Editor(s): Ferguson, Polly J.; Haberman, Rebecca H.
doi : 10.1097/BOR.0000000000000820
September 2021 - Volume 33 - Issue 5 - p v
Kim, Hanna
doi : 10.1097/BOR.0000000000000816
September 2021 - Volume 33 - Issue 5 - p 371-377
This review provides updates regarding the role of interferon (IFN) in juvenile dermatomyositis (JDM), including comparison to interferonopathies and therapeutic implications.
Roberts, Jordan E.; Henderson, Lauren A.
doi : 10.1097/BOR.0000000000000818
September 2021 - Volume 33 - Issue 5 - p 378-386
To review diagnosis, clinical characteristics and treatment of multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2).
Abinun, Marioa; Slatter, Mary A.b
doi : 10.1097/BOR.0000000000000823
September 2021 - Volume 33 - Issue 5 - p 387-397
A small proportion of children affected by rheumatic diseases suffer from severe, progressive disease, resistant to conventional antirheumatic therapies and to biologic agents interfering with inflammatory cytokines, costimulatory molecules expressed on immune system cells and intracellular signalling pathways. Adding to the poor prognosis is a high risk from significant morbidity and mortality associated with long-term treatment with multiple, often combined anti-inflammatory and immunosuppressive agents. Carefully selected patients from this unfortunate group may benefit from treatment with haematopoietic stem cell transplantation.
Ozen, Seza
doi : 10.1097/BOR.0000000000000821
September 2021 - Volume 33 - Issue 5 - p 398-402
Familial Mediterranean fever (FMF) is the prototypic autoinflammatory disease. Although the gene associated with the disease was identified 24 years ago, we still have to learn about the pathogenesis of its inflammation and the variation in the phenotype. In this review, we discuss some recent findings in FMF, such as changes in our understanding of the genetics, aims to define new criteria, and factors contributing to the disease presentation.
Randell, Rachel L.a; Becker, Mara L.a,b
doi : 10.1097/BOR.0000000000000811
September 2021 - Volume 33 - Issue 5 - p 403-408
Biologics and novel targeted therapeutics have transformed the management of pediatric rheumatic diseases over the past two decades; however, questions about short-term and long-term safety remain. Safety data gathered from recent clinical trials, long-term extensions of prior trials, registries, and other real-world evidence are summarized here for biologics and novel therapeutics commonly prescribed for pediatric rheumatic diseases.
Haberman, Rebecca H.a; Jaros, Brian D.b; Scher, Jose U.a
doi : 10.1097/BOR.0000000000000813
September 2021 - Volume 33 - Issue 5 - p 409-411
Sparks, Jeffrey A.a; Wallace, Zachary S.b; Robinson, Philip C.c,d
doi : 10.1097/BOR.0000000000000812
September 2021 - Volume 33 - Issue 5 - p 412-418
Although the literature to date on COVID-19 outcomes in those with immune-mediated inflammatory disease has been largely reassuring there remain many unanswered questions. These include the impact of specific medications on outcomes and the antibody response after COVID-19 vaccination.
Mehta, Pujaa,b; Fajgenbaum, David C.c
doi : 10.1097/BOR.0000000000000822
September 2021 - Volume 33 - Issue 5 - p 419-430
The COVID-19 pandemic is a global public health crisis with considerable mortality and morbidity. A role for cytokine storm and therapeutic immunomodulation in a subgroup of patients with severe COVID-19 was proposed early in the pandemic. The concept of cytokine storm in COVID-19 has been criticised, given the lack of clear definition and relatively modest cytokinaemia (which may be necessary for viral clearance) compared with acute respiratory distress syndrome and bacterial sepsis. Here we consider the arguments for and against the concept of cytokine storm in COVID-19.
Sattui, Sebastian E.a; Crow, Mary K.a; Navarro-Millán, Irisa,b
doi : 10.1097/BOR.0000000000000817
September 2021 - Volume 33 - Issue 5 - p 431-445
Given the role of inflammation in severe forms of COVID-19, glucocorticoids and disease-modifying antirheumatic drugs (DMARDs) have been assessed as potential COVID-19 therapies.
Wahezi, Dawn M.; Peskin, Malki; Tanner, Tamara
doi : 10.1097/BOR.0000000000000814
September 2021 - Volume 33 - Issue 5 - p 446-452
The purpose of this review is to discuss the clinical management of children with pediatric rheumatic disease (PRD) during the Coronavirus disease of 2019 (COVID-19) pandemic, as well as the unique role of the pediatric rheumatologist during a time of emerging post-COVID inflammatory sequelae including, multisystem inflammatory syndrome in children (MIS-C).
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