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1.
Cureus ; 16(8): e68323, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-39350862

RESUMO

Henoch-Schönlein purpura (HSP), also known as IgA vasculitis, is a hypersensitivity vasculitis characterized by palpable purpuric lesions associated with polyarthralgia, abdominal discomfort, and renal involvement. We present the case of a 41-year-old man who was admitted to the emergency department due to generalized purpuric lesions and abdominal pain. During the complementary study, there was no evidence of thrombocytopenia or coagulopathy but confirmed microscopic haematuria. The diagnosis of HSP was supported by the presence of leukocytoclastic vasculitis with perivascular IgA deposits in the skin biopsy. After excluding infectious, autoimmune, and neoplastic pathologies, the possibility of HSP associated with taking lisinopril, which had been recently initiated after hospitalization for acute heart failure, was assumed. Angiotensin-converting enzyme (ACE) inhibitor suspension and treatment with systemic corticosteroids lead to significant clinical regression, supporting our suspicion.

2.
J Cutan Pathol ; 2024 Sep 22.
Artigo em Inglês | MEDLINE | ID: mdl-39307568

RESUMO

BACKGROUND: Due to the immune-mediated nature of non-infectious cutaneous vasculitis, skin biopsy specimens are often submitted for direct immunofluorescence (DIF) testing when vasculitis is considered clinically. However, evidence regarding the clinical value of DIF has not been rigorously appraised. OBJECTIVE: In this scoping review, we aimed to systematically evaluate the peer-reviewed literature on the utility of DIF in vasculitis to assist with the development of appropriate use criteria by the American Society of Dermatopathology. METHODS: Two electronic databases were searched for articles on DIF and vasculitis (January 1975-October 2023). Relevant case series involving more than or equal to three patients, published in English, and with full-text availability were included. Additional articles were identified manually via reference review. Due to study heterogeneity, findings were analyzed descriptively. RESULTS: Of 255 articles identified, 61 met the inclusion criteria. Cumulatively representing over 1000 DIF specimens, several studies estimated DIF sensitivity to be 75%. While vascular immunoglobulin A (IgA) deposits on DIF were associated with renal disease, other systemic associations were inconsistent. Vascular IgG deposition may be overrepresented in ANCA-associated vasculitis. Granular vascular and epidermal basement membrane zone Ig deposition differentiated hypocomplementemic from normocomplementemic urticarial vasculitis. Few studies have assessed the added value of DIF over routine microscopy alone in vasculitis. CONCLUSIONS: This scoping review discovered that DIF testing for vasculitis has been performed not only for diagnostic confirmation of vasculitis but also for disease subtype classification and prediction of systemic associations. Future studies on test sensitivity of DIF compared to that of histopathology are needed.

3.
Skin Res Technol ; 30(9): e13921, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39252568

RESUMO

OBJECTIVE: Investigate Proanthocyanidins (PCs) efficacy and mechanisms in treating Henoch-Schönlein purpura (HSP)-like rat models, focusing on inflammatory and oxidative stress (OS) responses. METHODS: An HSP-like rat model was established using ovalbumin (OVA) injection, leading to symptoms mimicking HSP. The study measured inflammatory markers (IL-4, IL-17, TNF-α), OS markers (MDA, SOD, CAT), and assessed the TLR4/MyD88/NF-κB signaling pathway's involvement via histopathological and immunofluorescence analyses. RESULTS: PCs treatment significantly improved HSP-like symptoms, reduced inflammatory cell infiltration, and decreased IgA deposition in renal mesangial areas. Serum analyses revealed that PCs effectively lowered IL-4, IL-17, TNF-α, and MDA levels while increasing SOD and CAT levels (p < 0.05). Crucially, PCs also downregulated TLR4, MyD88, and NF-κB expressions, highlighting the blockage of the TLR4-mediated signaling pathway as a key mechanism. CONCLUSION: PCs show promising therapeutic effects in HSP-like rats by mitigating inflammatory responses and oxidative damage, primarily through inhibiting the TLR4/MyD88/NF-κB pathway. These findings suggest PCs as a potential treatment avenue for HSP, warranting further investigation.


Assuntos
Modelos Animais de Doenças , Vasculite por IgA , Fator 88 de Diferenciação Mieloide , NF-kappa B , Estresse Oxidativo , Proantocianidinas , Transdução de Sinais , Receptor 4 Toll-Like , Animais , Receptor 4 Toll-Like/metabolismo , Estresse Oxidativo/efeitos dos fármacos , Fator 88 de Diferenciação Mieloide/metabolismo , Vasculite por IgA/tratamento farmacológico , Ratos , NF-kappa B/metabolismo , Proantocianidinas/farmacologia , Transdução de Sinais/efeitos dos fármacos , Masculino , Inflamação/tratamento farmacológico , Ratos Sprague-Dawley
4.
Cureus ; 16(9): e69389, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39282485

RESUMO

Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by palpable purpura, arthralgia or arthritis, GI symptoms, and renal involvement. Superior mesenteric artery (SMA) syndrome, a rare condition, occurs when the third part of the duodenum is compressed between the aorta and the SMA, leading to upper intestinal obstruction. This case report describes the clinical presentation, diagnostic process, and management of an eight-year-old girl with HSP complicated by SMA syndrome. The patient initially presented with abdominal pain and vomiting, eventually developing the characteristic rash of HSP. While initial management was supportive, her condition deteriorated. Treatment with intravenous methylprednisolone resulted in significant symptom improvement and resolution of both SMA syndrome and HSP manifestations. This case highlights the need to recognize SMA syndrome as a potential complication of HSP and demonstrates the effectiveness of steroid therapy in managing this condition. Further research is needed to develop comprehensive treatment guidelines for HSP patients with SMA syndrome.

5.
Rheumatol Int ; 44(11): 2613-2620, 2024 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-39249140

RESUMO

Coronavirus Disease 2019 (COVID-19), caused by SARS-CoV-2, has negatively affected global health. COVID-19 has been associated with a variety of autoimmune and inflammatory disorders, complicating its respiratory manifestations. SARS-CoV-2 triggers inflammatory reactions which may involve multiple organs and systems. The proof for IgA involvement in the immune reactions to coronavirus infection is growing, particularly in the case of IgA immune complex deposition diseases such as IgA vasculitis (IgAV) and IgA nephropathy.This report presents a case of IgAV caused by SARS-CoV-2 in a 53-year-old man. His symptoms included papillomatous, bright red rashes, urticaria throughout the body, aphthous stomatitis, pain in all joints and muscles, weakness, malaise, abdominal pain, face swelling, and arterial hypertension (160/100 mmHg). He received intravenous methylprednisolone (250 mg) and then oral methylprednisolone (16 mg) treatment, which improved his condition. This improvement included the disappearance of abdominal and joint pain and skin rashes.This article also provides an overview of published cases of IgAV after SARS-CoV-2. It may alert rheumatologists and allied specialists of clinical features of IgAV and guide them how to diagnose and treat this disease.


Assuntos
COVID-19 , Imunoglobulina A , Metilprednisolona , SARS-CoV-2 , Humanos , Masculino , COVID-19/complicações , COVID-19/imunologia , Pessoa de Meia-Idade , SARS-CoV-2/imunologia , Metilprednisolona/uso terapêutico , Imunoglobulina A/imunologia , Vasculite por IgA/tratamento farmacológico , Vasculite por IgA/diagnóstico , Vasculite por IgA/imunologia , Vasculite/tratamento farmacológico , Vasculite/etiologia , Vasculite/imunologia
6.
PeerJ ; 12: e18175, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39346080

RESUMO

Purpose: To investigate the association between the Dietary Inflammatory Index (DII) and disease severity as well as complications in children diagnosed with Henöch-Schönlein purpura (HSP), shedding light on the potential influence of dietary factors on HSP. Methods: A retrospective cohort study was conducted, enrolling children aged 2-14 years diagnosed with HSP. Participants were divided into low and Pro-inflammatory dietary groups based on their DII scores. Biomarkers, nutrient intake, blood lipid profiles and disease complications were compared between the two groups. Spearman correlation analysis was performed to assess the relationship between DII and complications. Results: A total of 115 patients, including 56 patients with anti-inflammatory dietary and 59 with pro-inflammatory dietary, were included. The pro-inflammatory dietary group demonstrated significantly elevated of C-reactive protein, tumor necrosis factor-α, interleukin-6, erythrocyte sedimentation rate, white blood cell count, eosinophils, IgE, consumption of total calories, protein, carbohydrates, fiber, fat intake, total cholesterol, LDL cholesterol, HDL cholesterol, triglycerides, VLDL cholesterol, complications of renal, skin, gastrointestinal, coagulation and respiratory in comparison to the anti-inflammatory dietary group. DII was positively correlated with renal, skin, gastrointestinal, coagulation and respiratory complications. Conclusion: The study highlights the potential influence of dietary inflammatory potential, as quantified by the DII, on disease severity and complications in children with HSP. Understanding the interplay between dietary patterns and inflammatory responses in pediatric vasculitis has implications for the management of HSP, emphasizing the relevance of considering dietary interventions to optimize clinical outcomes and improve the overall well-being of affected children.


Assuntos
Dieta , Vasculite por IgA , Índice de Gravidade de Doença , Humanos , Criança , Estudos Retrospectivos , Vasculite por IgA/sangue , Masculino , Feminino , Pré-Escolar , Adolescente , Dieta/efeitos adversos , Inflamação/sangue , Biomarcadores/sangue , Proteína C-Reativa/análise , Proteína C-Reativa/metabolismo , Fator de Necrose Tumoral alfa/sangue
7.
Life (Basel) ; 14(8)2024 Jul 25.
Artigo em Inglês | MEDLINE | ID: mdl-39202674

RESUMO

PURPOSE OF REVIEW: IgA vasculitis (IgAV), formerly Henoch-Schonlein purpura, is the most common systemic vasculitis in childhood. In adults, however, this condition is poorly understood, yet associated with more severe disease and poorer outcomes. This necessitates the need for early diagnosis and management. SCOPE OF REVIEW: We describe the pathophysiology, clinical manifestations, and diagnosis of IgAV in adults. Poor outcomes are often due to the high frequency of glomerulonephritis in IgAV-IgA vasculitis-associated nephritis (IgAVN). We hence also aim to summarize the latest clinical data regarding treatment strategies in IgAVN. The diagnosis and differentiation in histology between IgAVN and IgA nephropathy (IgAN) remain a challenge. Review of treatment therapies: Pathological mechanisms between IgAVN and IgAN appear to be consistent between the two, and data from IgAN are often extrapolated to IgAVN. The role of various immunosuppression therapies remains controversial, and in this review, we will discuss immunosuppression use and highlight evidence surrounding emerging and promising novel therapies in IgAVN/IgAN. Our aim for this review is to guide future treatment strategies and direct future studies.

8.
J Am Coll Emerg Physicians Open ; 5(4): e13270, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-39104916

RESUMO

Acute compartment syndrome is a surgical emergency requiring rapid recognition in the emergency department to minimize morbidity and mortality. It is most commonly caused by traumatic extremity fractures, which account for about 75% of cases. Atraumatic acute compartment syndrome is substantially less common with current evidence mostly limited to case reports, and diagnosis is made more challenging by the absence of an obvious traumatic injury. We present the case of a young adult female patient with IgA vasculitis who developed recurrent, atraumatic acute compartment syndrome and was successfully managed with prompt fasciotomy. This is the first case of spontaneous intramuscular hemorrhage, a rare sequela of IgA vasculitis, leading to recurrent, atraumatic acute compartment syndrome. This case highlights the importance of both a thorough physical exam and maintaining a high suspicion for acute compartment syndrome in the absence of injury to ensure patients receive prompt surgical evaluation for definitive care.

9.
Eur J Dermatol ; 34(4): 378-383, 2024 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-39193674

RESUMO

There is a lack of systematic studies on single-organ cutaneous small vessel vasculitis (SOCV). To evaluate prognostic clinical and laboratory parameters, including systemic immune-inflammation biomarkers (SIIB) in SOCV inpatients. This study investigated the clinical and laboratory data of 178 inpatients. Blood tests were performed at baseline. SIIB were assessed based on neutrophil-to-lymphocyte ratio (NLR) and pan-immune-inflammation value (PIV). Univariable and multivariable statistics were performed. Both NLR and PIV were significantly higher in SOCV patients than in healthy controls. However, the SIIB values observed in SOCV patients were as high as those in psoriasis patients. On logistic regression analysis, disease manifestation on the upper extremities strongly predicted the absence of severe disease (OR: 0.31, 95% CI: 0.13 to 0.73; p = 0.0071). Moreover, older age (OR: 2.3, 95% CI: 1.11 to 4.77; p = 0.025) and severe disease (OR: 2.4, 95% CI: 1.16 to 4.94, p = 0.018) were significant independent predictors of longer hospital stay, whereas female sex was an independent protective factor for longer hospitalization (OR: 0.52, 95% CI: 0.28 to 0.96, p = 0.038). Lower serum C3 was a strong independent predictor of disease recurrence (OR: 13.9, 95% CI 3 to 63.4; p = 0.0007). The increase in SIIB observed in patients with SOCV reflects that systemic inflammatory alterations also play a role in SOCV patients. We identified several clinical and laboratory-based independent predictors of SOCV severity, length of hospitalization, and disease recurrence that may aid prognostication of SOCV patients.


Assuntos
Tempo de Internação , Neutrófilos , Recidiva , Índice de Gravidade de Doença , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Tempo de Internação/estatística & dados numéricos , Adulto , Prognóstico , Biomarcadores/sangue , Fatores Sexuais , Complemento C3/análise , Complemento C3/metabolismo , Idoso , Fatores Etários , Linfócitos/patologia , Vasculite Leucocitoclástica Cutânea/patologia , Estudos de Casos e Controles , Contagem de Linfócitos
10.
Pediatr Dermatol ; 2024 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-39169477

RESUMO

Immunoglobulin A (IgA) vasculitis, or Henoch-Schonlein purpura, is the most common systemic vasculitis in children, clinically presenting as palpable purpura in combination with arthritis, gastrointestinal involvement, or kidney injury. Subcutaneous edema is reported in patients with IgA vasculitis, and it commonly affects the lower extremities, especially around joints. Here, we report a case of IgA vasculitis with a rare presentation of edema isolated to the periorbital area in a 7-year-old boy, who subsequently developed crescentic glomerulonephritis with nephrotic range proteinuria. Isolated periorbital edema is an uncommon cutaneous feature of IgA vasculitis.

11.
HCA Healthc J Med ; 5(2): 157-164, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38984230

RESUMO

Introduction: Immunoglobulin A vasculitis (IgA)is a rare condition characterized by palpable purpura, often involving the skin, gastrointestinal tract, joints, and kidneys. Presentation is usually acute and is more common in children and adolescents of Southeast Asian and European descent. In the adult population, it is less common and therapies are not as well-established. Case Presentation: Disease prevalence of IgA vasculitis outside Southeast Asian and European populations is not well-documented. In this case series, we present 2 cases of IgA vasculitis in 2 older adult males, one of Native American descent and one of African American descent. Conclusion: IgA vasculitis must be considered within the adult population, and it is not limited to certain ethnic groups. Further research is needed to give clarity on the best treatment options for adults with IgA vasculitis. We believe that patients presenting with IgA vasculitis are best managed in a multidisciplinary approach, especially those patients with limited improvement despite the initiation of corticosteroids. Our 2 cases should raise awareness of IgA vasculitis in patients with skin rashes and elevated creatinine levels.

12.
Cureus ; 16(7): e64457, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-39007015

RESUMO

This systematic review aims to evaluate the therapeutic efficacy of Dihuang decoction (DD), anti-inflammatory drugs (AIDs), blood circulation improvement drugs (BCIDs), and conventional therapy (CT) in the management of Henoch-Schönlein purpura (HSP) and to establish their comparative effectiveness rankings. Using the Population, Intervention, Comparison, Outcome, Study (PICOS) design framework, we developed a detailed search strategy. The literature search included databases such as PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure, Wanfang Data, Weipu Journal Data, and the Chinese Biomedical Database, covering studies published up to June 2024. We included randomized controlled trials that featured the DD as the experimental intervention, with three remaining treatments as comparators. Our analysis encompassed 63 studies with 5,435 participants, divided into 2,817 in the experimental group and 2,618 in the control group. The network meta-analysis suggested that the DD potentially surpasses AIDs, BCIDs, and CT in the management of HSP. This conclusion is supported by its superior SUCRA (Surface Under the Cumulative Ranking) scores across various measures, including the overall effective rate of medication, time to relief or disappearance of the rash, incidence of adverse reactions, time to relief or disappearance of abdominal pain, time to relief or disappearance of arthritic swelling or pain, IgA levels, and the relapse rate within six months (SUCRA scores: 100.0%, 88.3%, 79.8%, 94.4%, 99.9%, 88.3%, and 95.4%, respectively). In terms of overall effectiveness rate, the SUCRA efficacy rankings are as follows: DD > AIDs > AIDs+BCID > BCID > CT. Regarding rash relief and regression time, the SUCRA efficacy rankings are as follows: DD > CT > AIDs+BCID > AIDs. For the incidence rate of adverse reactions, the SUCRA efficacy rankings are as follows: DD > CT > AIDs > BCID > AIDs+BCID. For the relief and disappearance of abdominal pain, the SUCRA efficacy rankings are as follows: DD > CT > AIDs+BCID > AIDs. In terms of relief and disappearance of joint swelling and pain, the SUCRA efficacy rankings are as follows: DD > AIDs+BCID > AIDs. Regarding IgA changes, the SUCRA efficacy rankings are as follows: DD > CT > BCID > AIDs+BCID > AIDs. For the six-month recurrence rate, the SUCRA efficacy rankings are as follows: DD > AIDs > AIDs+BCID > CT. The DD appears to be a more effective alternative for treating HSP compared to AIDs, BCIDs, and CT. We hope that this study will provide better assistance to clinical practice.

13.
Acta Med Indones ; 56(2): 218-226, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-39010779

RESUMO

Henoch-Schönlein purpura (HSP) is an immunoglobulin A (IgA)-mediated systemic vasculitis, which is one of the rare adverse reactions to hepatitis B vaccination. Low vitamin D levels were found to be present in the majority of HSP patients.A 19-year-old woman was admitted with a purpuric rash on bilateral lower limbs and joint pain on her left index finger in January 2020. A previous history of rash occurred one week after the patient received her first dose of recombinant hepatitis-B vaccination. Routine hematological examination, creatinine, urinalysis, C3, and C4 showed normal results. HBsAg, Anti-HCV, and ANA tests were negative, and anti-HBs were elevated. Vitamin D is very low. The patient was diagnosed with HSP and given mycophenolate mofetil, methylprednisolone, vitamin D3, and folic acid. Within 1 month of therapy, the rash still occurred frequently, so mycophenolate mofetil was changed to mycophenolic acid, the dose of methylprednisolone was increased and fexofenadine was administered. In the next 3 months, the rash has improved. However, patients reported knee joint pain and hair loss. In May 2021, the patient underwent tonsillectomy due to acute exacerbation of chronic tonsillitis. Thereafter, the patient reported that the rash had completely resolved and never worsened, and the vitamin D assay was normal.Hepatitis B vaccination is one of the etiologies of HSP, although it is rare, so it is important to ask about the vaccination history in patients with suspected HSP. Correction of vitamin D and performing tonsillectomy provide better treatment results in HSP cases in this patient.


Assuntos
Colecalciferol , Vacinas contra Hepatite B , Vasculite por IgA , Tonsilectomia , Humanos , Feminino , Vasculite por IgA/induzido quimicamente , Colecalciferol/administração & dosagem , Colecalciferol/efeitos adversos , Vacinas contra Hepatite B/efeitos adversos , Vacinas contra Hepatite B/administração & dosagem , Adulto Jovem , Tonsilectomia/efeitos adversos
14.
Cureus ; 16(6): e63462, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-39077295

RESUMO

Henoch-Schönlein purpura (HSP) also known as rheumatoid purpura is the most common vasculitis in children. This condition affects small blood vessels, predominantly targeting the skin, digestive system, joints, and kidneys. Short-term prognosis mainly depends on abdominal complications, while long-term prognosis is mainly determined by the severity of kidney involvement, which occurs in about 35% of cases. Although uncommon, other organs such as the lungs, heart, or nervous system may also be affected. Compartment syndrome of the hand and forearm is a very rare complication of HSP. To our knowledge, only two cases have been reported in the literature. We describe the case of a four-year-old child who presented with rheumatoid purpura complicated by compartment syndrome of the hand and forearm successfully managed through emergency fasciotomy.

15.
Front Microbiol ; 15: 1423951, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39027091

RESUMO

Introduction: A variety of studies have shown a link between the gut microbiota and autoimmune diseases, but the causal relationship with Henoch-Schönlein purpura (HSP) and immune thrombocytopenic purpura (ITP) is unknown. Methods: This study investigated the bidirectional causality between gut microbiota and HSP and ITP using Mendelian randomization (MR). Large-scale genetic data of gut microbiota at phylum to species level from the MiBioGen consortium and the Dutch Microbiome Project were utilized. Genome-wide association studies (GWAS) summary statistics for HSP and ITP came from FinnGen R10. Various MR methods were applied to infer causal relationships, including inverse variance weighted (IVW), maximum likelihood (ML), cML-MA, MR-Egger, weighted median, weighted model, and MR-PRESSO. Multiple sensitivity analyses and Bonferroni correction were conducted to enhance robustness and reliability. Results: Based on the IVW estimates, 23 bacterial taxa were identified to have suggestive associations with HSP and ITP. Remarkably, after Bonferroni correction, family Alcaligenaceae (OR = 2.86, 95% CI = 1.52-5.37; IVW, p = 1.10 × 10-3, ML, p = 1.40 × 10-3) was significantly associated with ITP as a risk factor, while family Bacteroidales S24 7group (OR = 0.46, 95% CI = 0.29-0.74; IVW, p = 1.40 × 10-3) was significantly associated with ITP as a protective factor. No significant associations between HSP and ITP and gut microbiota were found in reverse analyses. Conclusion: Our study provides evidence of causal effects of gut microbiota on HSP and ITP, highlighting the importance of further research to clarify the underlying mechanisms and develop targeted therapeutic interventions for these autoimmune diseases.

16.
Ir J Med Sci ; 193(5): 2397-2406, 2024 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-38967706

RESUMO

BACKGROUND: The compositional and structural changes of gut microbiota were closely related to the status of Henoch-Schönlein purpura (HSP). AIMS: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.


Assuntos
Microbioma Gastrointestinal , Vasculite por IgA , Humanos , Vasculite por IgA/microbiologia , Feminino , Masculino , Criança , Pré-Escolar , Fezes/microbiologia , RNA Ribossômico 16S/análise , Gastroenteropatias/microbiologia
17.
Proc (Bayl Univ Med Cent) ; 37(4): 655-658, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38910806

RESUMO

We describe the case of a 19-year-old woman who presented with abdominal pain, vomiting, and a palpable purpuric rash. The patient subsequently developed dysentery and was found to have an infection from Shiga toxin-producing Escherichia coli. The patient also met diagnostic criteria for IgA vasculitis (also known as Henoch Schönlein purpura) but had negative immunofluorescence biopsies of the rash. The patient was treated with steroids and achieved recovery. To our knowledge, this is the first documented case of IgA vasculitis in the setting of an enterohemorrhagic E. coli infection. This case highlights an atypical presentation of IgA vasculitis and the need to include small vessel vasculitis as a differential diagnosis when treating patients of all ages.

18.
Pediatr Int ; 66(1): e15781, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38863300

RESUMO

BACKGROUND: Immumoglobulin A (IgA) vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (HSP), is a self-limiting systemic vasculitis in children. Kidney involvement is associated with a long-term unfavorable outcome and can lead to significant morbidity. This study was conducted to describe the clinical and laboratory characteristics of childhood IgAV with kidney involvement and to identify risk factors associated with IgAV nephritis (IgAVN). METHODS: This was an ambidirectional descriptive study of 77 children with IgAV. All demographic data, clinical features, and laboratory tests were collected from electronic medical records from January 2010 to December 2022. Risk factors for kidney involvement in IgAV were assessed using multivariate logistic regression. Kaplan-Meier survival analysis was used to calculate the time to commencement of kidney involvement. RESULTS: Twenty-five children (32.4% of the IgAV patients) developed IgAVN. The common findings in IgAV with kidney involvement were microscopic hematuria (100%), nephrotic range proteinuria (44%), and non-nephrotic range proteinuria (40%). Multivariate logistic regression showed that age greater than 10 years (adjusted hazard ratio, AHR 4.66; 95% confidence interval, CI, 1.91-11.41; p = 0.001), obesity (body mass index, BMI, z-score ≥ +2 standard deviations, SDs) (AHR 3.59; 95% CI 1.41-9.17; p = 0.007), and hypertension at onset (AHR 4.78; 95% CI 1.76-12.95; p = 0.002) were associated significantly with kidney involvement. During follow up, most IgAV patients developed nephritis within the first 9 months. CONCLUSION: Age greater than 10 years, obesity, and hypertension at presentation were predictive factors for IgAVN. Our study emphasized that IgAV patients with risk factors should be closely monitored for at least 1 year after the onset of the disease.


Assuntos
Vasculite por IgA , Humanos , Masculino , Feminino , Criança , Fatores de Risco , Vasculite por IgA/complicações , Vasculite por IgA/epidemiologia , Vasculite por IgA/diagnóstico , Pré-Escolar , Adolescente , Estudos Retrospectivos , Proteinúria/etiologia , Proteinúria/epidemiologia , Estimativa de Kaplan-Meier , Hematúria/etiologia , Hematúria/epidemiologia , Modelos Logísticos , Rim/patologia , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/epidemiologia
19.
Gut Pathog ; 16(1): 28, 2024 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-38824586

RESUMO

BACKGROUND: Intussusception, a common cause of abdominal pain in children, often lacks clear underlying causes and is mostly idiopathic. Recurrence, though rare, raises clinical concerns, with rates escalating after each episode. Factors like pathological lead points and Henoch-Schönlein purpura (HSP) are associated with recurrent cases. On the other hand, the prevalence of Helicobacter pylori (H. pylori), often asymptomatic, in children has been declining. Although its infection is reported to be linked with HSP, its role in recurrent intussusception remains unexplored. Further research is needed to understand the interplay among H. pylori (culprit pathogen), HSP (trigger), and intractable intussusception so as to develop effective management strategies. CASE PRESENTATION: A two-year-old girl experienced four atypical episodes of intussusception at distinct locations, which later coincided with HSP. Despite treatment with steroids, recurrent intussusception persisted, suggesting that HSP itself was not a major cause for intractable presentations. Subsequent identification of H. pylori infection and treatment with triple therapy resulted in complete resolution of her recalcitrant intussusception. CONCLUSION: This instructive case underscored a sequence wherein H. pylori infection triggered HSP, subsequently resulting in recurrent intussusception. While H. pylori infection is not common in young children, the coexistence of intractable intussusception and steroid-resistant recurrent HSP necessitates consideration of H. pylori infection as a potential underlying pathogen.

20.
Front Pharmacol ; 15: 1339153, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38841368

RESUMO

Treatment of glomerulonephritis presents several challenges, including limited therapeutic options, high costs, and potential adverse reactions. As a recognized Chinese patent medicine, Tripterygium wilfordii poly-glycosides (TWP) have shown promising benefits in managing autoimmune diseases. To evaluate clinical effectiveness and safety of TWP in treating glomerulonephritis, we systematically searched PubMed, Cochrane Library, Web of Science, and Embase databases for controlled studies published up to 12 July 2023. We employed weighted mean difference and relative risk to analyze continuous and dichotomous outcomes. This meta-analysis included 16 studies that included primary membranous nephropathy (PMN), type 2 diabetic kidney disease (DKD), and Henoch-Schönlein purpura nephritis (HSPN). Analysis revealed that additional TWP administration improved patients' outcomes and total remission rates, reduced 24-h urine protein (24hUP) and decreased relapse events. The pooled results demonstrated the non-inferiority of TWP to glucocorticoids in achieving total remission, reducing 24hUP, and converting the phospholipase A2 receptor (PLA2R) status to negative. For DKD patients, TWP effectively reduced 24hUP levels, although it did not significantly improve the estimated glomerular filtration rate (eGFR). Compared to valsartan, TWP showed comparable improvements in 24hUP and eGFR levels. In severe cases of HSPN in children, significant clinical remission and a reduction in 24hUP levels were observed with the addition of TWP treatment. TWP did not significantly increase the incidence of adverse reactions. Therefore, TWP could offer therapeutic benefits to patients with PMN, DKD, and severe HSPN, with a minimal increase in the risk of side effects.

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