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Sugar Land, TX, United States

Horneff G.,Asklepios Clinic Sankt Augustin | Foeldvari I.,Hamburger Zentrum fur Kinder und Jugendrheumatologie | Minden K.,Childrens University Hospital Charite and Epidemiology Unit | Moebius D.,Carl Thiem Clinics | Hospach T.,Pediatric Rheumatology

Objectives. TNF inhibitors have markedly improved the therapeutic options for JIA patients. The report on malignancies observed in children exposed to TNF inhibitors flagged up questions about a potential increased risk especially for lymphoma. Method. Cases with malignancies observed in the German registry have been reviewed to give detailed information on the patients' pretreatment and outcome. Results. From 2001 to 2009, five cases of malignancies were documented in the German JIA biologics registry actually covering 1260 patients, one of each non-Hodgkin's lymphoma, Hodgkin's lymphoma, thyroid carcinoma, yolk sac carcinoma and cervical dysplasia. All patients have been exposed to a number of cytotoxic drugs including MTX, LEF, AZA, CSA before institution of TNF-α blockers. The malignant diagnosis has been given after exposure to etanercept for 3 weeks up to >6 years. Two patients have also been exposed to adalimumab or infliximab. At occurrence of the malignancy, three patients were still on etanercept, four on MTX and one on infliximab. In three patients, the malignancy occurred at adulthood. All patients recovered. Conclusion. While in some of the patients exposed to TNF inhibitors the exposure time was too short for a meaningful relationship, in others the extensive pretreatment may also be causative. Two lymphomas have been observed while other entities occurred only once. JIA patients exposed to biologics or cytotoxic drugs have to be followed carefully and long-term observation has to be continued in adulthood. © The Author 2010. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. Source

Santos J.A.,Hospital Dona Estefania | Arostegui J.I.,Hospital Clinic IDIBAPS | Brito M.J.,Pediatric Infectious Diseases Unit | Neves C.,Primary Immunodeficiency | Conde M.,Pediatric Rheumatology

Hyperimmunoglobulinemia D and periodic fever syndrome (HIDS; MIM# 260920) is a rare recessively-inherited autoinflammatory condition caused by mutations in the MVK gene, which encodes for mevalonate kinase, an essential enzyme in the isoprenoid pathway. HIDS is clinically characterized by recurrent episodes of fever and inflammation. Here we report on the case of a 2. year-old Portuguese boy with recurrent episodes of fever, malaise, massive cervical lymphadenopathy and hepatosplenomegaly since the age of 12. months. Rash, arthralgia, abdominal pain and diarrhea were also seen occasionally. During attacks a vigorous acute-phase response was detected, including elevated erythrocyte sedimentation rate, C-reactive protein, serum amyloid A and leukocytosis. Clinical and laboratory improvement was seen between attacks. Despite normal serum IgD level, HIDS was clinically suspected. Mutational MVK analysis revealed the homozygous genotype with the novel p.Arg277Gly (p.R277G) mutation, while the healthy non-consanguineous parents were heterozygous. Short nonsteroidal anti-inflammatory drugs and corticosteroid courses were given during attacks with poor benefits, whereas anakinra showed positive responses only at high doses. The p.R277G mutation here described is a novel missense MVK mutation, and it has been detected in this case with a severe HIDS phenotype. Further studies are needed to evaluate a co-relation genotype, enzyme activity and phenotype, and to define the best therapeutic strategies. © 2014 Elsevier B.V. Source

Aguiar C.L.,Cohen Childrens Medical Centernorth Shore Long Island Jewish Medical Center | Soybilgic A.,University of Illinois at Chicago | Avcin T.,University of Ljubljana | Myones B.L.,Pediatric Rheumatology
Current Rheumatology Reports

Antiphospholipid syndrome (APS) is a multisystem autoimmune condition characterized by vascular thromboses associated with persistently positive antiphospholipid antibodies. There is currently a paucity of data (incidence, prevalence, thrombosis risk, and effective treatment) in pediatric APS. The purpose of this report is to review the current literature on APS in children and neonates, identify the gaps in current knowledge, and suggest avenues for studies to fill those gaps. © 2015, Springer Science+Business Media New York. Source

Goldzweig O.,Pediatric Rheumatology | Carrasco R.,Dell | Hashkes P.J.,Pediatric Rheumatology Unit | Hashkes P.J.,Case Western Reserve University
Seminars in Arthritis and Rheumatism

Objectives: Intraarticular corticosteroid injections are an important part of the treatment for juvenile idiopathic arthritis due to the ability to achieve high concentration of the medication in the affected joint, while minimizing potential systemic adverse effects. There may be some systemic absorption of corticosteroids resulting in systemic adverse events. Our aim was to demonstrate the potential of adverse events due to the systemic absorption of intraarticular corticosteroids through presentation of 2 case reports, a review of our practices and a systematic review of the literature. Methods: We reviewed the intraarticular injections performed at our 3 centers in 2010 and 2011 for the prevalence of systemic adverse events. We searched PubMed for articles in English on systemic adverse effects of intraarticular corticosteroid injection in children, using numerous keywords, as well as review articles and textbooks on juvenile rheumatoid/idiopathic arthritis up to and including December 2011. Results: We report the development of severe acneiform rashes in 2 adolescents with juvenile idiopathic arthritis following bilateral knee intraarticular injections of triamcinolone hexacetonide. The prevalence of systemic adverse events at our centers was in 4/179 (2.2%) injections, the 2 cases reported above, 1 case of insomnia in a 2-year-old child and 1 case of cushingoid features following injection of 21 joints. While in the literature there are some reports of general "Cushing-like" appearances, there are only very few reports of specific skin and other organ/system adverse effects resulting from systemic corticosteroid absorption. Conclusion: It is important to recognize the potential of rare adverse events that are attributable to the systemic absorption of intraarticular corticosteroids in children. © 2012 Elsevier Inc. Source

Robinson A.B.,Pediatric Rheumatology | Hoeltzel M.F.,University of Missouri - Kansas City | Wahezi D.M.,Childrens Hospital at Montefiore | Becker M.L.,University of Missouri - Kansas City | And 6 more authors.
Arthritis Care and Research

Objective To investigate aspects of juvenile dermatomyositis (DM), including disease characteristics and treatment, through a national multicenter registry. Methods Subjects meeting the modified Bohan and Peter criteria for definite juvenile DM were analyzed from the cross-sectional Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry between 2010 and 2012 from 55 US pediatric rheumatology centers. Demographics, disease characteristics, diagnostic assessments, and medication exposure data were collected at enrollment. Results A total of 384 subjects met the criteria for analysis. At enrollment, the median Childhood Myositis Assessment Scale score was 51 (interquartile range [IQR] 46-52), the median Childhood Health Assessment Questionnaire score was 0 (IQR 0-0.5), and the median physician and subject global assessment scores were 1 (IQR 0-2) and 1 (IQR 0-3), respectively, out of a maximum of 10. Of the diagnostic assessments, magnetic resonance imaging was more likely than electromyography or muscle biopsy to show abnormalities. A total of 329 subjects had ≥2 diagnostic studies performed, and >34% of these subjects reported ≥1 negative study. Ninety-five percent had been treated with corticosteroids and 92% with methotrexate, suggesting that these medications were almost universally prescribed for juvenile DM in the US. Conclusion In 2 years, the ongoing CARRA Registry has collected clinical data on 384 children with juvenile DM and has the potential to become one of the largest juvenile DM cohorts in the world. More research is needed about prognostic factors in juvenile DM, and differences in therapy based on manifestations of disease need to be explored by practitioners. This registry provides the infrastructure needed to advance clinical and translational research and represents a major step toward improving outcomes of children with juvenile DM. Copyright © 2014 by the American College of Rheumatology. Source

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