Recurrent Henoch-Schönlein Purpura Research Articles

A rare association of invasive infective endocarditis due to Abiotrophia defectiva with ventricular septal defect and recurrent Henoch-Schonlein purpura in a child

BackgroundHenoch-Schonlein purpura is the most common vasculitis in childhood, usually triggered by an upper respiratory tract infection and rarely observed in infective endocarditis patients. Abiotrophia defectiva is a rare causative agent of infective endocarditis associated with pre-existing heart disease, immunocompromised and prosthetic valves. Dental procedures are also a common predisposing factor.Case presentationWe present the first pediatric congenital heart disease case of infective endocarditis caused by Abiotrophia defectiva combined with recurrent Henoch-Schonlein purpura. A 10-year-old girl with uncorrected congenital heart defects and Henoch-Schonlein purpura developed a purple petechial rash again. Transthoracic echocardiography evaluation revealed multiple irregular vegetations on the right ventricular side of the ventricular septal defect and on the tricuspid valve leaflets. Blood cultures grew Abiotrophia defectiva. The girl received cardiac surgery for vegetation resection as well as congenital heart defect correction and tricuspid valve replacement. Five months after the surgery, the patient was in satisfactory condition without any signs of endocarditis or valve insufficiency and her purpuric rash disappeared.ConclusionsThe coexistence of recurrent Henoch-Schonlein purpura and infective endocarditis is possible. Abiotrophia defectiva belongs to the streptococcus with a high virulence. In addition, cardiovascular surgery is often required for pediatric infective endocarditis associated with Abiotrophia defectiva, and bioprosthetic valve replacement is considered feasible for irreparable tricuspid valve in children.

Differences in Manifestations and Gut Microbiota Composition Between Patients With Different Henoch-Schonlein Purpura Phenotypes.

BackgroundGut microbiota plays an important role in the pathogenesis of immune-mediated diseases. However, the complex pathogenesis of Henoch-Schonlein Purpura (HSP) remains elusive. This study aimed to characterize the gut microbiota in HSP patients and explore the potential association between gut microbiota composition and phenotypic changes in HSP.Methods16SrRNA gene sequencing and bioinformatic analyses were performed using total DNA extracted from the fecal microbiota of 34 children with HSP, including 18 primary cases, 16 recurrent cases, and 23 healthy children.ResultsThe diversity indexes showed significant differences in the microbial community among the primary HSP groups, the recurrent HSP group and healthy controls. The abundance of Escherichia-Shigella in the recurrent HSP group was significantly higher than that in the primary HSP group, and the constructed ROC curve had an AUC value of 0.750. According to the Spearman correlation analysis, the abundance of Bacteroides was positively associated with the serum IgG level in children with HSP, while the abundance of Lachnoclostridium was negatively correlated with the complement component 3 (C3). The diversity indexes of gut microbiota in the HSP group with abdominal symptoms were higher than those in the HSP group without GI involvement, and also higher than those in the healthy control group. In the HSP group with GI involvement, the abundance of Faecalibacterium was decreased, while the abundance of Streptococcus and Fusobacteria was increased, compared to the HSP group without GI involvement.ConclusionsThe gut microbiota of children with HSP was different from that of healthy children. The genus Escherichia-Shigella has a diagnostic value for HSP recurrence. Bacteroides and Lachnoclostridium may affect IgG and complement C3 levels in children with HSP. Abdominal symptoms in HSP children were related to gut microbiota (Streptococcus and butyric acid-producing bacteria).

A153 RECURRENT HENOCH-SCHONLEIN PURPURA FOLLOWING TREATMENT WITH ADALIMUMAB

Abstract Background Anti Tumour Necrosis Factor-α agents have revolutionised the management of inflammatory bowel disease. Cutaneous adverse events complicate therapy in up to 20% of cases. Most reactions are mild and do not warrant a change of therapy. Henoch–Schönlein purpura (HSP) has rarely been associated with anti-TNFα therapy. It is an acute vasculitis of small vessels that presents with cutaneous purpura of the lower limb, arthritis, nephritis and gastrointestinal involvement. Aims To describe the clinical course of a 16-year-old male presenting with recurrent HSP secondary to adalimumab for treatment of inflammatory bowel disease Methods A retrospective chart review of the patient’s electronic medical record. A literature review of the relevant medical literature. Results History 16 year old boy with Crohn’s disease, initial induction with exclusive enteral nutrition and maintained on adalimumab monotherapy since May 2017. Adalimumab initially 40 mg every 2 weeks, increased to every 10 days in September 2019 due to loose stool and mild inflammation throughout the colon on reassessment colonoscopy. Four separate incidences of purpuric rashes occurring 2–3 days after receiving adalimumab between November 2019 and July 2020. Purpura and petechiae of the lower limb with associated swelling of the feet and heel pain consistent with HSP. Purpura resolved within 1 to 3 weeks, treated with oral steroids on 1 occasion. First episode occurred following increase in dose frequency to every 10 days. Adalimumab frequency was further increased to every 7 days in March 2020. Following this he had 3 further HSP episodes. There was no history of preceding illness or other clear inciting event for HSP. Physical Exam Scattered petechiae on the lower limb bilaterally with large palpable purpura to his feet. Mild swelling to the right foot with tenderness to heel on palpation. Nil other joint swelling. No petechiae elsewhere. Investigations Urinalysis showed trace blood, no proteinuria. Mildly elevated CRP and ESR. Fecal calprotectin elevated >1800. Clinical Progress Rheumatology and Dermatology were consulted. Due to recurrent HSP and active luminal Crohn’s disease on repeat colonoscopy, adalimumab was discontinued and he started Ustekinumab September 2020. There has been no recurrence of relapsing rash and joint pains. Conclusions Four previous cases of HSP associated with Adalimumab have been described, three with Crohn’s disease and 1 with Ulcerative Colitis. Cutaneous manifestations occurred within 18 months of treatment in all previously reported cases. Our patient was treated with Adalimumab for 30 months prior to his first episode of HSP. This association with HSP is not unique to adalimumab, extending to other members of the anti TNFα class including infliximab and etanercept. Hypothesized mechanisms include antibody production, eosinophil activation, shifts in T cell responses and direct drug toxicity to vessel walls. Funding Agencies None

Effect of Qishen-Xiaodian Decoction combined with laser acupoint irradiation on recurrence oxidative stress and renal function oin children with henoch schonlein purpura

Objective To explore the effect of Qishen-Xiaodian Decoction combined with laser acupoint irradiation on oxidative stress and renal function on children with recurrent henoch schonlein purpura (HSP). Methods A total of 120 children with recurrent HSP in the dermatology department clinic of Hospital of Xinle City from January 2015 to November 2017 were divided into two groups according to the random number table method, with 60 cases in each group. The control group took loratadine, Troxerutin and vitamin C orally, while the treatment group took Chinese medicine combined with laser acupoint irradiation based onthe control group. Drugs were taken orally for 4 weeks, laser acupoint irradiation treatment for 2 weeks. The level of glutathione peroxidase (GSH-Px) in plasma was detected by ELISA, the levels of MDA and SOD in plasma were detected by thiobarbituric acid colorimetry and hydroxylamine method, the level of IgG, micro albumin (Alb), β2-microglobulin (β2-MG) in urine were detected by radioimmunoassay, and the clinical effect was evaluated. The recurrence was recorded. Results After treatment, the plasma GSH-Px (90.45 ± 15.36 μmol/L vs. 81.62 ± 13.68 μmol/L, t=3.318), SOD (99.64 ± 18.66 IU/ml vs. 84.21 ± 16.73 IU/ml, t=4.769) in the treatment group were significantly higher than the control group (P<0.01); the MDA (5.58 ± 1.31 μmol/L vs. 4.37 ± 1.36 μmol/L, t=4.964) was significantly lower than the control group (P<0.01); the ratio of urine IgG (3.48 ± 0.95 mg/L vs. 6.56 ± 1.47 mg/L, t=13.630), Alb (7.80 ± 2.94 mg/L vs. 12.73 ± 4.26 mg/L, t=7.378), β2-MG (4.02 ± 1.61 mg/L vs. 6.95 ± 2.10 mg/L, t=8.577) were significantly lower than those of the control group (P<0.01). The total effective rate was 95.0% (57/60) in the treatment group and 80.0% (48/60) in the control group. There was statistically significant difference between two groups (χ2=6.171, P=0.013). Follow-up of 6 and 12 months, the recurrence rate of the treatment group were significantly lower than that of the control group (χ2 value were 4.931, 4.574, P<0.05). Conclusions Qishen-Xiaodian Decoction combined with laser acupoint irradiation can improve the oxidative stress of children with recurrent HSP, protect the renal function, improve the clinical efficacy and reduce the recurrence rate. Key words: Purpura, schoenlein-henoch; Recurrence; Qishen-Xiaodian Decoction; Lasers, semiconductor; Oxidative stress; Kidney function tests

Urinalysis monitoring in children with Henoch-Schönlein purpura: Is it time to revise?

To describe the natural history and risk factors of renal involvement in our Henoch-Schönlein purpura (HSP) inception cohort. HSP patients followed at our center for at least 6months between 1/2009-4/2017 were included. A 2-year urinalysis (UA) monitoring protocol was adopted (6monthly and another 6 of 3monthly UA). Renal involvement included minimal renal involvement defined as isolated hematuria (urine red blood cells >5/high-power field or 10/µL) and/or proteinuria (urine protein >1+), and renal impairment defined as nephritic, nephrotic symptoms, or renal insufficiency. Recurrent HSP were excluded. Kaplan-Meier estimates and log-rank test were used to analyze the duration to onset and resolution of abnormal UA. Relationships between demographic and clinical features and renal involvement were studied using logistic regression analyses. Two hundred and thirty-eight patients (52.9% male) were analyzed. Median duration of follow up was 20.6 (interquartile range 11.3-24.4) months. Eighty-nine children (37.4%) developed abnormal UA either at diagnosis (n=43), or during follow up (n=46), mostly (91.0%) within 6months. Seventeen patients (7.1%) developed renal impairment. Among patients without renal impairment, an earlier subsidence (P=0.008) was noted in those with normal UA at diagnosis and most abnormal UA resolved by 18months in this subgroup. Older age at diagnosis was a risk factor of renal involvement (P<0.001). Prednisolone therapy for non-renal indications did not affect the onset or duration of renal involvement. Normal UA at diagnosis indicated a shorter duration of renal involvement. We propose a curtailed duration of follow up for those with normal and abnormal UA at diagnosis.

Recurrent adult onset Henoch-Schonlein Purpura: a case report

Henoch-Schonlein purpura is an immunoglobulin A (IgA)-immune complex mediated leukocytoclastic vasculitis that classically manifests with palpable purpura, abdominal pain, arthritis, and hematuria or proteinuria. The condition is much more predominant in children (90% of cases) and commonly follows an upper respiratory infection. We present a case of recurrent Henoch-Schonlein purpura (HSP) complicated by nephritis in an adult female initially categorized as IgA nephropathy (IgAN). We review the pathophysiologic basis of HSP nephritis as the variant of HSP accompanied by renal involvement and its pathogenetic commonality with IgA nephropathy.

Association of Clinical and Genetic Features in Familial Mediterranean Fever Among Children from Central Anatolia, Turkey

The aim of the present study was to perform a genotype/phenotype and gender correlation in children in addition to investigate distribution and frequency of the Mediterranean fever gene mutations. Records of 700 children with familial Mediterranean fever living in Sivas-Central Anatolia were retrospectively reviewed. Most common clinical features were fever (100 %), abdominal pain (92.7 %), and arthritis (23.3 %). Mutations were detected in 66.7 % of patients. Simple heterozygous, compound heterozygous, and homozygous mutations were detected respectively 66.4, 8.6 and 25 % of patients. The most frequent mutations were M694V, E148Q, M680I (G/C), V726A, and A744S. The mean severity score was higher in homozygous M694V, in heterozygous M694V and in heterozygous E148Q mutations than in others. It was higher in girls than in boys. Arthritis and abdominal pain had a meaningful relation with M694V and E148Q mutations. Appendectomy and rare complications (oral aphthae, scrotal edema, Henoch Schonlein purpura, and amyloidosis) were significantly seen more in M694V and in E148Q than in other mutations. High acute phase reactant response was highest in M694V and in E148Q. Ten patients had amyloidosis and 4 of them had recurrent Henoch Schonlein purpura. This study showed that, in addition to patients with M694V mutations, patients with E148Q mutations also had a more severe clinical course. Apart from that, disease severity score was higher in girls than in boys and the co-existence of Henoch Schonlein purpura and familial Mediterranean fever is significant.

Henoch–Schönlein purpura with uveitis: an unusual case and review of literature

Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA dominant immune complex deposition. It is characterized by a triad of palpable purpura (without thrombocytopenia), abdominal pain and arthritis. Uveitis is rarely associated with HSP with only 3 cases reported in literature. All these cases were in adult population and were associated with nephritis. However, this association is not reported in paediatric age group. We are reporting a case of an 11-year-old child of recurrent HSP with uveitis.

Recurrent Henoch-Schönlein Purpura in Children

Henoch-Schönlein purpura (HSP), also known as anaphylactoid purpura is a clinically recognizable systemic disorder occurring in children, mainly from ages 3 to 10 years. To describe the clinical, epidemiological, and laboratory findings in a group of patients with recurrent HSP, admitted to a tertiary pediatric center. Retrospective analysis of medical records of patients hospitalized due to HSP between 1969 and 2004. Two hundred sixty children (56.7% males) were hospitalized due to HSP, 7 (2.7%) more than once. There were no statistically significant differences in demographic or clinical characteristics between the patients with 1 event of HSP and patients with recurrence. Mean age of the subgroup with recurrence was 3.67 years (10 months to 7.4 years) at the first episode, and 5.03 years (2.2-10 years) at the second one, with a mean lag period of 13.5 +/- 2.8 months (range 2-26). The duration of the recurrent clinical symptoms ranged from 9 to 30 days, and in 72% of those patients, resolution took more than 14 days. In our inpatient population, no clinical or laboratory characteristics were found to be predictive of recurrence; the second episode was longer than the first and the lag period between the 2 episodes was substantially longer than previously reported. Hospital admissions for recurrent HSP are not common. Nevertheless, a good prognosis was the rule of our admitted patients.

Recurrent Henoch-Schonlein purpura presenting as gingival petechiae and mandibular pain

Henoch-Schonlein purpura (HSP), or anaphylactoid purpura, is a syndrome of small vessel vasculitis in children characterized by nonthrombocytopenic purpura in a characteristic distribution, arthritis with often severe arthralgia, and cramping abdominal pain. 1 The clinical course is variable, but is usually limited to a single episode that lasts from 4 to 6 weeks? While the majority of patients recover completely, recurrences ensue in approximately 40% of cases.? Uncommon presentations ofHSP include ocular, pulmonary, scrotal, testicular, and neurological involvement.' Oropharyngeal manifestations have been only rarely reported? and are not mentioned in extensive reviews of the usual and unusuaf manifestations of HSP. We present the case of a IS-year-old girl who presented with gingivitis, gingival petechiae, and mandibular pain as the primary manifestation of her recurrent HSP.