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Pediatric vasculitis

Sabrina Gmuca, JiaDe Yu, Pamela F Weiss, James R Treat, David D Sherry
Erythema ab igne (EAI) is a cutaneous finding caused by prolonged heat exposure and is characterized by a reticular, brownish-pigmented, often telangiectatic dermatosis. The eruption is reminiscent of livedo reticularis, which is typically seen in the setting of a number of rheumatologic conditions, most prominently vasculitis. Identification of key features distinguishing EAI from livedo reticularis can aid in the diagnosis of EAI and correct elucidation of the underlying etiology. Our patient presented with heating pad-induced EAI in the setting of chronic pain...
March 12, 2018: Pediatric Emergency Care
Weiguo Qian, Yunjia Tang, Wenhua Yan, Ling Sun, Haitao Lv
BACKGROUND: Kawasaki disease (KD) is the most common pediatric vasculitis. Several models have been established to predict intravenous immunoglobulin (IVIG) resistance. The present study was aimed to evaluate the efficacy of prediction models using the medical data of KD patients. METHODS: We collected the medical records of patients hospitalized in the Department of Cardiology in Children's Hospital of Soochow University with a diagnosis of KD from Jan 2015 to Dec 2016...
March 9, 2018: Italian Journal of Pediatrics
Ling Luo, Huanling Wang, Hongwei Fan, Jing Xie, Zhifeng Qiu, Taisheng Li
Chronic active Epstein-Barr virus (CAEBV) infection is a rare disease with high mortality. Most of CAEBV patients have been reported from Japan and are pediatric cases.The goal was to describe the clinical characteristics and the immunophenotypic features of peripheral lymphocytes in adult onset CAEBV patients.We retrospectively reviewed and analyzed all adult onset CAEBV cases admitted to Peking Union Medical College Hospital (PUMCH) between 2012 and 2016. Demographic, clinical, laboratory data, and the immunophentyping data of peripheral lymphocytes were collected...
March 2018: Medicine (Baltimore)
Christine Y Louie, Adam J Gomez, Richard K Sibley, Dorsey Bass, Teri A Longacre
Immunoglobulin A (IgA) vasculitis or Henoch-Schönlein purpura (HSP) typically occurs in the pediatric population, although rare cases also occur in adults. Gastrointestinal (GI) involvement is common. The "classic" histologic finding in IgA vasculitis (HSP) is leukocytoclastic vasculitis (LCV); other histologic features in biopsies of IgA vasculitis (HSP) have only been rarely described. The pathology archival files at our institution were searched for GI biopsies from patients with IgA vasculitis (HSP)...
February 12, 2018: American Journal of Surgical Pathology
Ali Nikkhah
Kawasaki disease (KD) is an inflammatory vasculitis. KD is classified into two groups based on clinical characteristics criteria, namely classic and incomplete. Cerebral vascular abnormality, especially arterial ischemic stroke (AIS) is very rare and unusual in KD. Here, we report a 4-year-old boy who was referred to our tertiary pediatric center with abrupt right hemiparesis and aphasia. At admission time, he had febrile illness and was toxic. On physical examination, we found unilateral left submandibular lymphadenopathy...
January 2018: Iranian Journal of Medical Sciences
A Escudier, F-X Mauvais, P Bastard, C Boussard, A Jaoui, V Koskas, E Lecoq, A Michel, M-C Orcel, P-E Truelle, D Wohrer, M Piram
Auto-inflammatory diseases are characterized by unexplained and recurrent attacks of systemic inflammation often involving the skin, joints, or serosal membranes. They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis...
January 29, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Ameni Jerbi, Sawsan Feki, Salma Ben Ameur, Hend Hachicha, Lamia Gargouri, Thouraya Kamoun, Abdelmajid Mahfoudh, Mongia Hachicha, Hatem Masmoudi
In adults, anti-neutrophil cytoplasmic antibodies (ANCA) are considered as serological markers of several diseases, especially vasculitis and glomerulonephritis. Since ANCA are rarely positive in children, few data about the clinical relevance of these auto-antibodies in pediatric population have been reported. Therefore, our study aims to describe the spectrum of disorders associated with positive ANCA in Tunisian children. This study had been carried out over a period of 12 years and a half. All patients under the age of 15 for whom ANCA screening was performed in our laboratory were included...
January 1, 2018: Annales de Biologie Clinique
Cristina Guerriero, Gaia Moretta, Giulia Bersani, Piero Valentini, Antonio Gatto, Donato Rigante
Background: Necrotizing vasculitides are basically characterized by vessel wall neutrophil infiltration and necrosis and they can occur as a primary process or secondary to an underlying disease. Although Henoch-Schönlein purpura (HSp) is the more frequent primary vasculitis in childhood, sometimes it has to be distinguished from other secondary vasculitides induced by infections, drugs, vaccines, or immune-mediated disorders. Main observations: We report a case of a 14-year-old girl with cutaneous necrotizing vasculitis, appearing in the course of acute Epstein-Barr virus infection...
December 1, 2017: Journal of Dermatological Case Reports
Siqi Feng, Ya Su, Li Luo, Jing Fengchuan, Qijian Yi
OBJECTIVE: To investigate the serum C1q/tumor necrosis factor-related protein-1 (CTRP1) levels in children with acute Kawasaki disease (KD), as well as the relationship between CTRP1 levels and laboratory variables. METHODS: Eighty-seven children with KD and 38 healthy controls (HC) were included in this study. General characteristics were obtained from all subjects. Serum CTRP1 levels in all subjects and serum tumor necrosis factor-α (TNF-α), interleukin-1β (IL-1β), and interleukin-6 (IL-6) levels in KD patients were measured using enzyme-linked immunosorbent assay...
January 23, 2018: Pediatric Research
O Monteventi, M Steinlin, M Regényi, E Roulet-Perez, P Weber, J Fluss
BACKGROUND: Cerebrovascular complications of Lyme neuroborreliosis (LNB) are poorly documented in the paediatric population. METHODS: We performed a retrospective analysis from prospectively registered cases of acute ischemic stroke (AIS) from the Swiss NeuroPaediatric Stroke Registry (SNPSR) from 2000 to 2015. Only cases with serologically confirmed LNB were included. In addition, a literature review on paediatric stroke cases secondary to Lyme neuroborreliosis in the same time frame was performed...
January 2018: European Journal of Paediatric Neurology: EJPN
Evangeline Pillebout, Agnès Jamin, Hamza Ayari, Pierre Housset, Melissa Pierre, Virginia Sauvaget, Denis Viglietti, Georges Deschenes, Renato C Monteiro, Laureline Berthelot
Henoch-Schönlein purpura is a systemic vasculitis characterized by IgA deposits, which target the skin, joints, and kidneys, among other organs. In children, prognosis is often good but little is known about biomarkers of pediatric nephritis. We hypothesized that biological markers, including cytokines, immunoglobulins, IgA-immune complexes, IgA glycosylation and neutrophil gelatinase-associated lipocalin (NGAL), may discriminate IgA vasculitis (IgAV) pediatric patients with renal involvement from those without renal involvement...
2017: PloS One
Gülçin Otar Yener, Zahide Ekici Tekin, Neşe Çallı Demirkan, Selçuk Yüksel
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain...
November 30, 2017: Rheumatology International
Ivana Musilova, Ctirad Andrys, Marcela Drahosova, Barbora Zednikova, Helena Hornychova, Lenka Pliskova, Helena Zemlickova, Bo Jacobsson, Marian Kacerovsky
BackgroundTo characterize the influence of microbial invasion of the amniotic cavity (MIAC) and/or intra-amniotic inflammation (IAI) on the intensity of the fetal inflammatory response and the association between the presence of the fetal inflammatory response syndrome (FIRS) and short-term neonatal morbidity in the preterm prelabor rupture of membranes (PPROM) between the gestational ages of 34 and 37 weeks.MethodsOne hundred and fifty-nine women were included in the study. The umbilical cord blood interleukin (IL)-6 concentrations were determined using enzyme-linked immunosorbent assay kits...
December 20, 2017: Pediatric Research
Florence A Aeschlimann, Simon W M Eng, Shehla Sheikh, Ronald M Laxer, Diane Hebert, Damien Noone, Marinka Twilt, Christian Pagnoux, Susanne M Benseler, Rae S M Yeung
BACKGROUND: Takayasu arteritis (TAK) is a large vessel vasculitis that rarely affects children. Data on childhood TAK are scarce. The aim of this study was to analyze the presenting features, course and outcome of children with TAK, compare efficacy of treatment regimens and identify high-risk factors for adverse outcome. METHODS: A single-center cohort study of consecutive children fulfilling the EULAR/PRINTO/PReS criteria for childhood TAK between 1986 and 2015 was performed...
November 22, 2017: Arthritis Research & Therapy
Jonathan L Eliason, Dawn M Coleman, Adam Gumushian, James C Stanley
OBJECTIVE: Chronic lower extremity ischemia in pediatric patients is uncommon. The intent of this study was to better define the arterial reconstructive options and their long-term durability in preadolescent and adolescent children having clinically relevant arterial occlusions affecting the lower extremity. METHODS: The medical records of 33 consecutive pediatric patients who underwent lower extremity revascularization for chronic ischemia at the University of Michigan from 1974 to 2016 were reviewed...
November 18, 2017: Journal of Vascular Surgery
Vikas Gupta, Amita Aggarwal, Ranjan Gupta, Abhra Chandra Chowdhury, Vikas Agarwal, Able Lawrence, Ramnath Misra
AIM: Henoch-Schönlein purpura (HSP), a primary vasculitis, characterized by purpura, abdominal pain, arthritis and renal involvement, is predominantly a disease of childhood. However, rarely it can occur in adults in whom it is believed to be a more severe form with poor renal outcomes. We aimed to answer if the age of onset affected the clinical spectrum and renal outcomes of the disease in a north Indian population. Hence, we studied the differences in clinical spectrum and renal outcomes between adult-onset HSP and childhood-onset HSP...
January 2018: International Journal of Rheumatic Diseases
Maja Steinlin
Over the last decades, the importance of inflammatory processes in pediatric stroke have become increasingly evident. Ischemia launches a cascade of events: activation and inhibition of inflammation by a large network of cytokines, adhesion and small molecules, protease, and chemokines. There are major differences in the neonatal brain compared to adult brain, but developmental trajectories of the process during childhood are not yet well known. In neonatal stroke ischemia is the leading pathophysiology, but infectious and inflammatory processes have a significant input into the course and degree of tissue damage...
August 2017: Seminars in Pediatric Neurology
Ana Paula Sakamoto, Clovis Artur Silva, Marco Felipe Castro da Silva, Anandreia Simões Lopes, Gleice Clemente Souza Russo, Adriana Maluf Elias Sallum, Katia Kozu, Eloisa Bonfá, Claudia Saad-Magalhães, Rosa Maria Rodrigues Pereira, Claudio Arnaldo Len, Maria Teresa Terreri
OBJECTIVES: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. METHODS: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. RESULTS: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%)...
November 2017: Revista Brasileira de Reumatologia
M Wintermark, N K Hills, G A DeVeber, A J Barkovich, T J Bernard, N R Friedman, M T Mackay, A Kirton, G Zhu, C Leiva-Salinas, Q Hou, H J Fullerton
BACKGROUND AND PURPOSE: Childhood arteriopathies are rare but heterogenous, and difficult to diagnose and classify, especially by nonexperts. We quantified clinical and imaging characteristics associated with childhood arteriopathy subtypes to facilitate their diagnosis and classification in research and clinical settings. MATERIALS AND METHODS: The Vascular Effects of Infection in Pediatric Stroke (VIPS) study prospectively enrolled 355 children with arterial ischemic stroke (2010-2014)...
October 5, 2017: AJNR. American Journal of Neuroradiology
Seung Dohn Yeom, Hye Soo Ko, Jong Hyuk Moon, Min Ji Kang, Ji Won Byun, Gwang Seong Choi, Jeonghyun Shin
Sweet syndrome (acute, febrile, neutrophilic dermatosis) is characterized by the acute onset of an eruption of painful nodules or erythematous or violaceous plaques on the limbs, face and neck. These symptoms are accompanied by fever. The diagnostic features include histopathological findings of dermal neutrophilic infiltration without leukocytoclastic vasculitis or peripheral blood leukocytosis. Sweet syndrome is associated with infection, malignancies, autoimmune disease, pregnancy, and drugs. Patients with Sweet syndrome demonstrate a complete and rapid response to systemic steroid administration...
October 2017: Annals of Dermatology
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