus liver cirrhosis.