Henoch-Schönlein Research Articles

Predictive Value of Laboratory Indexes on Renal Involvement in Children with Henoch-Schönlein Purpura.

ObjectiveHenoch–Schönlein purpura (HSP) is the most common vasculitis in children. Renal involvement is the main long-term complication of HSP, and presently there is no way to predict which children may have irreversible renal damage from the outset. This study aimed to explore the prediction value of laboratory indexes on renal involvement in children with HSP, which could help the early identification and intervention.MethodsChildren with HSP hospitalized at the First Affiliated Hospital of Henan University of Chinese Medicine from June 2019 to December 2020 were included. The demographic findings, clinical features, laboratory findings including blood routine examination, serum immunoglobulin, complement, T cell subsets levels, liver and kidney function, coagulation function were recorded. Laboratory indexes were analyzed, logistic regression analysis was performed to identify the independent predictors in HSP patients with renal involvement, and receiver operating characteristic (ROC) curves were further used to assess the value of prediction indexes, as well as the efficacy of combined diagnosis.ResultsThe study included 146 HSP patients, among them, 50 patients (34.2%) had renal involvement. Age, platelet distribution width (PDW), CD3+ and fibrinogen (FIB) were positively correlated with renal involvement, while the levels of Immunoglobulin G (IgG), C-reactive protein (CRP), and neutrophil/lymphocyte ratio (NLR) were negatively correlated with renal involvement. The area under the ROC Curve (AUC) of these biomarkers ranged from 0.6284 to 0.7009, and among the combinations, a combination of NLR, CRP, CD3+, FIB, PDW, IgG and age had the best AUC value (0.9774).ConclusionAge, PDW, CD3+, FIB, CRP, NLR and IgG were prediction indexes for renal involvement in HSP patients, and these indexes can be combined appropriately to improve the diagnostic efficacy.

CAVITARY PRIMARY PULMONARY LYMPHOPLASMOCYTIC LYMPHOMA COMPLICATING HENOCH–SCHÖNLEIN PURPURA

Non-Hodgkin lymphoma (NHL) may occur in the chest, often as secondary involvement but occasionally as primary disease. Low-grade pulmonary B-cell lymphoma is the most frequent form. The diagnosis based on histological examination of surgical samples. Henoch–Schönlein purpura (HSP) as a systemic vasculitis typically less commonly affects adults. Triggers including infections, medications and malignancy for HSP have been recognized. We report a patient presenting with HSP who had primary pulmonary lymphoplasmocytic lymphoma (PPLL) as an underlying malignancy. Case: 57-year-old male patient developed chest pain with a hemoglobin level 5.9g/dL. Symptoms resolved after erythrocyte transfusions. He has been diagnosed as having type 2 myocardial infarction. The detailed investigation contributed to warm autoimmune hemolytic anemia (AIHA) diagnosis. Steroid was started. He had high eryhtrocyte sedimentation rate. Further workup revealed bilateral multiple hilar lymphadenopathies and nodular cavitary pulmonary lesions on torax CT. The clinical picture and laboratory evaluation were not consistent with invasive fungal infection and tuberculosis. Purified protein derivative (PPD) skin test was negative. Bronchoalveolar lavage did not reveal any atypical cell and culture positivity. Thoracoscopic lymph node excision was performed. Histologic investigation showed plasma cells in the paracortical area with a slight increase in kappa to lambda ratio (3:1). A fine needle aspiration biopsy of lung tissue revealed lymphoplasmocytosis. PET-CT documented cavitary nodular lesions and hilar lympadenomegalies but no other suspicious lesion. Biopsy sample from one lesion sized 18 × 12 mm with SUVmax 5 revealed plasma cell infiltration with an IgG kappa phenotype. PPLL was diagnosed. Meanwhile AIHA responded to steroid but recurred during dose tapering. PPLL treatment with bortezomib and rituximab based regimen was decided. AIHA went in remission but relapsed after one year with HSP associated clinical picture. He had severe abdominal pain with intestinal wall thickness. Biopsy samples from kidney showed IgA vasculitis and from skin granular type of IgA and C3 deposition in the walls of small diameter vessels in the papillary dermis. Pulse steroid followed by cyclophosphamide controlled the clinical picture. We wished to highlight that in adults presenting with HSP may be a sign of underlying malignancy relapse.

M162 HEMORRHAGIC BULLOUS HENOCH-SCHÖNLEIN PURPURA - AN ATYPICAL PRESENTATION OF THE MOST COMMON CHILDHOOD VASCULITIS

Henoch-Schönlein Purpura (HSP) is the most common form of systemic vasculitis in children. This immune mediated Immunoglobulin A (IgA) vasculitis can have a diverse presentation, thus thorough workup should be considered when evaluating a pediatric patient with progressive rash and arthralgias.

PHÙ NỀ PHẦN MỀM DƯỚI DA GÂY BIẾN DẠNG MẶT Ở MỘT TRẺ BỊ BAN XUẤT HUYẾT HENOCH-SCHÖNLEIN

Introduction: Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children, characterized by purpuric rash in combination with gastrointestinal, joint, and renal manifestations. Subcutaneous edema in HSP is transient, common in the feet and hands, rare in other locations. Objectives: We reported a 6-year-old boy with HSP who presented with the characteristics purpura and bilateral ankle arthritis. Case report: He did not have abdominal pain and no abnormalities in urine test. After 3 days of treatment at the Department of Pediatrics, Bach Mai Hospital with oral prednisolone 1 mg/kg/day, the patient's arthritis improved, but he had severe edema causing disfiguring face. The characteristic of edema was non-pitting and painless. We remained the dose of prednisolone, subcutaneous edema gradually decreased and disappeared after 4 days. Conclusion: Subcutaneous edema in HSP can be found at any location, not only in common sites such as the feet and hands. Clinicians need to be aware of this manifestation to avoid confusion in diagnosis and treatment HSP. Keywords: Henoch-Schönlein purpura, subcutaneous edema, disfiguring face

Qianghuo Shengshi decoction exerts anti-inflammatory and analgesic via MAPKs/CREB signaling pathway

Ethnopharmacological relevanceTraditional Chinese Medicine Qianghuo Shengshi decoction (QSD) is widely used in the treatment of nervous headache, rheumatoid arthritis, sciatica, allergic purpura, and other clinical diseases in China. However, the underlying mechanisms of its anti-inflammatory and analgesic effects has not been elucidated. Aim of the studyThe aim of this study was to confirm the anti-inflammatory and analgesic effects and the underlying mechanism of QSD in vivo. In addition, this study was also to isolate and analyze the main active components of QSD by high performance liquid chromatography (HPLC). Materials and methodsIn this study, the acetic acid writhing test, hot plate test and ear swelling test and formalin test were carried out to explore the anti-inflammatory and analgesic effects of QSD. The doses were set to 7.8 g/kg, 15.6 g/kg and 31.2 g/kg body weight. Western blot was utilized to study further possible mechanisms of QSD. Moreover, the HPLC method was used to isolate and identify the components in the extraction of QSD. ResultsTwelve characteristic peaks were recognized in the HPLC spectrum, which all were the known compounds. The QSD exhibited dose-dependent effects in anti-inflammatory and analgesic aspects. Compared with model group, the writhing times of in groups of different doses of QSD (15.6 g/kg and 31.2 g/kg (oral administration = p.o.)) were reduced by 33.0% and 45.8% and indicated the QSD showed significant (p < 0.05) peripheral analgesic effect. QSD ((31.2 g/kg), p.o.) showed significant(p < 0.05) analgesic effect in the hot plate test. Inhibition rates of QSD ((15.6 g/kg and 31.2 g/kg), p.o.) in ear swelling test induced by p-xylene were 27.5% and 54.6% and demonstrated the significant (p < 0.05) anti-inflammatory activity. QSD ((31.2 g/kg), p.o.) significantly (p < 0.05) reduced times of paw licking in formalin test, and its inhibition rates were 34.3% and 28.0% in Phase I and Phase Ⅱ response, respectively. Western blot results showed that QSD inhibited the phosphorylation of mitogen-activated protein kinase (MAPK) protein and cAMP response element-binding protein (CREB). ConclusionsThese results of this study undoubtedly confirmed that QSD expressed obvious analgesic and anti-inflammatory activities. Anti-inflammatory and analgesic effects of QSD may be achieved by regulating the MAPKs protein and further regulating the expression of CREB. In all, QSD may play an anti-inflammatory and analgesic role through a variety of active ingredients.

Separating Fact from Fiction in the Diagnosis and Management of Food Allergy

Separating Fact from Fiction in the Diagnosis and Management of Food Allergy

S3024 Case of a Young Adult With Small Intestine Predominant Henoch Schonlein Purpura

Introduction: Henoch Schonlein Purpura (HSP) is the most common systemic vasculitis in young children, but is less commonly seen in adults. Diagnosis is based on the presence of palpable purpura without thrombocytopenia and with at least one other disease manifestation (arthritis, renal involvement, and/or abdominal pain). Gastrointestinal (GI) manifestations rarely precede the classic rash. Here we describe a young adult who initially presented with solely GI involvement of HSP. Case Description/Methods: A 25-year-old healthy male presented with two weeks of nausea, persistent sharp peri-umbilical abdominal pain of variable severity, and melena. Labs were notable for WBC 10.9 K/uL with neutrophilic predominance, CRP 66 mg/L, ESR 4 mm/hr, creatinine 0.88 mg/dL. Abdominal X-ray with mildly dilated gas-filled small bowel loops in central abdomen. CT abdomen/pelvis was unrevealing. EGD revealed antral-predominant nodular erythematous mucosal lesions in the stomach but otherwise normal appearing gastric mucosa (Fig 1a), histologically with surface hemorrhage. In the duodenum, diffuse erythematous mucosa with spontaneous hemorrhage was seen (Fig 1b), pathology consistent with duodenitis and erosions/ulcerations. Colonoscopy revealed right-sided erythema and granularity with biopsies showing non-specific inflammation. Interestingly, the day after endoscopic evaluation the patient developed bilateral lower extremity petechial rash (Fig 1c). Punch biopsies were diagnostic of IgA-mediated leukocytoclastic vasculitis. The overall presentation was consistent with HSP without renal involvement. Further work-up showed low C3 (consistent with HSP), negative ANA screen and ANCA, normal gastrin level, negative cryoglobulins, and unremarkable infectious work up. Discussion: HSP in adults is rare, especially if it presents with isolated GI disease. However, clinicians should maintain a high index of suspicion in adults with manifestations of small bowel inflammation. HSP mainly affects the small intestine, because the villi contain end-capillaries that get obstructed by immune complexes and cause necrosis. The rest of the GI tract is generally not involved by medium-vessel vasculitis due to its redundant circulation. The skin and epiphyseal plate have similar capillaries and are therefore frequently affected by HSP. Treatment with corticosteroids is controversial as most patients improve with supportive care. In this case, decision was made to treat with corticosteroids as patient returned with worsening disease activity.Figure 1.: a) Endoscopic view of gastric antrum/pylorus with multiple small nodular erythematous mucosal lesions; b) Endoscopic view of second portion of the duodenum showing diffuse erythematous mucosa with many erosive purple-hued mucosal lesions in a diffuse distribution with associated spontaneous bleeding; c) Classic purpuric rash on medial aspect of right foot.

Henoch–Schonlein Purpura in an Adult Presenting Atypically with Upper-Limb Cutaneous Involvement

Background: Henoch Schonlein Purpura (HSP) is a systemic vasculitis which manifests as a triad of skin rashes, joint pain and abdominal pain. The cutaneous manifestations include nonthrombocytopenic, nonblanching palpable purpura. The skin lesions are usually seen mainly in the lower limbs and this has been ascribed to the effects of gravity. The disease is common in childhood and relatively rare in adulthood. Case Report: A 60 year old female presented with two weeks history of rashes, abdominal pain, loose stools, knee and shoulder pain. The rashes were multiple and symmetrical and first noticed by her in left upper limb, within a day appeared on the other limb and lower limbs also. Skin biopsy report revealed leukocytoclastic vasculitis with neutrophilic infiltration and prominent nuclear fragmentation, involving the upper and middle layers of the dermis with IgA deposition on immunofluorescence. She was treated with Oral Prednisolone 20 mg BID. The rash had resolved at follow up with resolution of symptoms. The unique feature of this case was involvement of upper limbs and age of the patient. Conclusion: This case underlines the points that occasionally even the upper limb can be involved and even elderly adults can have this condition. There is a need for clinicians to be aware of the varied presentations of this condition.

STAPHYLOCOCCUS-ASSOCIATED GLOMERULONEPHRITIS MIMICKING IGA VASCULITIS IN AN ADULT

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Staphylococcus-associated glomerulonephritis (SAGN) is an immune complex–mediated glomerulonephritis (GN) which commonly presents with elevated creatinine, microscopic hematuria, and variable proteinuria; but on rare occasions may manifest as vasculitis that closely mimics Henoch-Schonlein Purpura (HSP). CASE PRESENTATION: A 62-year-old male with diabetes mellitus (DM) and liver cirrhosis presented with gangrene of the right hallux after a dog bite. Given concern for gas gangrene, he underwent Guillotine amputation. Blood cultures were positive for Methicillin sensitive staphylococcus aureus (MSSA). He was admitted to the ICU for septic shock and started on vasopressors and broad-spectrum antibiotics. His course was complicated by acute kidney injury (AKI) and a new onset diffuse palpable purpuric rash on day 3 of admission, for which a skin biopsy was performed. His serum creatinine was 1.4 mg/dl on admission and progressively worsened to 2.8 mg/dl requiring continuous renal replacement therapy (CRRT) initiation. Urinalysis showed 1+ protein with large blood and 20-40 red blood cells (RBC) per high power field. Urine microscopy revealed isomorphic RBC without red cell cast. Spot Urine protein-creatinine ratio was 0.4 mg/g. Immune work up was negative except for hypocomplementemia with C3 35 mg/dl, C4 8 mg/dl and CH50 19 unit/ml. Ultrasound abdomen showed cirrhosis. 2D-echocardiogram was negative for vegetations. Transesophageal echocardiogram and renal biopsy were not performed as patient was hemodynamically unstable. He remained critically ill and continued to decline despite initiation of CRRT and died due to refractory shock. Skin biopsy returned later, showing leukocytoclastic vasculitis with IgA deposition, confirming IgA vasculitis. DISCUSSION: SAGN is an immune complex mediated disease that occurs concurrently with staphylococcal infection. Pathophysiology is postulated to involve massive T cell and B cell activation by a staphylococcal superantigen. This leads to release of cytokines with overproduction of antibodies and systemic vasculitis with IgA-staphylococcal antigen immune complex formation and deposition. About 20% of the patients with SAGN present with leukocytoclastic vasculitis and can clinically mimic steroid responsive disease processes such as HSP or cryoglobulinemic vasculitis. Renal biopsy is essential to establish diagnosis as steroid and immunosuppression use in SAGN remain controversial and can worsen infection as well as renal disease. Unfortunately, renal biopsy could not be performed in this patient due to refractory shock; however, diagnosis of SAGN is being preferred owing to clinical characteristics like older age, DM, active MSSA infection and AKI with hypocomplementemia. CONCLUSIONS: It is important to make clinicians aware of the variable presentation of infection related GN, such as SAGN, HSP-nephritis, or post-streptococcal GN as treatment might differ. REFERENCE #1: 1. Akio Koyama, Kobayashi M et al. Glomerulonephritis associated with MRSA infection: A possible role of bacterial superantigen. Kidney International. Doi:10.1038/ki.1995.25 REFERENCE #2: 2. Tahrin Mahmood, Puckrin R et al. Staphylococcus-Associated Glomerulonephritis Mimicking Henoch-Schonlein Purpura and Cryoglobulinemic vasculitis in a patient with an epidural abscess: A case report and brief review of the literature. Can J Kidney Health Dis. 2018. Doi:10.1177/2054358118776325. DISCLOSURES: No relevant relationships by kalpana chintha, source=Web Response No relevant relationships by Stanley Dumond, source=Web Response No relevant relationships by Adam Green, source=Web Response No relevant relationships by Ananya Nanduri, source=Web Response No relevant relationships by Matk Sunga, source=Web Response

S3064 Henoch-Schӧnlein Purpura in the Context of Sars-CoV-2 Infection—Practice Management

Introduction: Henoch–Schönlein purpura (HSP), is a self-limiting vasculitis involving the small vessels of the skin, gastrointestinal tract, kidneys, joints, and rarely the lungs and central nervous system (CNS). The severe acute respiratory syndrome coronavirus-2 (Sars-CoV-2) infection may be a condition associated with the development of vasculitis sustained by an exacerbated IgA-mediated immune response. Case Description/Methods: In the context of the current pandemic, we report the case of a 32-year-old patient who showed clinical features of HSP that appeared in a few days after the diagnosis of Coronavirus Disease 2019 (COVID-19) mild form, confirmed by PCR test. He presented an acute onset with marked asthenia, edema and painful bilateral swelling of the knee, associated with difficulty walking. Small flat lesions distributed on the upper and lower extremity and on the posterior-chest have been identified, one day later. He needed hospitalization in a medical center where he received corticosteroid therapy, anticoagulant, antiviral with regression of skin lesions and joint swelling. After five days the skin lesions expand and after 48 hours there is intense abdominal pain accompanied by accelerated intestinal transit with blood. The objective examination reveals a rash on both lower extremity, which extends from the soles to the ankles (Figure 1). Examination of the knee joints shows no local erythema, but a painful limitation of passive movements is noted. Laboratory data indicate a biological inflammatory syndrome, proteinuria and hematuria. The immunological profile does not indicate the presence of non-COVID-19 reactive arthritis. Upper gastrointestinal examination performed at 4 days reveals gastric vascular spots. There are no evidence of suggestive lesions for inflammatory bowel disease (IBD) observed both on colonoscopy and abdominal CT based on 7 mm parietal thickening of the descending colon and sigmoid. Clinical, laboratory, imaging and endoscopic data establish the diagnosis of HSP with nephritic syndrome and reactive arthritis in the context of Sars-CoV-2 infection. Under cortisone treatment, purpura disappears completely after 10 days. Discussion: In our patient the presence of purpura in the absence of thrombocytopenia, as a mandatory criterion, in association with other criteria, namely arthritis reactive at onset, renal and digestive impairment, support according to EULAR / PRINTO / PRES criteria, the diagnosis of Henoch–Schönlein purpura.Figure 1

A POSSIBLE RELATIONSHIP BETWEEN SERUM HOMOCYSTEINE LEVEL AND IgA NEPHROPATHY IN CHILDREN

Objective: The evidences from experimental and epidemiological studies suggests that elevated serum homocysteine levels may lead to renal injury and may be a significant risk factor for the development of chronic kidney disease. The aim of this study was to investigate a possible relationship between serum homocysteine level and crescent formation in children with IgA nephropathy and Henoch-Schonlein purpura nephritis. Material and Methods: A total of 31 patients diagnosed as biopsy proven IgA nephropathy and Henoch-Schonlein purpura nephritis and idiopathic crescentic glomerulonephritis in three Pediatric Nephrology centers within the last five years and 25 healthy controls were enrolled in the study. Results: Homocysteine levels of patients were higher than the upper limit of normal value and also higher than the controls (p=0.0001). There was no significant difference between the patients with or without crescent formation regarding homocysteine levels (p&gt;0.05). Presence or severity of proteinuria was not related to homocysteine levels (p&gt;0.05). Conclusion: Serum homocysteine levels are elevated in patients with IgA nephropathy and Henoch-Schonlein purpura nephritis.&nbsp;Our results suggest that elevated serum homocysteine levels may be related to segmental glomerulosclerosis in these patient groups.

Ураження суглобів при пурпурі Шенлайна-Геноха

Актуальність. У переважного числа хворих геморагічний васкуліт або пурпура Шенлайна-Геноха (ПШГ) у дитячому віці дебютує із суглобового синдрому, що належить до діагностичних критеріїв цього захворювання. Характер артриту в пацієнтів після транс­формації хвороби з ювенільної ПШГ залишається невивченим. У дорослому віці суглобова патологія може мати вигляд мігруючого інтермітуючого або хронічного поліартриту, але ці відомості вимагають уточнення. Мета дослідження: вивчити клініко-рентгено-сонографічні ознаки перебігу суглобового синдрому у хворих на ПШГ і оцінити особливості артропатії при захворюванні, що почалося в дитячому й дорослому віці. Матеріали та методи. Під наглядом перебували 174 хворих на ПШГ (52,9 % чоловіків і 47,1 % жінок). У 92 пацієнтів васкуліт дебютував у дитячому віці (у середньому в 12 років), а у 82 — у дорослому (у 25 років). І, ІІ і ІІІ ступені активності патологічного процесу встановлено в співвідношенні 1 : 2 : 2. Результати. Ураження суглобів спостерігалось у кожного другого хворого на ПШГ, що тісно пов’язано з віком пацієнтів у дебюті хвороби, тривалістю й активністю патологічного процесу, серопозитивністю за високим вмістом імуноглобуліну A й ревматоїдного фактора, до того ж клінічний, рентгенологічний і сонографічний характер перебігу суглобового синдрому (залучення променезап’ясткових, кульшових і крижо-клубових з’єднань, розвиток асептичних остеонекрозів, остеоузур, менісцитів та інтраартикулярних хондромних тіл) залежить від екстрасуглобових проявів захворювання (уражень серця, нирок, підшлункової залози, центральної нервової системи), супроводжується підвищенням концентрацій у крові β2-мікроглобуліну й циркулюючих імунних комплексів, що разом зі значеннями фібриногенемії мають прогностичну значущість, а диморфізм ПШГ, що трансформована з ювенільної, виявляється меншим сироватковим рівнем циркулюючих імунних комплексів і ревматоїдного фактора. Висновки. Зміни з боку суглобів, незалежно від віку хворих на ПШГ у дебюті захворювання, відмічаються з однаковою частотою в кожному другому випадку, але в дорослих пацієнтів артропатія перебігає тяжче з приєднанням додаткових клінічних, рентгенологічних і сонографічних ознак.

Terminal Ileitis: A Rare Complication of Henoch-Schönlein Purpura in Children

Henoch–Schönlein purpura (HSP) is characterized by nonthrombocytopenic palpable purpura, arthritis or arthral­gia, and gastrointestinal and/or renal involvement. Gastrointestinal symptoms are reported in 50%-75% and they are related to a previous group A streptococcal infection in 40%. A healthy 5-year-old girl presented with a three-week history of a recurrent purpuric rash on the lower limbs, ar­thralgia and angioedema, without renal involvement. During the third relapse, she had severe, diffuse and persistent abdominal pain and bloody stools. An abdominal ultrasound revealed transmural edema of the last ileal segment, compatible with ileitis. She received prednisolone for five days, with full clinical recovery. Antistreptolysin O titer was elevated. The remaining laboratory tests were normal (antinuclear, anti-neutrophil and anti-Saccharomyces cerevisiae antibodies; rheumatoid factor; stool cultures, parasitological examination and viral antigen tests). One month later, an abdominal ultrasound revealed no abnormalities. Terminal ileitis is a very rare complication of HSP in children but has a good prognosis.

Variations of tongue coating microbiota in children with Henoch-Schönlein purpura nephritis

BackgroundVariations in the oral microbiota have been significantly correlated with the progress of autoimmune diseases, such as immunoglobulin A nephropathy and Henoch-Schönlein purpura (HSP). However, there is no report outlining the character of tongue coating microbiota variations in children with Henoch-Schönlein purpura nephritis (HSPN). MethodA total of 20 children with HSPN and 14 healthy controls were recruited for this research. Tongue coating samples of two groups were collected for 16S rRNA gene sequencing. The diversity, principal component analysis (PCA), nonmetric multidimensional scaling (NMDS), partial least squares discriminant analysis (PLS-DA), and linear discriminant analysis (LDA) effect size (LEfSe) were performed. Microbial function was assessed using the PICRUST. ResultsThe ACE and Chao indices were greatly lower in the HSPN group than in the HG (P = 0.001). The Shannon and Simpson indices were dramatically reduced in children with HSPN compared with those in the healthy controls (P = 0.005). Bacteroidales, Selenomonadales, Lactobacillales, Fusobacteriales, Neisseriales, and Actinomycetales composed more than 80% of all sequences, while Bacteroidales was the most generous order in both groups. PCA, NMDS and PLS-DA showed a marked difference between the control and HSPN groups. LEfSe analysis showed alteration of tongue coating microbiota in the HSPN group. There were 30 metabolic functions significantly differed between the two groups. ConclusionsChildren with HSPN have substantially various tongue coating microbiota compared to healthy controls. Even though this research does not indicate causality, it is beneficial to enhance the possibility for coming microbial-based treatments to enhance the clinical effects of HSPN in children.

Altered B cell compartment associated with Tfh cells in children with Henoch-Schonlein Purpura

AimIgA-producing B cells have been found to be associated with children diagnosed with Henoch-Schonlein purpura (HSP). The aim of the present study was to determine whether children with HSP possess altered B-cell subsets.MethodsA total of 14 children diagnosed with HSP and age- and sex-matched healthy controls (HCs) were enrolled in our study. Peripheral blood mononuclear cells were isolated, and the percentage and absolute number of B-cell subsets and Follicular helper T (Tfh) cells were determined by flow cytometry. Finally, Spearman’s correlation coefficient was used to analyse the correlation between the percentage of Tfh cells and B-cell subsets.ResultsWe found that compared to HCs, the frequency and absolute number of total B cells were significantly higher in children with HSP, but the percentages of plasma cells and naïve B cells were significantly lower. A significantly increased percentage and absolute number of memory nonswitched B cells were found in children with HSP compared with HCs. We observed that the expression of C-X-C chemokine receptor type 5 (CXCR5) on total CD4+ T cells and the percentage of CD4+CXCR5+ cells were significantly increased in patients with HSP. Moreover, significantly correlations between Tfh cells and various B-cell subsets were observed.ConclusionsOur study showed a Tfh-cell-associated altered B cell compartment in children with HSP.

Ураження нирок при геморагічному васкуліті Шенлейна — Геноха, що почався в дитячому і дорослому віці

Метою роботи стала порівняльна оцінка клініко-лабораторних і морфологічних проявів ниркової патології при геморагічному васкуліті Шенлейна — Геноха, що почався в дитячому та дорослому віці. Ураження нирок відзначено у 71 % від числа хворих (незалежно від віку пацієнтів у дебюті хвороби нефропатія зустрічалася з однаковою частотою), серед яких нефротичний синдром сформувався у 4 % випадків, хронічна хвороба нирок II, III, IV i V стадій — у 28 % спостережень (співвідношення 10 : 5 : 1 : 1), причому, за даними нефробіоптатів, у пацієнтів із трансформацією хвороби з ювенільної виявилися меншими ступені ураження ниркових артеріол, клубочків, канальців й строми, нижчими темпи прогресування ниркової недостатності, рівні артеріального тиску та периферійного судинного опору, а в цілому дебютний вік хворих впливає на подальшу частоту IV, V і VI класів генохівського гломерулонефриту, визначає рівень лімфогістіоцитарної інфільтрації судинних стінок й депозицій імуноглобулінів в клубочках та судинах.

An Assessment on the Effectiveness of the Immature Granulocyte Percentage in Predicting Internal Organ Involvement Among Children With Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP) is the most common childhood systemic vasculitis. The present study aims to investigate the effectiveness of the immature granulocyte (IG) percentage as a new marker for predicting internal organ involvement in HSP. This study included 75 patients below 18 years old who were diagnosed with HSP. The mean age was 7.48±2.77 years. The male/female ratio was 1.14. The findings showed that 35 (46.7%) of the patients had an internal organ involvement. The mean IG percentage was 0.88±0.68 among the patient group with HSP internal organ involvement, while it was 0.31±0.15 in the group without internal organ involvement, and a significant difference was determined between the 2 groups (P=0.000). The findings showed that the patients with renal involvement had the highest mean IG percentage (IG; 1.00±0.21). When the cutoff value for the IG percentage was specified as 0.45 to predict internal organ involvement, the sensitivity was 77.1%, and the specificity was 85%. In this study, the findings showed that IG percentage increased among patients with internal organ involvement in HSP and that its sensitivity, specificity, and predictive values were higher in predicting internal organ involvement compared with other markers.

Study on the Application Effect of Children Nursing in Allergic Purpura

Study on the Application Effect of Children Nursing in Allergic Purpura

Neutrophil-to-lymphocyte Ratio: A Biomarker for Predicting Systemic Involvement in Henoch-Schonlein Purpura.

Henoch-Schonlein purpura (HSP) is one of the commonest entities included within the category of cutaneous vasculitis (CV). Our work is purposed to explore the predictive value of neutrophil-to-lymphocyte ratio (NLR) for systemic involvement in Henoch- Schonlein purpura patients. This ratio is known as an inflammatory marker, and is used to assess the systemic inflammation associated with various diseases. Our objective is to establish whether it can be applied for the prediction of renal and gastrointestinal (GI) or purely renal involvement in Henoch-Schonlein purpura. To determine the relationship between neutrophil-to-lymphocyte ratio and systemic involvement in Henoch-Schonlein purpura Methods: This is a retrospective review of the patients who were diagnosed with Henoch-Schonlein purpura in our hospital between 2012 and 2018. A total of 57 patients met our inclusion criteria. Pre-treatment neutrophil-to-lymphocyte ratio was significantly associated with renal and/or GI manifestations of the disease (p<0.001). The optimal cut-off value of this ratio for predicting systemic involvement was 2.48, with a 95% specificity and a 94% sensitivity. In addition, pretreatment ratio was also found to be significantly correlated with the severity of relevant systemic manifestations of Henoch-Schonlein purpura (r=0.831; p<0.01). The small number of patients recruited for our research, its retrospective design, and the inclusion of patients attending the same hospital. This study suggests that neutrophil-to-lymphocyte ratio is suitable as a potential indicator for predicting the systemic involvement in Henoch-Schonlein purpura.

Henoch Schonlein purpura in pediatric patients with inflammatory bowel disease: A case series.

Retrospective chart review was conducted to identify the clinical features of Henoch Schonlein purpura (HSP) in five children with inflammatory bowel disease (IBD). All five children, four of which were on anti-TNF therapies, experienced the onset of HSP after their IBD diagnosis. HSP averaged 20.8months in duration. The patients in our cohort, particularly those on anti-TNF therapy for inflammatory bowel disease, experienced chronic and recurrent courses of HSP.