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Acute kidney injury (AKI) is common in lupus nephritis (LN) and a risk factor for development of chronic kidney disease. In adults with LN, AKI severity correlates with the incidence of kidney failure and patient survival. Data on AKI outcomes in children with LN, particularly those requiring kidney replacement therapy (KRT), are limited.A multicenter, retrospective cohort study was performed in children diagnosed between 2010 and 2019 with LN and AKI stage 3 treated with dialysis (AKI stage 3D). Descriptive statistics were used to characterize demographics, clinical data, and kidney biopsy findings; treatment data for LN were not included. Logistic regression was used to examine the association of these variables with kidney failure.Fifty-nine patients (mean age 14.3 years, 84.7% female) were identified. The most common KRT indications were fluid overload (86.4%) and elevated blood urea nitrogen/creatinine (74.6%). Mean follow-up duration was 3.9 ± 2.9 years. AKI recovery without progression to kidney failure occurred in 37.3% of patients. AKI recovery with later progression to kidney failure occurred in 25.4% of patients, and there was no kidney recovery from AKI in 35.6% of patients. Older age, severe ( 50%) tubular atrophy and interstitial fibrosis, and National Institutes of Health (NIH) chronicity index score 4 on kidney biopsy were associated with kidney failure.Children with LN and AKI stage 3D have a high long-term risk of kidney failure. Severe tubular atrophy and interstitial fibrosis at the time of AKI, but not AKI duration, are predictive of kidney disease progression. A higher resolution version of the Graphical abstract is available as Supplementary information.

Background Multisystem inflammatory syndrome in children (MIS-C) is an acute, febrile, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated syndrome, often with cardiohemodynamic dysfunction. Insight into mechanism of disease is still incomplete. Objective Our objective was to analyze immunologic features of MIS-C patients compared to febrile controls (FC). Methods MIS-C patients were defined by narrow criteria, including having evidence of cardiohemodynamic involvement and no macrophage activation syndrome. Samples were collected from 8 completely treatment-naive patients with MIS-C (SARS-CoV-2 serology positive), 3 patients with unclassified MIS-C–like disease (serology negative), 14 FC, and 5 MIS-C recovery (RCV). Three healthy controls (HCs) were used for comparisons of normal range. Using spectral flow cytometry, we assessed 36 parameters in antigen-presenting cells (APCs) and 29 in T cells. We used biaxial analysis and uniform manifold approximation and projection (UMAP). Results Significant elevations in cytokines including CXCL9, M-CSF, and IL-27 were found in MIS-C compared to FC. Classic monocytes and type 2 dendritic cells (DCs) were downregulated (decreased CD86, HLA-DR) versus HCs; however, type 1 DCs (CD11c+CD141+CLEC9A+) were highly activated in MIS-C patients versus FC, expressing higher levels of CD86, CD275, and atypical conventional DC markers such as CD64, CD115, and CX3CR1. CD169 and CD38 were upregulated in multiple monocyte subtypes. CD56dim/CD57−/KLRGhi/CD161+/CD38− natural killer (NK) cells were a unique subset in MIS-C versus FC without macrophage activation syndrome. Conclusion Orchestrated by complex cytokine signaling, type 1 DC activation and NK dysregulation are key features in the pathophysiology of MIS-C. NK cell findings may suggest a relationship with macrophage activation syndrome, while type 1 DC upregulation implies a role for antigen cross-presentation.

Tumor necrosis factor receptor-associated factor 3 (TRAF3) is a central regulator of immunity. TRAF3 is often somatically mutated in B cell malignancies, but its role in human immunity is not defined. Here, in five unrelated families, we describe an immune dysregulation syndrome of recurrent bacterial infections, autoimmunity, systemic inflammation, B cell lymphoproliferation, and hypergammaglobulinemia. Affected individuals each had monoallelic mutations in

ObjectiveFibromyalgia (FM) is defined by idiopathic, chronic, widespread musculoskeletal pain. In adults with FM, a metaanalysis of lower-leg skin biopsy demonstrated 45% pooled prevalence of abnormally low epidermal neurite density (END). END < 5th centile of the normal distribution is the consensus diagnostic threshold for small-fiber neuropathy. However, the clinical significance of END findings in FM is unknown. Here, we examine the prevalence of small-fiber pathology in juvenile FM, which has not been studied previously.MethodsWe screened 21 patients aged 13–20 years with FM diagnosed by pediatric rheumatologists. Fifteen meeting the American College of Rheumatology criteria (modified for juvenile FM) underwent lower-leg measurements of END and completed validated questionnaires assessing pain, functional disability, and dysautonomia symptoms. The primary outcome was proportion of FM patients with END < 5th centile of age/sex/race-based laboratory norms. Cases were systematically matched by ethnicity, race, sex, and age to a group of previously biopsied healthy adolescents with selection blinded to biopsy results. All 23 controls matching demographic criteria were included.ResultsAmong biopsied juvenile FM patients, 53% (8/15) had END < 5th centile vs 4% (1/23) of healthy controls (P < 0.001). Mean patient END was 273/mm2 skin surface (95% CI 198–389) vs 413/mm2 (95% CI 359–467, P < 0.001). As expected, patients with FM reported more functional disability, dysautonomia, and pain than healthy controls.ConclusionAbnormal END reduction is common in adolescents with FM, with similar prevalence in adults with FM. More studies are needed to fully characterize the significance of low END in FM and to elucidate the clinical implications of these findings.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, characterized predominantly by respiratory symptoms, has affected a small subset of children. Neurologic manifestations have been described in adults, including encephalitis/meningitis, encephalopathy, strokes, seizures, and anosmia,1,2 but there are few reports of neurologic manifestations in children with SARS-CoV-2.3

Made available in DSpace on 2022-04-29T08:30:47Z (GMT). No. of bitstreams: 0 Previous issue date: 2021-07-01 Objective: Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population. Methods: An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations. We analysed patterns of symptoms, diagnostic workup, and applied the 2016 ACR/EULAR classification criteria. Results: We identified 300 children with Sjögren syndrome. The majority of patients n = 232 (77%) did not meet 2016 ACR/EULAR classification criteria, but n = 110 (37%) did not have sufficient testing done to even possibly achieve the score necessary to meet criteria. Even among those children with all criteria items tested, only 36% met criteria. The most common non-sicca symptoms were arthralgia [n = 161 (54%)] and parotitis [n = 140 (47%)] with parotitis inversely correlating with age. Conclusion: Sjögren syndrome in children can present at any age. Recurrent or persistent parotitis and arthralgias are common symptoms that should prompt clinicians to consider the possibility of Sjögren syndrome. The majority of children diagnosed with Sjögren syndromes did not meet 2016 ACR/EULAR classification criteria. Comprehensive diagnostic testing from the 2016 ACR/EULAR criteria are not universally performed. This may lead to under-recognition and emphasizes a need for further research including creation of paediatric-specific classification criteria. Division of Pediatric Rheumatology Department of Pediatric and Adolescent Medicine Mayo Clinic Division of Rheumatology Department of Pediatrics University of Utah School of Medicine Division of Rheumatology The Children's Hospital of Philadelphia Department of Internal Medicine and Pediatrics The University of Chicago Medical Center Department of Pediatrics Duke University School of Medicine Department of Pediatrics Hematology and Oncology Medical University of Gdansk Children's Mercy Hospital University of Missouri-Kansas City Division of Rheumatology Irccs Ospedale Pediatrico Bambino Gesù Department of Pediatric Rheumatology Institute of Rheumatology Department of Pediatrics Columbia University Medical Center Division of Rheumatology Children's National Hospital George Washington University School of Medicine and Health Sciences National Institutes of Health National Institute of Arthritis and Musculoskeletal and Skin Diseases Department of Pediatrics University of Texas McGovern Medical School at Houston Division of Pediatric Rheumatology Children's Healthcare of Atlanta Emory University School of Medicine Instituto de Puericultura e Pediatria Martagão Gesteira Universidade Federal Do Rio de Janeiro Pediatric Rheumatology Hospital Sant Joan de Déu Universitat de Barcelona Barcelona Rheumatology Unit Department of Medicine School of Medical Science University of Campinas Division of Rheumatology Department of Pediatrics University of Indiana School of Medicine Riley Hospital for Children at Indiana University Health Pediatric Rheumatology Unit São Paulo State University (UNESP), Botucatu Rheumatology Service Royal Children's Hospital Melbourne and Murdoch Children's Research Institute Division of Pediatric Allergy Immunology and Rheumatology Department of Pediatrics College of Medicine University of Florida Division of Oral Medicine Department of Oral and Maxillofacial Diagnostic Sciences College of Dentistry University of Florida Rheumatology Department Hospital General Universitario Gregorio Marañón Division of Immunology Boston Children's Hospital Department of Pediatrics Harvard Medical School Department of Rheumatic Diseases Tokyo Metropolitan Tama Medical Center Division of Rheumatology Allergy and Immunology Stead Family Department of Pediatrics Carver College of Medicine University of Iowa Pediatric Rheumatology Unit São Paulo State University (UNESP), Botucatu

Supplemental Digital Content is available in the text., Objectives: The disease caused by severe acute respiratory syndrome coronavirus 2, known as coronavirus disease 2019, has resulted in a global pandemic. Reports are emerging of a new severe hyperinflammatory syndrome related to coronavirus disease 2019 in children and adolescents. The Centers for Disease Control and Prevention has designated this disease multisystem inflammatory syndrome in children. Our objective was to develop a clinical inpatient protocol for the evaluation, management, and follow-up of patients with this syndrome. Data Sources: The protocol was developed by a multidisciplinary team based on relevant literature related to coronavirus disease 2019, multisystem inflammatory syndrome in children, and related inflammatory syndromes, as well as our experience caring for children with multisystem inflammatory syndrome in children. Data were obtained on patients with multisystem inflammatory syndrome in children at our institution from the pre-protocol and post-protocol periods. Data Synthesis: Our protocol was developed in order to identify cases of multisystem inflammatory syndrome in children with high sensitivity, stratify risk to guide treatment, recognize co-infectious or co-inflammatory processes, mitigate coronary artery abnormalities, and manage hyperinflammatory shock. Key elements of evaluation include case identification using broad clinical characteristics and comprehensive laboratory and imaging investigations. Treatment centers around glucocorticoids and IV immunoglobulin with biologic immunomodulators as adjuncts. Multidisciplinary follow-up after discharge is indicated to manage continued outpatient therapy and evaluate for disease sequelae. In nearly 2 months, we admitted 54 patients with multisystem inflammatory syndrome in children, all of whom survived without the need for invasive ventilatory or mechanical circulatory support. After institution of this protocol, patients received earlier treatment and had shorter lengths of hospital stay. Conclusions: This report provides guidance to clinicians on evaluation, management, and follow-up of patients with a novel hyperinflammatory syndrome related to coronavirus disease 2019 known as multisystem inflammatory syndrome in children. It is based on the relevant literature and our experience. Instituting such a protocol during a global pandemic is feasible and is associated with patients receiving treatment and returning home more quickly.

We describe 2 patients with coronavirus disease who had multiple clinical features suggestive of Kawasaki disease (KD). Both patients presented with fever lasting >5 days and were found to have rash, conjunctival injection, and swollen lips. One patient also had extremity swelling, whereas the other developed desquamation of the fingers. In both cases, laboratory results were similar to those seen in KD. These patients had highly unusual but similar features, and both appeared to respond favorably to treatment. It remains unclear whether these patients had true KD or manifestations of coronavirus disease that resembled KD.

This case series describes clinical characteristics, treatment, and outcomes of 17 previously healthy SARS-CoV-2-infected children and adolescents with an inflammatory phenotype overlapping with but distinct from Kawasaki disease and toxic shock syndrome admitted to a New York City hospital in late April and early May 2020.

Objective The objective of this article is to examine the quality, content, and readability of information and resources in the English language and accessible on the internet by pediatric patients with systemic lupus erythematosus (SLE) and their families in North America. Methods Keywords relevant to SLE were generated by an undergraduate student, a first-year medical student, and a third-year pediatric resident, and a search was conducted across five commonly used search engines. Quality of information found was evaluated independently by an undergraduate student, a graduate student, a first-year medical student, and a third-year pediatric resident using the DISCERN tool. Two pediatric rheumatologists assessed website accuracy and completeness. Readability of websites was determined using the Flesch-Kincaid grade level and Reading Ease score. Results Out of 2000 websites generated in the search, only 34 unique websites met inclusion criteria. Only 16 of these websites had DISCERN scores above 50% (fair quality). Overall quality of website information was fair with mean ±standard deviation (SD) DISCERN quality score of 44 ± 7 (range: 30-56). Only nine websites of 34 had DISCERN scores above 50 (66%, indicating greater quality) and were further assessed for completeness. Flesch-Kincaid grade level was 11 ± 1 (mean±SD) and reading ease score was 39 ± 10 (mean±SD, range of 11-61). Conclusion Our study highlights the need for more complete, readable information regarding the unique needs of pediatric patients with childhood-onset SLE and their families.

BackgroundNeurological involvement has been reported in up to 80% of adults with Primary Sjogren’s syndrome (pSS) with psychiatric abnormalities including anxiety, depression, and cognitive dysfunction being common. Psychosis due to pSS has been reported in adult patients but has never been previously reported in the adolescent/pediatric literature. Here we describe for the first time four cases of adolescent Sjogren’s syndrome that presented with psychotic symptoms. Rituximab treatment was followed by improvement of psychiatric symptoms in all patients.Case presentation1: 16 year old female without significant past medical history presented to the emergency department with 4 days of abnormal behavior, tremors, insomnia, polyphagia, polyuria, and suicidal ideation.2: 16 year old female with a 4 year history of severe anxiety, OCD, and tic disorder treated with fluoxetine with partial benefit presented with an abrupt and severe worsening of anxiety, OCD and new auditory hallucinations.3: 19 year old female without significant past medical history presented with a 3 day history of progressively altered behavior, incoherent speech, insomnia, headache, and tangential thoughts.4: 17 year old female without significant past medical history presented with new onset suicidal ideation, paranoia, confusion, and emotional lability.ConclusionPsychosis is more common in autoimmune disease than previously known. To our knowledge, the four teenage women described above are the first reported patients with adolescent pSS manifesting as psychosis. pSS should be considered in the differential diagnosis of young patients with new psychiatric disorders, even in the absence of sicca symptoms. Psychiatric symptoms improved with rituximab infusions in all 4 of our patients, which suggests rituximab may be an effective treatment option that should be considered early after the diagnosis of pSS-associated psychiatric disturbance.

Background To describe the demographic, clinical, and treatment characteristics of youth diagnosed with juvenile primary fibromyalgia syndrome (JPFS) who are seen in pediatric rheumatology clinics. Methods Information on demographics, symptoms, functioning, and treatments recommended and tried were obtained on patients with JPFS as part of a multi-site patient registry (the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry). Data were summarized using descriptive statistics. In a subset of patients completing registry follow-up visits, changes in symptoms, pain, and functioning were evaluated using growth modeling. Results Of the 201 patients with JPFS enrolled in the registry, most were Caucasian/White (85%), non-Hispanic (83%), and female (84%). Ages ranged from 9 to 20 years (M = 15.4 + 2.2). The most common symptoms reported were widespread musculoskeletal pain (91%), fatigue (84%), disordered sleep (82%), and headaches (68%). Pain intensity was rated as moderate to severe (M = 6.3 + 2.4/10). Scores on measures of functioning indicated mild to moderate impairment, with males observed to report significantly greater impairments. For the 37% of the initial cohort having follow-up data available, indicators of function and well-being were found to either worsen over time or remain relatively unchanged. Conclusions The symptoms of JPFS remained persistent and disabling for many patients treated by pediatric rheumatologists. Further study appears warranted to elucidate gender differences in the impact of JPFS symptoms. Work also is needed to identify accessible and effective outpatient treatment options for JPFS that can be routinely recommended or implemented by pediatric rheumatology providers.

OBJECTIVE Improved treatments for juvenile idiopathic arthritis (JIA) have increased remission rates. We conducted this study to investigate how patients and caregivers make decisions about stopping medications when JIA is inactive. METHODS We performed a mixed-methods study of caregivers and patients affected by JIA, recruited through social media and flyers, and selected by purposive sampling. Participants discussed their experiences with JIA, medications, and decision-making through recorded telephone interviews. Of 44 interviewees, 20 were patients (50% ages

OBJECTIVES: To examine whether patients with multisystem inflammatory syndrome in children (MIS-C) demonstrated well-defined clinical features distinct from other febrile outpatients, given the difficulties of seeing acute care visits during the severe acute respiratory syndrome coronavirus 2 pandemic and the risks associated with both over- and underdiagnosis of MIS-C. STUDY DESIGN: This case-controlled study compared patients diagnosed with and treated for MIS-C at a large urban children's hospital with patients evaluated for fever at outpatient acute care visits during the peak period of MIS-C. Symptomatology and available objective data were extracted. Comparisons were performed using t tests with corrections for multiple comparisons, and multivariable logistic regression to obtain ORs. RESULTS: We identified 44 patients with MIS-C between April 16 and June 10, 2020. During the same period, 181 pediatric patients were evaluated for febrile illnesses in participating outpatient clinics. Patients with MIS-C reported greater median maximum reported temperature height (40°C vs 38.9, P

ObjectiveThe objective of this article is to describe and compare clinical features, treatment, and renal outcomes of children with membranous lupus nephritis (MLN), through analysis of a national multicenter registry.MethodsPatients with pediatric systemic lupus erythematosus (SLE) and MLN from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry were included. Demographic, disease and medication-related data were collected between 2010 and 2014 from 59 CARRA Legacy Registry sites.ResultsA total of 132 individuals had MLN, either in isolation or in combination with proliferative LN. Seventy-four patients had pure MLN. The proportion of patients with daily corticosteroid treatment was similar among groups (96%, 91%, and 96%, for class III+V, IV+V, and V, respectively, p = 0.67). Proportion of individuals exposed to any disease-modifying antirheumatic drug (DMARD) or biologic was similar among the three groups (83%, 91%, 95% for class III+V, IV+V, and V, respectively, p = 0.189). Proportion of patients with decreased glomerular filtration rate (less than 90 ml/min/1.73 m2) was significantly different among groups (4%, 38%, and 4%, for class III+V, IV+V, and V, respectively, p ConclusionThis is the largest reported cohort of children with MLN. More research is needed to understand treatment practices for pediatric MLN, particularly decisions related to pharmacologic treatment of pure MLN. More work is also needed to identify prognostic factors and predictors of outcome for pediatric MLN. Future observational studies will be a first step toward understanding and formulating a standardized approach to treatment of pediatric membranous LN and allowing for the initiation of prospective comparative effectiveness studies and interventional trials.

Objective.To describe treatment practices for childhood pure membranous lupus nephritis (MLN).Methods.Survey study of Childhood Arthritis and Rheumatology Research Alliance and American Society of Pediatric Nephrology members.Results.There were 117 respondents who completed the survey (60 pediatric nephrologists, 57 pediatric rheumatologists). Steroids and nonsteroid immunosuppression (NSI) were routinely used by the majority for MLN. Mycophenolate mofetil was the favored initial NSI. Nephrologists used steroids (60% vs 93%) and NSI (53% vs 87%) less often than did rheumatologists for MLN without nephrotic syndrome (NS).Conclusion.Pediatric rheumatologists and nephrologists both recommend steroids and NSI for children with MLN, with or without NS.

Author(s): Fotis, Lampros; Shaikh, Nur; Baszis, Kevin; French, Anthony; Tarr, Phillip; Grevich, Sriharsha; Lee, Peggy; Ringold, Sarah; Leroux, Brian; Leahey, Hannah; Yuasa, Megan; Foster, Jessica; Sokolove, Jeremy; Lahey, Lauren; Robinson, William; Newsom, Joshua; Stevens, Anne; Karasawa, Rie; Tamaki, Mayumi; Tanaka, Megumi; Sato, Toshiko; Yudoh, Kazuo; Jarvis, James N; Moncrieffe, Halima; Bennett, Mark F; Tsoras, Monica; Luyrink, Lorie; Xu, Huan; Prahalad, Sampath; Morris, Paula; Dare, Jason; Nigrovic, Peter A; Rosenkranz, Margalit; Becker, Mara; O’Neil, Kathleen M; Griffin, Thomas; Lovell, Daniel J; Grom, Alexei A; Medvedovic, Mario; Thompson, Susan D; Zhu, Lisha; Jiang, Kaiyu; Wong, Laiping; Buck, Michael J; Chen, Yanmin; Moncrieffe, Halima; Brungs, Laura; Liu, Tao; Wang, Ting; Jarvis, James N; Alsaeid, Khaled; Alfailakawi, Jasim; Alenezi, Hamid; Alsaeed, Hazim; Beukelman, Tim; Natter, Marc; Ilowite, Norm; Mieszkalski, Kelly; Burrell, Grendel; Best, Brian; Bristow, Helen; Carr, Shannon; Dennos, Anne; Kaufmann, Rachel; Kimura, Yukiko; Schanberg, Laura; Blier, Peter R; Boneparth, Alexis; Wenderfer, Scott E; Moorthy, L Nandini; Radhakrishna, Suhas M; Sagcal-Gironella, Anna Carmela P; von Scheven, Emily; Gedik, Kader Cetin; Siddique, Salma; Aguiar, Cassyanne L; Erkan, Doruk; Cohen, Ezra; Lee, Yvonne; Dossett, Michelle; Mehta, Darshan; Davis, Roger; Gilbert, Mileka; Goilav, Beatrice; Meidan, Esra

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. Persistent pain is the most common and distressing symptom of JIA, and pain in childhood arthritis is multifactorial. Children and adolescents with persistent pain due to JIA experience significantly more problems with physical, emotional, social, and school functioning than healthy individuals. Assessment of pain at each office visit is the cornerstone of effective pain management and should include an evaluation of pain intensity, interference, and coping. Following the biopsychosocial model of pain management, a multi-modal approach is recommended for pain control in children with arthritis. Pharmacologic strategies for the treatment of pain in JIA include aggressive treatment of the underlying disease as well as the use of acetaminophen and systemic and topical non-steroidal anti-inflammatory drugs for persistent mild pain. Opioids can be considered in the case of moderate to severe persistent pain. Physical therapies and psychological interventions such as cognitive behavioral therapy are also key components of pain management in JIA.

Behçet disease is a systemic vasculitis of unknown etiology that can affect the neurologic system. Neuro-Behçet disease is not well defined in children and adolescents, and the diagnosis is difficult to make in this population as they often present with insufficient symptoms to meet diagnostic criteria. Psychiatric symptoms as the initial manifestation of neuro-Behçet disease has rarely been reported. We describe a 17-year-old boy who presented with acute psychosis and was subsequently diagnosed with neuro-Behçet disease. A rare combination of both cerebral venous thrombosis and parenchymal central nervous system involvement was identified by neuroimaging. Although treatment guidelines for neuro-Behçet disease are limited, the patient made demonstrative clinical and radiographic improvement with a combination of corticosteroids, anticoagulation, and immunosuppressants, including a tumor necrosis factor-α (TNFα) blocking agent.

* ALT: : alanine aminotransferase AST: : aspartate aminotransferase BUN: : blood urea nitrogen CBC: : complete blood count CNS: : central nervous system CSF: : cerebrospinal fluid CT: : computed tomography ECG: : electrocardiography ED: : emergency department EEG: : electroencephalography ESR: : erythrocyte sedimentation rate GI: : gastrointestinal GU: : genitourinary Hct: : hematocrit Hgb: : hemoglobin MRI: : magnetic resonance imaging WBC: : white blood cell A 2-month-old term girl presents to an ED with a 3-week history of episodic difficulty in breathing together with screaming during and after feeding. The episodes are described as rapid breathing and shortness of breath that happen only with feeds and are associated with sweating and pallor, occurring multiple times a day. The patient has no other signs, including fever, runny nose, nasal congestion, drooling, noisy breathing, difficulty swallowing, or arching of the back with feeds. Her weight gain is appropriate for age. The patient has been evaluated for these complaints and felt to have colic or gastroesophageal reflux disease, for which ranitidine was prescribed. Her birth and perinatal histories are unremarkable. On physical examination, the following are found: temperature, 37.2°C; heart rate, 160 beats/min; respiratory rate, 52 breaths/min; pulse oximetry, 96% in room air; and blood pressure, 92/52 mm Hg. She is alert and has moderate respiratory distress with tachypnea and subcostal retractions. Her precordium is hyperdynamic. On auscultation, there is a gallop rhythm but no murmurs. Pulses are palpable but weak in four limbs, and capillary refill is prolonged at 4 seconds. There is no hepatomegaly. The findings on the rest of the examination are within normal limits. Laboratory findings include a normal CBC, capillary blood gas, and serum concentrations of lactic acid and electrolytes. Liver function tests, including serum albumin, prothrombin time, and partial thromboplastin time, also yield normal results. Cardiac enzymes are normal except for a mildly elevated serum troponin concentration at 0.43 ng/mL (normal 0 to 0.4 ng/mL). Additional evaluation leads to the diagnosis. A 15-year-old girl is hospitalized for progressive right thumb redness and swelling. She reports tactile fevers and chills. She has pain with movement of the thumb and tenderness over the affected area, but no limitation of motion or paresthesia. She was …

Melanoma differentiation-associated protein 5 (MDA-5) antibodies have been linked to a distinct clinical phenotype in patients with inflammatory myopathy. The spectrum of MDA-5 associated connective tissue disease is still not well characterized, and it may extend beyond classically defined inflammatory myopathies such as polymyositis and dermatomyositis. We report a case of an 11-year-old boy who presented with inflammatory polyarthritis and persistent fasciitis. He was found to have positive MDA-5 antibodies. The patient did not meet the criteria for diagnosis of inflammatory myositis as he had no evidence of muscle disease and lacked the characteristic skin manifestations seen in juvenile dermatomyositis. This case demonstrates the wide clinical spectrum of MDA-5 associated disease.

Thrombotic thrombocytopenic purpura (TTP) is a well-described complication of systemic lupus erythematosus (SLE).1 Historically, plasma exchange has been the mainstay of treatment for TTP as well as for other thrombotic microangiopathies.2 Recently, the complement inhibitor eculizumab has demonstrated efficacy in treating atypical hemolytic uremic syndrome (aHUS).3 Whether eculizumab therapy could be beneficial in the treatment of TTP is unknown. We report a case of pediatric SLE-associated TTP treated with eculizumab, in conjunction with steroids, plasma exchange, and additional immunosuppressant therapies.

Background/Purpose: There are limited data describing longitudinal trends of symptoms and outcomes among children seeking treatment for Juvenile Primary Fibromyalgia Syndrome (JPFS). Pediatric rheumatologists often are the first point of subspecialty contact for children and adolescents with JPFS and typically evaluate the outcome of their initial treatment recommendations over one or more follow-up visits. Using baseline and follow-up data entered into the Childhood Arthritis and Rheumatology Research Alliance (CARRA) registry for patients with JPFS, we sought to determine the extent to which symptoms, function, and treatment recommendations change over time in this patient population. Methods: Deidentified information on symptoms, treatments, and function (CHAQ scores, subjective well-being numerical rating scale, and 5-point HRQOL rating scale) were extracted from the CARRA registry for all baseline and follow-up entries between May 2010 and September 2013. Data were analyzed for 66 patients (38% of the baseline cohort) having at least one follow-up visit recorded in the registry; the follow-up period ranged from 0.25 to 2.5 years from baseline (M = .88 years +/− .51 years). The analyzed cohort was 82% female and ranged in age (at baseline) from 9–21 years (M = 15.0 +/− 2.28 years). Multilevel growth models were used to determine time-related trends in symptoms, function, and treatments since completion of baseline evaluation; logistic growth models were used for models with binary outcome variables. Results: Pain scores were in the moderate to severe range at baseline (M = 6.00/10, SD = 2.74) and significantly worsened over time (b = .43 ± .19, t(91) = 2.17, p = .03). Similarly, indicators of function and well-being either worsened over time (for CHAQ: b = .10 ± .05, t(111) = 2.05, p = .04; for subjective well-being: b = .67 ± .21, t(91) = 3.28, p

Background/Purpose: Children and adolescents with juvenile primary fibromyalgia syndrome (JPFS) often present to pediatric rheumatologists for evaluation. Limited data are available on the characteristics of and treatments used for JPFS, particularly in males. Methods: We evaluated deidentified data from baseline visits of JPFS patients entered in the Childhood Arthritis & Rheumatology Research Alliance (CARRA) registry between May 2010 and September 2013. Data regarding demographics, symptoms, functional measures and treatment are compared as a function of gender. Results: There were 172 patients (27 males), ages 8–21 years (Mean (M) = 15.4 +/− 2.3). Patients were symptomatic for a mean of 1.7 +/− 2.2 years prior to their first visit, with no significant difference between males and females (M = 2.2 vs. 1.6 respectively, t(169) = 1.19, p = .2). Widespread pain (89%), fatigue (83%), disordered sleep (77%), headaches (61%), and extremity numbness/tingling (32%) were the most commonly reported symptoms. Females had more numbness and tingling (35% vs. 13% respectively, χ2 = 4.4, p = 0.04). Table 1 lists treatments used. Males were significantly more likely to have used gabapentin (26% vs. 8%, χ2 = 7.6, p

B-cell activating factor (BAFF), a member of the family of TNF-like cytokines, supports the survival and differentiation of B cells. The successful development of belimumab, a human antibody targeting soluble BAFF, has marked an important milestone in the development of biologic therapy for treatment of systemic lupus erythematosus (SLE), although much remains unknown regarding the clinical utility of BAFF inhibition in SLE and other autoimmune diseases. In the present review, we provide an overview of the knowledge concerning BAFF's role in murine and human B-cell development and maturation, as well as the clinical and mechanistic effects of BAFF inhibition in human SLE.