Vasculitis In Children Research Articles

Henoch-Schönlein purpura in Saudi Arabia: characteristics and rare vital organ involvement.

Henoch-Schönlein purpura (HSP) is the most common small vessel vasculitis in children with an annual incidence of between 10 and 30 per 100,000. It is an inflammation that affects many organs primarily in the skin, gastrointestinal (GI) tract, musculoskeletal, also vital organs like kidneys and lungs which may lead to chronic kidney disease and pulmonary hemorrhage. In this review, we are showing the characteristics of Saudi patients with HSP and the relation with preceding infections, gender, and seasonal variations in addition to the common and most severe systemic involvements. We found cases that were challenging to diagnose and others with unfortunate fetal complications.

Kawasaki syndrome: role of superantigens revisited.

Kawasaki syndrome (KS) is an acute vasculitis in children complicated by the development of heart disease. Despite its description over 50years ago, the etiology of coronary artery disease in KS is unknown. High dose intravenous immunoglobulin is the most effective approach to reduce cardiovascular complications. It remains unclear why patients with KS develop coronary artery aneurysms. A subset of patients is resistant to immunoglobulin therapy. Given the heterogeneity of clinical features, variability of history, and therapeutic response, KS may be a cluster of phenotypes triggered by multiple infectious agents and influenced by various environmental, genetic, and immunologic responses. The cause of KS is unknown, and a diagnostic test remains lacking. A better understanding of mechanisms leading to acute KS would contribute to a more precision medicine approach for this complex disease. In the current viewpoint, we make the case for microbial superantigens as important causes of KS.

Kawasaki disease in siblings in close temporal proximity to each other-what are the implications?

Kawasaki disease (KD) is the commonest medium vessel vasculitis in children. The etiology of KD remains an enigma despite extensive research. Infections are considered to be one of the triggers for KD, especially in genetically susceptible hosts. KD occurring within a short time interval among siblings is an important clinical observation supporting this hypothesis. In addition, siblings of children with KD are at a higher risk of developing the disease as compared with other children. Screening for KD in febrile siblings, therefore, seems prudent. This would help initiate timely therapy and prevent complications. We briefly review 16 English language reports of KD in siblings diagnosed within 1 month of each other to highlight its etiological and therapeutic implications.Key Points• KD should be suspected in febrile children who have a sibling recently diagnosed with KD.• Etiological studies should also focus on siblings who develop KD in close temporal proximity.

Minocycline-induced antineutrophil cytoplasmic antibody-associated vasculitis in children

Chronic exposure to minocycline, a commonly used anti-acne medication, results in a variety of autoimmune syndromes. Minocycline-induced vasculitis often associated with the presence of antinuclear antibody (ANA) and antineutrophil cytoplasmic antibody (ANCA) has been reported in Western literature, mainly in adults. Herein, we report a rare case of a 15-year-old male child on minocycline treatment for 1 year, who presented with fever, skin rash, and polyarthralgia for 4 months. His erythrocyte sedimentation rate (ESR) level was elevated, ANA and myeloperoxidase ANCA were positive, and skin biopsy revealed leukocytoclastic vasculitis. Upon discontinuation of minocycline and prescribing a short course of oral steroids, all his symptoms resolved and ESR normalized within 2 weeks. The child remained asymptomatic with normal acute-phase reactants at 2-month follow-up. This outcome suggested that minocycline was the main cause of ANCA-associated vasculitis.

Multisystemic manifestations of IgA vasculitis.

Immunoglobulin A vasculitis (IgAV), also known as Henoch-Schönlein Purpura, is one of the most common kind of systemic vasculitis in children, and due to the involvement of small blood vessels throughout the body, this disease can cause a variety of symptoms in different organs. Our aim was to review the data on various systemic manifestations of IgAV. A research of the literature was performed in PubMed database, utilizing the MeSH terms "IgA vasculitis" and "Henoch Schönlein Purpura". According to the predetermined structure of the manuscript, we extracted and sorted out the relevant data. Clinically, almost all the patients will present with palpable skin purpura, together with arthritis, gastrointestinal tract involvement, or kidney damage. Other rare systemic manifestations include neurological symptoms, scrotal involvement, and cardiopulmonary disease. When uncommon complications occur, patients may be misdiagnosed as other diseases, thus delaying treatment. Although the course of IgAV is mostly self-limited, misdiagnosis can also lead to a poor prognosis. A comprehensive awareness to the clinical manifestations of IgAV is the necessary prerequisite for its timely diagnosis. Prompt diagnosis and adequate treatment are essential for optimal results.

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.

AB1009 THE EFFICACY OF TOCILIZUMAB ON THE TREATMENT OF TAKAYASU ARTERITIS IN CHINESE CHILDREN

Background:Takayasu arteritis (TA) is the most prevalent large-vessel vasculitis in children. Patients with TA have a high mobidity and mortality.It remains a therapeutic challenge because corticosteroids monotherapy can rarely cure TAK and the relapse rate is high during GC tapering.Objectives:The aim of this study is to investigate the efficacy and safety of tocilizumab (TCZ)in Chinese children with Takayasu arteritis(TAK).Methods:We retrospectively studied 6 TAK children treated with TCZ in our hospital from July 2017 to October 2018. The demographic and clinical data, laboratory examination results and vascular imaging data were collected.Results:Six pediatric patients with critical or refractory TAK treated with TCZ were analyzed, including 3 males and 3 females.The diagnosis age was ranging in age from 2 to 13 years(median age:7 years).Three patients were initially treated with TCZ and Mycophenolate Mofetil(MMF) as the first-line regimen without corticosteroid or with a quite rapid GC taper duration,two of which had lifte-threatening coronary arteries involved and heart failure.The other three paitients were swcithed to TCZ from conventional disease modifying anti-rheumatic drugs (DMARDs) or other biologics due to being refractory to them and recurrent relapses.Four patients were given TCZ at 4 weeks regular intervals for 10 to 22 months,while two patients withdrew TCZ because of disease deterioration and unbearable abdominal or chest pain after the second dose.After 6 months follow-up,four patients experienced significant clinical and biological improvement with angiographically progression in one patient. A corticosteroid-sparing effect is obvious. Drug-related side effects occur in 1 patients manifesting as a mild elevated liver fuction. Neither neutropenia nor infection was observed.Conclusion:Our study shows a clinical, biological, and radiological response in patients with refractory TAK treated with TCZ.References :[1]Hellmich B, Agueda A, Monti S,et al.2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2019;0:1–12. doi:10.1136/annrheumdis-2019-215672.[2]BravoMancheño B, Perin F, Guez Vázquez Del ReyMDMR, García Sánchez A, Alcázar Romero PP. Successful tocilizumab treatment in a child with refractory Takayasu arteritis.Pediatrics 2012;130(6):e1720-724.[3]Goel R, Danda D, Kumar S, Joseph G. Rapid control of disease activity by tocilizumab in 10 «difficult-to-treat» cases of Takayasu arteritis. Int J Rheum Dis 2013;16(6):754–61.[4]Cañas CA, Cañas F, Izquierdo JH, Echeverri A-F, Mejía M, Bonilla-Abadía F, et al. Efficacy and safety of anti-interleukin 6 receptor monoclonal antibody (tocilizumab) in Colombian patients with Takayasu arteritis. J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis 2014;20(3):125–9.[5]Batu ED, Sönmez HE, Hazirolan T, Özaltin F, Bilginer Y, Özen S. Tocilizumab treatment in childhood Takayasu arteritis: case series of four patients and systematic review of the literature. Semin Arthritis Rheum 2017 Feb;46(4):529–35.Disclosure of Interests:None declared

Henoch-Schönlein Purpura in Children: An Updated Review.

Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic smallvessel vasculitis with a predilection for the skin, gastrointestinal tract, joints, and kidneys. It is the most common form of systemic vasculitis in children. The study aimed to familiarize physicians with the etiopathogenesis, clinical manifestations, evaluation, and management of children with Henoch-Schönlein purpura. A PubMed search was conducted in January 2020 in Clinical Queries using the key terms "Henoch-Schönlein purpura" OR "IgA vasculitis" OR "anaphylactoid purpura". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. This paper is based on, but not limited to, the search results. Globally, the incidence of HSP is 10 to 20 cases per 100, 000 children per year. Approximately 90% of cases occur in children between 2 and 10 years of age, with a peak incidence at 4 to 7 years. The diagnosis should be based on the finding of palpable purpura in the presence of at least one of the following criteria, namely, diffuse abdominal pain, arthritis or arthralgia, renal involvement (hematuria and/or proteinuria), and a biopsy showing predominant IgA deposition. Most cases are self-limited. The average duration of the disease is 4 weeks. Long-term complications are rare and include persistent hypertension and end-stage kidney disease. Therapy consists of general and supportive measures as well as treatment of the sequelae of the vasculitis. Current evidence does not support the universal treatment of HSP patients with corticosteroids. Oral corticosteroids may be considered for HSP patients with severe gastrointestinal pain and gastrointestinal hemorrhage. Most cases of HSP have an excellent outcome, with renal involvement being the most important prognostic factor in determining morbidity and mortality. Unfortunately, early steroid treatment does not reduce the incidence and severity of nephropathy in children with HSP. In HSP children who have severe nephritis or renal involvement with proteinuria of greater than 3 months, an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker should be considered in addition to corticosteroids to prevent and/or limit secondary glomerular injury.

Correlation of gut microbial compositions to the development of Kawasaki disease vasculitis in children.

Aim: Here, we hypothesize that dysbiotic gut microbiota might contribute to the development of Kawasaki disease (KD), a pediatric disease with unknown etiology. This is the second report on gut microbiota composition in KD patients. Materials & results: 16S amplicon sequencing was performed on fecal DNA samples and revealed predominance of bacterial pathogens, such as Fusobacterium, Neisseria, Shigellaand Streptococcus, in the gut of KD patients, but absent or suppressed after immunoglobulin/antibiotics therapy. In addition, beneficial bacteria propagated after the therapy. Conclusion: We conclude that prevalence of Fusobacteria, Shigellaand Streptococcus might contribute to KD pathogenesis.

IgA vasculitis with nephritis in children.

Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (HSP), is the most common form of systemic vasculitis in children. Although the first case of IgAV was described more than 200 years ago, its etiology still remains unclear. Nephrological symptoms are observed in 30-50% of children during the course of the disease, and in up to 91% of cases within 6 weeks of the onset of the first symptoms. Whereas other organ manifestations of IgAV are mostly benign and self-limiting, nephritis may lead to chronic kidney disease (CKD) and end-stage renal disease (ESRD). Appropriate treatment commenced early enough can stop the disease progression. However, even in severe cases there are no evidence-based guidelines, which makes the therapeutic decisions more difficult. In this article, which is the most up-to-date overview regarding IgAV, we discuss the disease's pathogenesis, the clinical forms of renal involvement in the course of the disease, the risk factors for adverse prognosis and treatment options in accordance with current recommendations.

Serum IP-10 and IL-17 from Kawasaki disease patients induce calcification-related genes and proteins in human coronary artery smooth muscle cells in vitro

BackgroundKawasaki disease (KD) is one of the major causes of heart disease and vasculitis in children under 5 years old in the world. Clinical evidence has shown that coronary artery calcification may develop in KD patients, however the mechanism has not been elucidated. Previous studies have found that interferon-γ-inducible protein (IP)-10 and interleukin (IL)-17 can be elevated and may play a role in KD development and coronary artery lesion formation. The purpose of this in vitro study was to investigate the possible role of plasma circulating IP-10 and IL-17 of KD patients in vascular calcification development and its underlying mechanism.ResultHuman coronary artery smooth muscle cells (HCASMCs) were used in this study. We found that HCASMCs treated with IP-10/IL-17-containing KD serum and co-treated with IP-10/IL-17 recombinant proteins could induce a phenotype that may promote vascular calcification by the bone morphogenetic protein (BMP) 6 autocrine effect. Moreover, the BMP6 autocrine stimulation in IP-10/IL-17 co-treated HCASMCs could upregulate the smad1/5-runx2 signaling activation, thus increasing the expression of bone matrix-related proteins, i.e., osteopontin, osteocalcin, and alkaline phosphatase.ConclusionsThe presented in vitro results provided new insights into the comprehension of the pathogenesis of vascular calcification in SMCs in KD progression. Although additional in vivo experimental models should be completed to confirm the in vivo relevance of these in vitro findings, the results related to the autocrine role of BMP6 may provide a new direction for theranostic drug development to treat KD.

Clinical and genetic characteristics of familial Mediterranean fever-associated vasculitides in children of Armenia

Family Mediterranean fever (FMF), or a periodic disease, is the most common hereditary disease among Armenians. FMF manifests mainly in childhood, presenting a significant problem for the pediatric health care system in Armenia. The clinical presentations of FMF have much in common with that of vasculitides: fever, abdominal pains, arthritis, myalgias, and skin damage. The data available in the literature indicate the high incidence of vasculitides in patients with FMF in the ethnically significant groups compared with the general population. Objective: to investigate the clinical and genetic characteristics of FMF-associated vasculitides in children of Armenia. Subjects and methods. The National Pediatric FMF Center, Arabkir Medical Complex, Institute of Child and Adolescent Health, examined a group of 715 children (438 boys, 277 girls; mean age, 8.64+0.17 years) with FMF. The diagnosis of FMF was confirmed using the Tel-Hashomer criteria and the MEFV gene mutation analysis. The Epi-Info 2000 program was used for statistical analysis. Results and discussion. The rate of vasculitides was 4.3% (n=31), being generally higher than expected. In particular, the diagnoses made were Henoch-Scho nlein pirpura (HSP) in 11 (1.5%) children, protracted febrile myalgia (PFM) in 20 (2.7%), and Behcet’s disease (BD) in 1 (0.1%) patient. The patients were characterized by early-onset FMF (at an average of 3 years) with a moderate/severe course, frequent episodes, a predominance of articular syndrome, more commonly acute relapsing arthritis, with the addition of clinical presentations of vasculitis on average 5—6 years after the onset of FMF, and genetically by the M694V homozygous genotype (M694V/M694V). HSP and PFM were observed in 2.9 and 4.6% of patients with the M694V homozygous genotype, respectively (p<0.02). Conclusion. The rate of FMF-associated vasculitides in the children of Armenia was 4.3%: HSP, PFM, and BD were diagnosed in 1.5, 2.7, and 0.1% of patients, respectively. The findings allow HSP and PFM to be considered as additional markers for the severe course of FMF and the M694V homozygous genotype as a risk factor for PFM. They also indicate the feasibility of the MEFV gene mutation screening in children with vasculitides in Armenia for an association with FMF for early diagnosis of the disease, timely administration of colchicine, and prevention of complications.

Small bowel ischemia as an unusual complication of Henoch‐Schönlein purpura in an adult: A case report

AbstractHenoch‐Schönlein purpura (HSP) is a systemic vasculitis affecting small vessels. It is the most common systemic vasculitis in children and is rare in adults. Serious gastrointestinal complications are more common in children than in adults. We describe a case of new‐onset HSP in a previously healthy 63‐year‐old male who required an emergency laparotomy for acute small bowel ischemia.

Involvement of activated cytotoxic T lymphocytes and natural killer cells in Henoch-Schönlein purpura nephritis.

ObjectivesImmunoglobulin A vasculitis/Henoch–Schönlein purpura (IgAV/HSP) is a major cause of vasculitis in children. It is often accompanied by nephritis (HSPN) and could progress to chronic kidney disease. Galactose‐deficient IgA1 was recently reported to be involved in the pathogenesis of HSPN, for which immunosuppressive drugs are considered key treatment. However, the involvement of immune cells in the development of HSPN remains unclear.MethodsWe compared gene expressions of peripheral blood mononuclear cells (PBMCs) among healthy controls (n = 10), IgAV/HSP patients (n = 21) and HSPN patients (n = 8), which required nephritis development within 3 months of IgAV/HSP onset. Immunohistochemistry analysis and flow cytometry were performed to assess renal biopsy specimens and PBMCs, respectively. Serum CX3CL1 levels were measured by ELISA.Results GNLY and GZMB expressions increased in HSPN patients, consistent with increased number of glomerular granulysin‐ and/or granzyme B‐positive cells demonstrated by immunohistochemistry analysis. Additionally, circulating cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells were activated with the up‐regulated surface expressions of human leucocyte antigen DR (HLA‐DR) and CX3CR1 in HSPN patients with severe proteinuria. Renal biopsies demonstrated increased number of CD8+ cells and/or CD56+ cells and up‐regulated expression of glomerular CX3CL1, a specific ligand for CX3CR1, along with increased serum CX3CL1 level.ConclusionActivated CTLs and NK cells play roles in the development of nephritis in IgAV/HSP patients and can be used as novel biomarkers for HSPN.

Elevated Levels of Platelet Activating Factor and Its Acetylhydrolase Indicate High Risk of Kawasaki Disease.

Kawasaki disease (KD) is a systemic vasculitis in children, which is related to inflammation and abnormal activation of immune system. Platelet activating factor (PAF) and its acetylhydrolase (PAF-AH) may play an important role in the pathogenesis of KD. This study aimed to investigate diagnosis and prognostic value of serum PAF and PAF-AH in KD. One hundred thirteen KD children were divided into coronary artery lesion (CAL) KD, noncoronary artery lesion (NCAL) KD, intravenous immunoglobulin (IVIG)-responsive KD, and IVIG-nonresponsive KD group. Seventy cases of fever control (F) group and 71 cases of normal control (N) group were set up. Peripheral venous blood was collected to detect serum PAF and PAF-AH levels, combined with other inflammatory mediators. Results showed that the serum levels of PAF and PAF-AH were significantly elevated in the KD group compared with F group and N group (P < 0.05). And the levels of conventional inflammatory mediators in KD group were significantly higher than those of F group (P < 0.05). In children with fever (KD group and F group), the area under the receiver operating characteristic curve (AUC) for PAF in prediction of KD was 0.804, and the estimated sensitivity and specificity were 79.6% and 74.3% with a cutoff of PAF >201.77 ng/mL, respectively; the AUC for PAF-AH in prediction of KD was 0.587, and the estimated sensitivity and specificity were 61.9% and 55.7% with a cutoff of PAF-AH >0.153 μmol/min/mL, respectively. Compared with NCAL group, PAF and C-reactive protein were higher in CAL group (P < 0.05). The AUC for PAF in prediction of CAL KD was 0.679, and the estimated sensitivity and specificity were 96.0% and 40.9% with a cutoff of PAF >225.52 ng/mL, respectively. Thus, serum levels of PAF and PAF-AH were significantly elevated in the acute phase of KD. Serum PAF and PAF-AH contributed to the diagnosis of KD, and serum PAF has a greater diagnostic value for KD. At the same time, elevated serum PAF has a certain predictive value for the occurrence of coronary artery lesions in Kawasaki disease rather than IVIG-nonresponsive KD.

Apolipoprotein M Serum Levels Correlate with IgA Vasculitis and IgA Vasculitis Nephritis.

Objective IgA vasculitis (lgAV) is the most frequent vessel vasculitis in children, and the prognosis is related to the children's age and degree of nephritis. This study is aimed at investigating serum apolipoprotein M (apoM) levels in patients with lgAV patients and at evaluating the association between apoM and disease severity. Methods A total of 109 lgAV patients and 76 age- and sex-matched healthy controls were included. The age and gender of the study participants were matched. ApoM levels were measured by an enzyme-linked immunosorbent assay. Additionally, the serum levels of lipids, apolipoproteins, kidney biochemical profiles, immunoglobulins (IgA, IgG, IgM, and IgE), and the complements (C3 and C4) were assessed using an automatic biochemical analyzer. Results ApoM was increased significantly in lgAV patients compared to healthy controls. ApoM, meanwhile, was lower in patients with nephritis than in those without nephritis. The apoM levels were higher in classes I and II IgA vasculitis nephritis (lgAVN) patients than in classes III and IV. Besides, the apoM serum level < 24.81 mg/L was an independent predictive factor for lgAVN and can be independently associated with the presence of nephritis in lgAV patients. Meanwhile, the serum apoM concentration negatively correlated with the ISKDC grading score in lgAVN patients. Conclusions Serum apoM was elevated in lgAV patients and decreased gradually with the ISKDC grading score. ApoM (OR = 0.32, 95%CI = 0.12‐0.85, p = 0.023) was identified as a protective factor for nephritis in all lgAV patients.

Von Willebrand factor parameters as potential biomarkers for disease activity and coronary artery lesion in patients with Kawasaki disease.

Elevated von Willebrand factor (vWF):Antigen plasma levels have been observed in conjunction with cardiovascular diseases or vasculitis. The association of Kawasaki disease, a vascular inflammatory disease and vWF:Antigen, vWF:Collagen binding activity, and vWF multimers is unknown. We therefore investigated vWF parameters in 28 patients with acute Kawasaki disease in association with disease activity and coronary artery lesions. VWF:Antigen and vWF:Collagen binding activity were assessed via enzyme-linked immunoassay. The ratio of both (vWF:Collagen binding activity and VWF:Antigen) was calculated and vWF multimeric structure analysis performed. We analyzed the association between vWF parameters and our clinical data focusing on coronary artery outcome. VWF:Antigen and vWF:Collagen binding activity levels were significantly higher in the acute than in the disease's convalescence phase, and correlated positively with CRP levels. Neither variable was associated with coronary artery lesions. The vWF:Collagen binding activity/vWF:Antigen ratio, however, was significantly decreased in patients with a coronary artery lesion (z-score > 2; N = 10; mean ratio 0.96 vs. 0.64; p = 0.031) and even more so in those with a coronary artery aneurysm (z-score > 2.5; N = 8; mean ratio 0.94 vs. 0.55; p = 0.02). In a sub-analysis, those patients with a very low ratio in the acute phase presented a persistent coronary artery aneurysm at their 1-year follow-up.Conclusion: This study suggests that comprehensive analysis of vWF parameters may help to both monitor KD inflammation and facilitate the identification of those patients carrying an increased risk for coronary artery lesion.What is Known:• Von Willebrand factor (VWF)-parameters represent surrogate markers for vascular inflammation.• Kawasaki disease is a generalized vasculitis in children, which can be complicated by coronary artery lesions.What is New:• In those Kawasaki disease patients with coronary artery lesions, the vWF:CB/vWF:Ag ratio was significantly decreased.• VWF parameters may help to identify patients at risk for coronary artery lesions.

CLINICO EPIDEMIOLOGICAL PROFILE OF HENOCH-SCHONLEIN PURPURA IN CHILDREN: A RETROSPECTIVE STUDY

Background: The incidence of Henoch-Schonlein purpura (HSP), the most common systemic vasculitis in children, is steadily increasing. It is an immunoglobulin A (IgA) mediated systemic small-vessel vasculitis. HSP is now referred to as IgA vasculitis as per the Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides in 2012. Objective: The objective of the study was to study the clinical profile, pattern of organ involvement, treatment modalities, and outcomes of HSP. Materials and Methods: A retrospective study was done from January 2016 to June 2018 using the medical records of our institute. During the study period, 27 children were diagnosed with HSP. The age, gender, clinical presentation, and laboratory data including complete blood count, C-Reactive protein, erythrocyte sedimentation rate, and complete urinalysis, USG abdomen, and recurrence were studied. Results: The ratio of male:female was 3:1. Palpable purpura was present in all the patients and was the initial presentation reported in most of the cases. Arthritis was seen in 44% and abdominal pain in 66% of the cases. Renal manifestations in the form of hematuria were reported in 22% and proteinuria in 11% of the patients. Recovery with conservativemanagement was seen in two patients, whereas others required steroids. Conclusion: HSP is a common vasculitis seen in childhood and it might have the risks for various complications.

The Detection of Occult Retinal Vasculitis on Fluorescein Angiography in Pediatric Uveitis

To evaluate the usefulness of fluorescein angiography (FA) to detect occult retinal vasculitis in children with otherwise apparently quiescent intermediate uveitis, posterior uveitis, and panuveitis based on clinical examination alone. Retrospective chart review. Pediatric uveitis patients evaluated at the Children's Medical Center in Dallas, Texas. Retrospective chart review of pediatric patients treated with immunosuppressive therapy in the uveitis clinic at the Children's Medical Center, Dallas, Texas, between September 2015 and September 2016. Patients with noninfectious uveitis requiring immunosuppressive therapy, in which posterior segment involvement (intermediate uveitis, posterior uveitis, or panuveitis) was known or suspected, and whose disease apparently was quiescent on clinical examination were included. The incidence of occult retinal vasculitis detected on FA alone. Fourteen pediatric patients met inclusion criteria. Six patients (43%) demonstrated intermediate uveitis, and 8 patients (57%) demonstrated panuveitis. Eleven patients (79%) were found to show additional evidence of occult retinal vasculitis on FA. Fluorescein angiography can be an important tool in evaluating pediatric uveitis patients with known or suspected posterior involvement for the presence of occult retinal vasculitis. Failure to control occult retinal vasculitis adequately may be a contributing factor to seemingly recalcitrant cases, inability to wean off immunomodulatory therapy, and long-term complications leading to poor prognosis.

Clinical Characteristics of Biopsy-Proven IgA Vasculitis in Children and Adults: A Retrospective Cohort Study

ObjectiveTo describe the differences in clinical characteristics and outcome between adult- and childhood-onset biopsy-proven IgA vasculitis (IgAV) in North America. Patients and MethodsPatients with IgAV diagnosed from January 1, 1997, through December 31, 2016, were retrospectively identified. Data were abstracted from direct medical record review. Kaplan-Meier methods were used to estimate survival rates. ResultsA total of 243 patients with IgAV were included (227 [93.4%] white, 141 [58.0%] male); 174 patients were adults (≥21 years), and 69 were younger than 21 years. Compared with patients younger than 21 years, adults at baseline more frequently had ulcerative skin lesions (19 [10.9%] vs 1 [1.4%]; P=.02) and nephrotic-range proteinuria (21 of 96 [21.9%] vs 1 of 38 [2.6%]; P=.007) but less commonly had abdominal pain (59 [33.9%] vs 42 [60.9%]; P<.001), ischemic gastrointestinal tract involvement (18 [10.3%] vs 14 [20.3%]; P=.04), and arthralgias (66 [37.9%] vs 42 [60.8%]; P<.001). During 389 person-years of follow-up, 29 deaths were observed. Five-year survival rates for patients aged younger than 21, 21 to 50, and 51 years or older were 100%, 94%, and 40%, respectively. In comparison to data from the United States life tables for whites, patients 51 years or older at diagnosis had a greater than 7-fold increased risk of mortality (standardized mortality, 7.60 [95% CI, 5.01-11.06]; P<.001). ConclusionIgA vasculitis in adults is associated with more severe skin/kidney involvement and poorer renal outcome. Among adults with IgAV, patients aged 51 years or older at diagnosis have significantly higher mortality (P<.001).