Diagnosis Of Henoch-Schonlein Purpura Research Articles

Varicella-associated disseminated intravascular coagulation secondary to Henoch-Schönlein purpura with renal and gastrointestinal system involvement in a child: A case report.

Immunocompromised patients who developed varicella-zoster virus (VZV)-associated disseminated intravascular coagulation (DIC) previously included recipients of bone marrow, hematopoietic stem cell, or organ transplantations, patients with primary nephropathy receiving corticosteroid therapy, cancer patients receiving chemotherapy, and patients with human immune deficiency virus infection. The case reported here is novel because, to our knowledge, there has been no report of VZV-associated DIC after the onset of Henoch-Schönlein purpura (HSP). To report the successful treatment of a novel pediatric case with VZV-associated DIC secondary to HSP. An 8-year-old girl developed VZV-associated DIC 24 days after diagnosis of HSP with renal and gastrointestinal involvement. She was treated with methylprednisolone at a local hospital for 19 days, and suddenly developed fever starting from day 4 in our hospital. Her fever persisted with vesicular skin rashes on her back, strong abdominal and lower back pain, epistaxis, hematochezia, erosion and bleeding on her lips, in her mouth and at puncture sites on day 5. She was diagnosed with DIC with the laboratory evidence of dramatically decreased platelet count and fibrinogen, prolonged activated partial thromboplastin time and prothrombin time, and increased fibrin degradation products including d-dimers. She also developed multiple organ dysfunction syndrome. On day 7, the patient VZV nucleic acid result turned out to be positive. Methylprednisolone treatment was discontinued, and she was given a multi-modality therapy including medications of acyclovir and antibiotics, intravenous gamma-immunoglobulin, various blood product transfusions, continuous renal replacement therapy, plasma exchange, and administration of liver and gastrointestinal system protection drugs. The patient multi-organ function damage gradually recovered. After VZV control, the patient was treated with oral methylprednisolone again for HSP with nephritis. Urine analysis was normal 1 year later, and oral hormone was discontinued. No complication or relapse occurred during 2 years of follow-up. This case report, for the first time, adds HSP treated with corticosteroids to the spectrum of clinical conditions that progressed to life-threatening secondary varicella-associated DIC. Early identification of varicella infection and DIC, combined with timely antiviral, immunoglobulin transfusion, plasma exchange, and other combined therapies are essential for saving patients' lives.

THE RELATIONSHIP BETWEEN INITIAL CLINICAL FINDINGS AND RENAL INVOLVEMENT OF HENOCH SCHÖNLEIN PURPURA IN PEDIATRIC PATIENTS

OBJECTIVE: Henoch-Schönlein purpura (HSP) is an IgA-associated systemic vasculitis characterized by three important clinical signs of non-thrombocytopenic palpable purpura, abdominal pain, and arthritis. Renal involvement generally determines the prognosis. In this study, it was aimed to determine the relationship between skin, gastrointestinal system (GIS), the severity of joint and renal involvement on the clinical features of children who were followed up with the diagnosis of HSP. MATERIAL AND METHODS: In this prospective cross-sectional study, 74 pediatric patients followed up between January 2011 and January 2012 were evaluated with their history, physical examination, laboratory measurements, treatment received during follow-up, and response to treatment. System involvement of the patients was classified as skin, renal, GIS, and joint involvement. RESULTS: It was determined that 45.9% of the cases were female and 54.1% were male. A significant correlation was found between the severity of arthritis and renal involvement in the first month (p<0.05). There was a significant correlation between the severity of abdominal pain and renal involvement at the time of admission, and also the severity of abdominal pain and severity of renal involvement at the sixth month (p<0.05). A significant correlation was found between the response of abdominal pain to painkillers and renal involvement in the first month (p<0.05). CONCLUSIONS: Severe joint and GIS involvement at the beginning of the disease and severe GIS involvement at the beginning were found to be associated with severe renal involvement in the first and sixth months. Patients who initially had severe abdominal pain, and therefore could not move at all, fall behind from the game, prefer to lie down continuously, and/or those with melena should be evaluated more carefully in terms of renal involvement.

A Case Study: Henoch-Schonlein Purpura in a Geriatric Patient with Gastrointestinal Involvement

IgA-mediated immune vasculitis known as Henoch-Schonlein purpura affects the small blood vessels in the joints, kidneys, GI tract, skin, and, less frequently, the brain and lungs. The present case report describes a 62-year-old female patient with Henoch-Schonlein purpura (HSP). HSP is a type of vasculitis that affects small vessels and is characterized by purpura, arthritis, abdominal pain, and kidney involvement. In this case, the patient presented with a rash with red lesions on the upper and lower limbs, crusted lesions on both lower limbs, and acute onset abdominal pain with hematochezia. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon(fig.1). Biopsies revealed a leukocytoclastic vasculitis in the skin (fig2) and gastrointestinal tract. The patient had a history of hypertension and smoking, and laboratory investigations revealed leucocytosis, anaemia, and electrolyte imbalances. The diagnosis of HSP was made based on the clinical presentation, laboratory findings, and biopsy results. The patient was treated with a combination of steroids, antibiotics, and supportive care, which led to significant improvement of symptoms and laboratory findings. Steroid therapy was given according to guidelines for the treatment of HSP, and tapering the steroid therapy helped to improve gastrointestinal symptoms. The case report highlights the importance of careful monitoring and appropriate treatment of HSP in geriatric patients, as well as the potential benefits of immunosuppressant therapy.

Effect of MEFV variants on the presentation and clinical course of Henoch-Schonlein purpura in children?

Objective: We aimed to evaluate MEFV mutation frequency and the effects of MEFV mutations on the clinical course including renal involvement in children with Henoch-Schonlein Purpura (HSP).
 Methods: Children with a diagnosis of HSP who were evaluated for the presence of MEFV mutations were enrolled in this study. Patients were primarily assigned into two groups based on the presence of MEFV mutations as Group 1 including patients without mutations and Group 2 including patients with mutations in at least one allele (heterozygous, homozygous, or compound heterozygous). We also investigated specifically the effects of M694V mutation on the course of HSP by comparing patients with M694V mutation in at least one allele with patients not carrying M694V mutation.
 Results: Forty-seven patients (23 female) were enrolled. MEFV mutation rate (53%) was 3.5 times the rate in general population. M694V was the most common mutation (48%). Patients with MEFV mutations, especially those with M694V mutation, had lower incidence of preceding infection, but increased inflammatory markers, scalp edema and relapse rate. Renal involvement and long-term prognosis were not affected by the presence of MEFV mutations.
 Conclusions: MEFV mutations cause susceptibility to develop HSP and are associated with increased inflammation and altered clinical course. However, renal involvement and long-term prognosis were not affected by the presence of MEFV mutations.

Articular involvement in Henoch-Schönlein Purpura: A review of literature.

Henoch-Schönlein purpura (HSP), the most typical kind of pediatric vasculitis, can also affect adults. Over the last 10 years, research has been increasing on improvements in HSP diagnosis, physiopathology, symptoms, and therapy. Joint involvement is highly frequent in this condition; however, it typically undergoes spontaneous resolution and does not lead to long-term complications. To provide a deeper understanding of the constituting pathogenic mechanisms and clinical presentation of articular involvement, focusing on the effect of neutrophil activation on systemic small vessels. This literature review utilized a systematic search of academic databases, employing specific keywords to select recent peer-reviewed articles and scholarly sources on the topic. The manifestations of joint involvement in HSP can vary in severity and frequency. Non-steroidal anti-inflammatory medications or acetaminophen are considered the first-line treatment for joint pain; however, corticosteroids may help achieve quick remission. In cases where standard treatment fails or manifestations persist, immunosuppressive drugs like rituximab, methotrexate, cyclophosphamide, or azathioprine have been used. While it tends to resolve without lasting joint damage, accurate diagnosis and appropriate management are crucial to ensure optimal patient outcomes.

Time of Onset and Risk Factors of Renal Involvement in Children with Henoch-Schönlein Purpura: Retrospective Study.

Background: Henoch-Schönlein purpura (HSP) is a common systemic vasculitis in children, involving the skin, musculoskeletal system, gastrointestinal tract and kidneys. Some studies in children have shown possible risk factors linked with the development and severity of HSP Nephritis (HSPN). The aim of this study was to research predicting factors for the development of HSPN. Methods: We retrospectively evaluated 132 pediatric patients with HSP, according to EULAR/PRINTO/PRESS criteria. All patients were screened for HSPN by urinalysis. Finally, we compared demographic, clinical and laboratory data in HSP patients with and without nephritis. Results: The median age at HSP diagnosis [6.2 (2.6–17.5) vs. 5.5 (0.8–15.4) years, p = 0.03] and the incidence of abdominal pain (48 vs. 27%, p = 0.01) were significantly higher in HSPN patients. No differences were evidenced regarding gender, allergic diseases, skin recurrences, gastrointestinal involvement, musculoskeletal involvement, scrotal involvement, and laboratory data (white blood cell count, neutrophil count, lymphocyte count, platelet count, C-reactive protein, erythrocyte sedimentation rate, and blood concentration of IgA). Conclusions: The age at diagnosis and abdominal pain were independent risk factors for renal involvement in HSP patients. However, due to the retrospective nature of this study, further long-term and prospective studies will be necessary.

Serum levels of degraded monosaccharides in children with Henoch-Schönlein purpura.

To examine the serum levels of degraded monosaccharides in children with Henoch-Schönlein purpura (HSP) and to study the clinical significance of degraded monosaccharides in HSP. A prospective analysis was performed on 132 children who were diagnosed with HSP from September 2019 to January 2022, and 132 healthy children were enrolled as the control group. High-performance liquid chromatography was used to determine the content of degraded monosaccharides in serum in both groups. The receiver operating characteristic (ROC) curve was used to evaluate the efficiency of degraded monosaccharides for the diagnosis of HSP. Compared with the control group, the HSP group had significantly higher serum levels of mannose, glucosamine, aminogalactose, and galactose (P<0.001). The four degraded monosaccharides had an area under the ROC curve of 0.919, 0.913, 0.832, and 0.932 respectively for the diagnosis of HSP (P<0.05). Children with HSP have higher serum levels of mannose, glucosamine, aminogalactose, and galactose than the healthy population. The levels of degraded monosaccharides may have an important value for the diagnosis of HSP.

Development and Validation of a Differential Diagnosis Model for Acute Appendicitis and Henoch-Schonlein Purpura in Children.

Objective: To study and develop a predictive model for the differential diagnosis of acute appendicitis (AA) and Henoch-Schonlein purpura (HSP) in children and to validate the model internally and externally. Methods: The complete data of AA and HSP cases were retrospectively analyzed and divided into internal and external verification groups. SPSS software was used for single-factor analysis and screening of independent variables, and R software was used for the development and verification of the diagnostic model. Lasso regression analysis was used to screen predictors and Lasso-logistic regression model was constructed, and K-fold cross-validation was used for the internal verification. In addition, nonfever patients were selected for model development and validation in the same way. Receiver operating characteristic (ROC) curves and calibration curves were drawn, respectively, to evaluate the 2 models. Results: Internal development and validation of the model showed that fever, neutrophil ratio (NEUT%), albumin (ALB), direct bilirubin (DBIL), C-reactive protein (CRP), and K were predictive factors for the diagnosis of HSP. The model was presented in the form of a nomogram, and the area under ROC curve of the development group and verification group was 0.9462 (95% confidence interval [CI] = 0.9402-0.9522) and 0.8931 (95% CI = 0.8724-0.9139), respectively. In the model of patients without fever, NEUT%, platelets (PLT), ALB, DBIL, alkaline phosphatase (ALP), CRP, and K were predictive factors for the diagnosis of HSP, and the area under ROC curve of the development group and verification group was 0.9186 (95% CI = 0.908-0.9293) and 0.8591 (95% CI = 0.8284-0.8897), respectively. Conclusion: In this study, 2 diagnostic models were constructed for fever or not, both of which had good discrimination and calibration, and were helpful to distinguish AA and HSP in children.

Henoch-Schonlein Purpura in a Newly Diagnosed HIV Patient.

HIV infection is associated with multisystemic manifestations due both to secondary infections caused by a decrease in the CD4+ T-cell count and to the pathogenicity of the HIV virus itself. A common renal manifestation is HIV-associated nephropathy, which is frequently seen in the African population with the APOL1 gene mutation; however, other forms of glomerulopathy such as IgA nephropathy, commonly noted in other ethnicities, are also seen. Vasculitis has rarely been associated with HIV infection and mainly involves small blood vessels, although any size of blood vessel may be involved. The association of Henoch-Schonlein purpura (HSP) with HIV is rare and not well understood. We describe a 53-year-old African American woman with a newly diagnosed HIV infection who presented with a purpuric rash over the bilateral lower extremities with haematuria. Initial work-up revealed renal dysfunction with elevated ESR. Urinalysis was positive for glomerular haematuria and sub-nephrotic range proteinuria. Serum complement level, c-antineutrophil cytoplasmic antibody (ANCA), p-ANCA and anti-nuclear antibody (ANA) were negative. Renal biopsy revealed mesangial IgA deposits with crescent glomerulopathy and fibrinoid necrosis, while skin biopsy revealed leucocytoclastic vasculitis. A diagnosis of HSP was made based on American College of Rheumatology (ACR) criteria. The patient’s renal function and purpura improved with a 5-day course of steroid pulse therapy. This case of HSP in a newly diagnosed HIV patient is unusual for the presence of crescentic glomerulopathy.LEARNING POINTSHenoch-Schonlein purpura (HSP) associated with HIV infection is uncommon but documented; however, all four features of HSP are rarely seen together.Crescent glomerulopathy is rarely seen in HIV-associated HSP.HSP associated with HIV is treated with antiretroviral drugs, while the role of steroid and immunosuppressive therapy remains controversial.

Real-time vascular and IgA dynamics during Henoch-Schönlein purpura by in vivo fluorescent microscopy.

Henoch-Schönlein purpura (HSP) is a typical cutaneous immune skin disease, usually diagnosed by invasive biopsy. In this study, we develop a noninvasive optical method by combining in vivo optical clearing, confocal microscopy and immune-staining together to present the real-time in vivo dynamics of blood vessels, IgA molecules, and T cells in a HSP rat model. The small vessels in the skin are found with acute damage and then hyperplasia, which enhances deposition of IgA complexes in blood vessels. The migrating T cells in blood vessels in HSP regions can be detected by setting fast line scanning in this method. Our method provides in vivo vascular, cellular, and molecular dynamics during HSP development and is thus of great potential in research and diagnosis of HSP and other skin diseases.

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

Epidemiological Study of Henoch-Schonlein Purpura and its Characteristics in Infected Children During 2009-2016

Introduction: Due to the different complications caused by Henoch-Schonlein purpura and in order to prevent additional treatment costs for patients, we decided to investigate the clinical features of cases of Henoch-Schonlein purpura. Materials and Methods: In this descriptive cross-sectional study, 52 children in whom the diagnosis of Henoch-Schonlein purpura was confirmed were enrolled in the study from 2009 to 2016. The instruments of this research include a researcher-made questionnaire with two parts. The first part is related to demographic characteristics including age, sex and season of referral and the second part was related to morbidity such as various symptoms. Data were collected from patients’ records and interviews with families and were analyzed using SPSS V.22. Results: Out of 52 patients, 30 (57.7%) were boys and 22 (43.3%) were girls. The mean age of patients was 3.02 to 6.58 years. Autumn and winter had the most clients of Henoch-Schonlein purpura. One of the most common manifestations seen in most patients was cutaneous manifestations. There was no significant relationship between gender and clinical manifestations and seasons of the year with these manifestations (p&gt; 0.05). The results showed that age was significantly associated with renal manifestations (P &lt;0.05). Conclusion: The results of this study showed that the prevalence of Henoch-Schonlein purpura disease is higher in boys. Cutaneous manifestations were seen in most patients and in patients with renal manifestations, the mean age was higher and significant. Keywords: Henoch-Schonlein purpura, clinical manifestations, complications, gender, age

Henoch-Schonlein Purpura in Northeast India: Peculiarities in Presentation

Background and Aim: Henoch-Schonlein purpura (HSP) is the most common childhood vasculitis characterized by leukocytoclastic vasculitis. This study was done to describe the presentation and immediate outcome of children admitted to HSP at our Institute. Methods: This retrospective study was conducted on children with HSP admitted to our department over a period of 7 years (January 2010 until December 2016). Results: Twenty-three children with a diagnosis of HSP were identified during the study period. The mean age was 9.4 years (4 years to 16 years). There were 15 girls and 8 boys with a male: female ratio of 1:1.9. The youngest child was 4 years old and most of the children (73.9%) were in the age group 5-12 years. Forty percent of the children presented between January and March. Major manifestations were rash (100%), joint pain (52%), renal involvement (52%), and abdominal pain (47.8%). Three (13.0%) children presented with systemic manifestations before the appearance of the rash. One child had MPGN 2 years before the onset of rash. There was no mortality. Most of the children recovered well; six (26%) had persistent hypertension and three (13%) had persistent proteinuria. Hypertensive emergency was seen in two children. One child had intussusception that resolved spontaneously. Conclusion: This study is the first study of Henoch Schonlein purpura from northeast India documenting certain peculiarities in the presentation. The results indicate a wide spectrum of presentations in HSP.

Characteristics of immune function in the acute phase of Henoch-Schönlein purpura.

Henoch-Schönlein purpura (HSP) is still diagnosed using symptoms and signs together with some histopathological findings. The purpose of this study was to summarize the characteristics and roles of cellular and humoral immunity in children with Henoch-Schönlein purpura (HSP). A total of 502 cases of patients with acute HSP were diagnosed and observed. The levels of T lymphocyte subsets, natural killer cells (NK cells), and B cells were analyzed by flow cytometry. The serum immunoglobulin G (IgG), immunoglobulin M (IgM), immunoglobulin A (IgA), and complement C3 (C3) and complement C4 (C4) levels were detected by velocity scatter turbidimetry. Compared with the healthy groups, the levels of cluster of differentiation 3 (CD3), cluster of differentiation 4 (CD4), B cells, and NK cells and ratio of CD4/CD8 in patients with HSP were decreased (P < 0.05). The levels of IgG, IgA, IgM, and C3 were increased (P < 0.05). Compared with the Kawasaki disease (KD) group, the levels of CD3, CD4, CD8, B cells, NK cells, and IgA in patients with HSP were increased (P < 0.05), and the ratio of CD4/CD8 and levels of IgM, C3, and C4 was decreased (P < 0.05). Compared with the pneumonia group, the levels of CD3, CD4, B cells, and IgA in patients with HSP were increased (P < 0.05), and the ratio of CD4/CD8 and levels of IgM and C4 was decreased (P < 0.05). Cellular immunity and humoral immunity were all involved in the pathogenesis of HSP. The decline of NK cells, B lymphocyte cells, CD3, CD4 the increased secretion of immunoglobulin, and the abnormal appearance of IgA and C3 may exist during the progression. It may indicate a worse prognosis and increasing the risk of dedifferentiation. Cellular immunity was lower, which lead to increased production of inflammatory mediators and increased secretion of immunoglobulin, which then mediated systemic small-vessel vasculitis. Key Points • The pathogenesis of Henoch-Schönlein purpura (HSP) was not completely illuminated. • There was a lack of disease-specific laboratory abnormalities that can be used in the clinical diagnosis of HSP. • We compared the laboratory abnormalities in the immune system of HSP with KD and pneumonia. • Cellular immunity and humoral immunity were all involved in the pathogenesis of HSP. Cellular immunity was lower, which lead to the following pathological changes.

Proteomic analysis of urine reveals biomarkers for the diagnosis and phenotyping of abdominal-type Henoch-Schonlein purpura.

BackgroundAbdominal-type Henoch-Schonlein purpura (HSP) is a common refractory disease in children. Currently, no specific diagnostic biomarker is available for HSP.MethodsChildren with abdominal type HSP were first diagnosed with three syndromes using Chinese traditional medicine. The urinary proteomes among the three syndromes of patients with abdominal type HSP and healthy controls were compared using two label-free proteomics quantifications, including data-dependent acquisition and data-independent acquisition.ResultsFor the comparison between patients with abdominal type HSP and healthy children, a total of 75 differential urinary proteins were identified by determining the overlap of the two experiments. The ingenuity pathway analysis (IPA) analysis showed that these differential proteins were correlated with the pathogenesis of abdominal type HSP. Of these, 37 proteins were distributed in 13 solid tissues as tissue-enriched proteins. Monitoring changes in these proteins might help us detect uncommon clinical manifestations of HSP. Patients with abdominal type HSP can be further distinguished into three syndromes based on the urine proteome. Finally, a panel of six urinary proteins (P25774, P09417, Q7Z5L0, P60900, P14550 and P09668) was constructed for both the diagnosis and phenotyping of abdominal type HSP.ConclusionsUrinary protein biomarkers for the diagnosis and phenotyping of abdominal type HSP were identified, which will contribute to the personalized treatment of patients with abdominal type HSP.

18660 Increased systemic symptoms in patients with positive direct immunofluorescence of skin biospies with Henoch-Schonlein purpura/IgA vasculitis: A retrospective chart review

Direct immunofluorescence (DIF) of skin biospies is an important tool in the diagnosis of Henoch-Schonlein purpura (HSP)/IgA vasculitis. In a retrospective chart review of IgA positive vasculitis patients seen by dermatology over the previous 5 years, we sought to determine whether presence of systemic symptoms classically seen in HSP correlated with a higher risk of renal disease. A total of 14 out of the 22 patients (63.63%) who underwent DIF staining of a skin biopsy tested positive for IgA. Of the patients that were positive for IgA on biopsy, 64.3% reported gastrointestinal symptoms, 28.6% had musculoskeletal involvement, and 14.3% reported fever.

Surgical management of small bowel-small bowel intussusception in Henoch-Schönlein Purpura

Abstract Henoch-Schonlein Purpura (HSP) is the most common vasculitis seen in children. It is characterized by rash, joint pain, and renal disease. Abdominal findings requiring surgical intervention are uncommon. A 13-year-old male presented to the emergency department with abdominal pain, new onset bilious emesis, and rash after three weeks of flu-like symptoms. Five days prior, he received treatment for strep pharyngitis from his primary care provider. A diagnosis of HSP was made after abdominal CT scan revealed signs of duodenal inflammation and a non-obstructing small bowel-small bowel intussusception. His abdominal pain persisted, and repeat ultrasound revealed persistence of the intussusception with concerns for decreased blood flow to the intussuscepted bowel. He was taken to the operating room for reduction of the intussusception. Vasculitic lesions were visualized within the duodenal mucosa and on the serosa of the small and large bowel. These rare operative images are presented below. The intussusception was reduced without need for resection. In HSP, intramural hemorrhage and submucosal inflammation can create lead points that trigger small bowel intussusceptions to occur. These intussusceptions are typically managed supportively and rarely require surgery. We present a case of HSP requiring surgery with depictions of both mucosal and serosal based vasculitis lesions.

S2300 Henoch-Schönlein Purpura Induced Terminal Ileitis Mimicking Crohn's Disease

INTRODUCTION: Henoch-Schönlein purpura (HSP), also known as immunoglobulin A (IgA) vasculitis, is an acute generalized immune complex-mediated small-vessel vasculitis. It characteristically presents with palpable purpura on buttocks and lower extremities. Renal dysfunction, joint involvement and gastrointestinal (GI) symptoms are also frequently seen. Although gastrointestinal involvement is common, it primarily shows up in the form of nonspecific symptoms such as abdominal pain, nausea, vomiting, diarrhea or constipation. Terminal ileitis associated with HSP has rarely been reported in the medical literature. CASE DESCRIPTION/METHODS: A 22-year-old male presented with bilateral knee arthralgia, abdominal pain and intractable nausea and vomiting accompanied by a non-blanching erythematous coalescing maculopapular rash on the dorsal aspect of the feet and around the ankles bilaterally (Figure 1). CT (Figure 2) showed fold thickening of proximal loops of jejunum and wall thickening and luminal narrowing of the terminal ileum extending several centimeters proximally. Fluid-filled mildly dilated small bowel loops measuring up to 3 cm in diameter were visualized. Additionally, significant abdominal and pelvic ascites was noted. There was no evidence of bowel obstruction. During colonoscopy (Figure 3), granular, erythematous, ulcerated, hemorrhagic and ecchymotic mucosa was visible in the terminal ileum. There was blood in the terminal ileum and the colon. However, no discrete areas of bleeding were identified. These findings were consistent with moderate to severe active terminal ileitis. A skin biopsy was performed which confirmed the diagnosis of HSP. Methylprednisolone was commenced and the patient experienced relief in his GI symptoms. DISCUSSION: Although terminal ileitis is the pathognomonic hallmark of Crohn’s disease, it is important to consider alternative causes such as HSP in the differential diagnosis of ileitis indicative of Crohn’s disease. The resemblance between inflammatory lesions in the terminal ileum in HSP and those seen in Crohn’s disease hints at a potential pathophysiological link between the two disorders which is supported by an emerging body of literature.Figure 1.: Non-blanching erythematous coalescing maculopapular rash on the dorsal aspect of both feet and around the ankles.Figure 2.: CT Abdomen & Pelvis Coronal Section: small bowel inflammatory changes involving the jejunum and ileum.Figure 3.: Colonoscopy showing moderate to severe active terminal ileitis.

Clinical and Demographic Features of 195 Pediatric Patients with Henoch-Schönlein Purpura, 12-Year Single Center Experience in Shiraz

Background: Henoch-Schönlein purpura (HSP) is one of the most common systemic types of vasculitis in children. Although it is a self-limited disease, life-threatening complications such as nephritis may occur. Early diagnosis and follow up might improve the long term outcome in renal involvement. There are few studies that have evaluated HSP in Iran. Objectives: The purpose of this study was to investigate demographic, laboratory data and clinical presentations of admitted HSP patients in a tertiary referral center, over a twelve-year period. Methods: This retrospective descriptive study evaluated 195 patients, diagnosed with HSP, who were admitted to Namazi Hospital in southwest of Iran (2006 - 2018). Demographic, clinical and laboratory findings, as well as treatment outcome of HSP patients were collected. Results: There were 118 males and 77 females with the mean age of 6.7 ± 3.21 years. About 70 (36%) patients showed common cold symptoms two weeks before HSP presentations. Admission course was 1 - 17 days (mean 4.55 ± 2.83) and autumn was recorded with the highest number of admitted patients (44.1%). In the course of hospitalization, 100% of the patients presented with palpable purpura, 61.02% with joint pain and 19.49% with abdominal pain. Moreover, 17.95% of the patients were noted with renal involvement. Laboratory data shows that more than half of patients (54%) had leukocytosis, only 9% of patients had positive CRP but all the patients had high erythrocyte sedimentation rate (ESR). Total of 43.1% of the patients received corticosteroids. Conclusions: The observed number of male patients with HSP was higher than females and the highest frequency of the HSP cases was observed in autumn. Joint pain and abdominal pain were the predominant clinical presentations, following skin purpura. The presented data can help with further HSP diagnosis and treatment plan.

Henoch-schönlein Purpura (HSP) in a patient on Abemaciclib

Henoch-Schonlein Purpura (HSP), also known as IgA vasculitis, is a small-vessel vasculitis associated with IgA-dominant immune deposits in the skin, gut and kidney [[1]Rigante D. Castellazzi L. Bosco A. Esposito S. Is there a crossroad between infections, genetics, and henoch-schönlein purpura?.Autoimmun Rev. 2013; 12: 1016-1021Crossref PubMed Scopus (69) Google Scholar]. It is usually characterized by a triad of symptoms including purpuric skin rash, abdominal pain or renal involvement and arthritis. In adults, HSP is seen in 3.4–14.3 cases per million [[1]Rigante D. Castellazzi L. Bosco A. Esposito S. Is there a crossroad between infections, genetics, and henoch-schönlein purpura?.Autoimmun Rev. 2013; 12: 1016-1021Crossref PubMed Scopus (69) Google Scholar]. Approximately two thirds of patients have a triggering event, most often an infection or an allergic reaction to drugs such as targeted therapies [[1]Rigante D. Castellazzi L. Bosco A. Esposito S. Is there a crossroad between infections, genetics, and henoch-schönlein purpura?.Autoimmun Rev. 2013; 12: 1016-1021Crossref PubMed Scopus (69) Google Scholar]. The cyclin-dependent kinases 4/6 (CDK4/6) inhibitors are a new class of drugs recently approved for the treatment of hormonal receptor positive/HER2 negative (HR+/HER2-) advanced breast cancer (BC) [[2]The role of CDK4/6 inhibitors in breast cancer. Conleth G. Murphy MB, BCh, BAO1,2.Curr Treat Options Oncol. 2019; volume 20 (Article number: 52)Google Scholar]. To date, no case report of HSP in patient on CDK4/6 inhibitors plus endocrine therapies has been described. In May 2019, an otherwise healthy 65-years-old female was diagnosed with stage IV HR+/HER2- BC with liver, lung and bone metastasis. Hypertension treated with ACE inhibitors was the only known comorbidity. From June 2019, she started first line treatment with a combination of CDK4/6 inhibitor (Abemaciclib 300 mg daily) and Letrozole. She was on treatment without relevant side effects and good clinical response. In October 2019, she presented to outpatient clinic referring one-day history of joint pain, severe asthenia, fever and spreading purpuric papules on lower limbs (Fig. 1A). Laboratory tests revealed a nephritic syndrome manifesting with a progressive renal failure (Creatinine 3,13 mg/dL), microhematuria and proteinuria. Over the next 6 hours, the purpura spread further over the sides and buttocks (Fig. 1B). Assuming a provisional diagnosis of HSP, the patient was started on intravenous dexamethasone 1 mg/kg body weight and systemic antibiotics, with clinical improvement. Blood cultures, serological and antiantibodies tests resulted negative. Renal biopsy was performed. Light microscopy revealed mesangial hypercellularity accompanied by diffuse IgA and C3 deposits in the mesangiu. The diagnosis of Abemaciclib-induced HSP was made. Finally, corticosteroid tapering was initiated and the patient was discharged after 15 days of hospitalization with complete normalization of renal function. One month later Abemaciclib was re-started at the lower dose (100 mg BID), without any other side effect. We speculate that the Abemaciclib could have triggered an abnormal IgA-mediated immune response against the small vascular walls endothelial cells [[3]Immunomodulatory roles of cell cycle regulators. Laphanuwat P and Jirawatnotai SImmunomodulatory roles of cell cycle regulators. Laphanuwat P and Jirawatnotai S (2019) Immunomodulatory Roles of Cell Cycle Regulators.Front. Cell Dev. Biol. 2019; 7: 23PubMed Google Scholar]. Recent research provides cumulative evidence for functions of cell cycle regulators in immune defense. Pre-clinical evidence reported an upregulation of select cytokines and chemokines (such as PD-L1, PD-L2), an increase of IFNγ-dependent enzymes and an upregulation of MHC class I and II in tumors treated with Abemaciclib [[4]Schaer D.A. Beckmann R.P. Dempsey J.A. Huber L. Forest A. Amaladas N. et al.The CDK4/6 inhibitor abemaciclib induces a T cell inflamed tumor microenvironment and enhances the efficacy of PD-L1 checkpoint blockade.Cell Rep. 2018; 22: 2978-2994Abstract Full Text Full Text PDF PubMed Scopus (147) Google Scholar]. Although uncommonly, HSP may follow treatment with Abemaciclib, which use is further increasing in BC treatment strategy. Hence, an increased awareness among oncologists could allow for early recognition and appropriate treatment. Informed consent was obtained from the patient included in the study. I declare that we have no conflict of interest.