A case of neonatal sweet syndrome associated with mevalonate kinase deficiency
Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an immunologic syndrome characterized by widespread neutrophilic infiltration. Histiocytoid Sweet syndrome (H-SS) is a histopatholog... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - September 12, 2023 Category: Rheumatology Authors: Margaret Irwin, Veeraya K. Tanawattanacharoen, Amy Turner, Mary Beth F. Son, Rebecca C. Hale, Craig D. Platt, Juan Putra, Birgitta A.R. Schmidt and Mollie G. Wasserman Tags: Case Report Source Type: research

Panniculitis with late onset enthesitis-related arthritis: a case report
Panniculitis, a type of inflammation of subcutaneous fat, is a relatively uncommon condition that usually presents as inflammatory nodules or plaques, with various proposed etiologic factors. The association b... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - September 11, 2023 Category: Rheumatology Authors: Wenxiu Mo, Fei Sun, Tongxin Han and Huawei Mao Tags: Case Report Source Type: research

Disease evolution in systemic juvenile idiopathic arthritis: an international, observational cohort study through JIRcohort
This study aims to identify predictors of disease evolution within the systemic JIA populati... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - September 7, 2023 Category: Rheumatology Authors: M. Wallimann, K. Bouayed, E. Cannizzaro, D. Kaiser, A. Belot, E. Merlin, S. Poignant, C. Wouters, F. Hofer, T. Saurenmann, A. Koryllou, R. Carlomagno, M. Mejbri, M. Hofer and K. Theodoropoulou Tags: Research article Source Type: research

Survey of adolescents ’ needs and parents’ views on sexual health in juvenile idiopathic arthritis
Although the advent of new therapeutics for juvenile idiopathic arthritis (JIA) patients has considerably lessened the impact of the disease and reduced its sequelae, the outcomes of JIA remain important in th... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - September 5, 2023 Category: Rheumatology Authors: Carine Savel, Aur élie Chausset, Pauline Berland, Candy Guiguet-Auclair, Laura Cabane, Bruno Fautrel, Philippe Gaudin, Pascale Guillot, Gilles Hayem, Delphine Lafarge, Etienne Merlin, Nadine Pezière, Christelle Sordet, Sonia Trope, Anne Tournadre, Sandr Tags: Research article Source Type: research

Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children; by definition, episodes occur every 2 to 8 weeks. However, in a ... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - September 1, 2023 Category: Rheumatology Authors: Mor Broide, Yoel Levinsky, Rotem Tal, Liora Harel, Shoval Shoham, Sabreen Abu Ahmad, Yonatan Butbul Aviel and Gil Amarilyo Tags: Research article Source Type: research

Disease characteristics of HLA-B27 positive and negative finnish patients with juvenile idiopathic arthritis - results of the 18-year cohort follow-up study
The aim of this long-term follow-up study was to compare the disease characteristics of HLA-B27 positive and negative patients with juvenile idiopathic arthritis (JIA). (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - September 1, 2023 Category: Rheumatology Authors: Suvi Oili Orvokki Peltoniemi, Mia Glerup, Pekka Lahdenne, Kari K Eklund and Kristiina Aalto Tags: Research article Source Type: research

Successful experience of tofacitinib treatment in patients with Fibrodysplasia Ossificans Progressiva
Fibrodysplasia ossificans progressive (FOP) is an ultra-rare genetic disorder that is caused by a mutation in the ACVR1 gene and provokes severe heterotopic ossification. Since flares of the disease are associate... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - August 29, 2023 Category: Rheumatology Authors: Irina P. Nikishina, Svetlana V. Arsenyeva, Valeria G. Matkava, Alia N. Arefieva, Mariya I. Kaleda, Alexandr V. Smirnov, Leonid M. Blank and Mikhail M. Kostik Tags: Research article Source Type: research

Significant pain decrease in children with non-systemic Juvenile Idiopathic Arthritis treated to target: results over 24 months of follow up
The aim of this study was to compare pain-scores in three targeted treatment-strategies in JIA-patients and to identify characteristics predicting persistent pain. (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - August 26, 2023 Category: Rheumatology Authors: Katinka Spekking, Janneke Anink, Piroska de Boer, Sytske Anne Bergstra, J. Merlijn van den Berg, Dieneke Schonenberg-Meinema, Lisette W. A. van Suijlekom-Smit, Marion A. J. van Rossum, Yvonne Koopman-Keemink, Rebecca ten Cate, Cornelia F. Allaart, Dani ë Tags: Research article Source Type: research

Pediatric-onset limited ANCA-associated vasculitis arising during pre-existing chronic recurrent multifocal osteomyelitis
Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by chronic vasculitis involving small to medium sized arteries, granulomatous inflammation of the upper and lower respiratory tract... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - August 24, 2023 Category: Rheumatology Authors: Esraa Eloseily and Michael Henrickson Tags: Case Report Source Type: research

Elevated serum IFN- γand IFN-γ/IL-6 ratio in Kikuchi-Fujimoto disease
In this study, we aim... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - August 22, 2023 Category: Rheumatology Authors: Tingyan He, Zixuan Shen, Jiayun Ling, Xiaona Zhu and Jun Yang Tags: Research article Source Type: research

A novel PRKDC mutation caused B lymphocytes V(D)J rearrangement disorder in the SLE-DAH like symptoms patient
Analyzed the clinical features and treatment process of the patient suffering from immunodeficiency with systemic lupus erythematosus(SLE)-like syndrome in a novel mutation of PRKDC. (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - August 14, 2023 Category: Rheumatology Authors: Hongwei Li, Yawen Zhang, Biyun Zhang and Dehui Chen Tags: Case Report Source Type: research

A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children
IgA vasculitis (IgAV) is a small vessel vasculitis that is more common in childhood. Very limited evidence exists on patients who experience an atypical disease course. The aim of this study was to describe a ... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - August 14, 2023 Category: Rheumatology Authors: Julien Marro, Chloe Williams, Clare E. Pain and Louise Oni Tags: Research article Source Type: research

Long-term efficacy of sialendoscopy in treating childhood Sj ögren’s disease with chronological monitoring by salivary gland ultrasonography: A novel approach
Childhood Sj ögren’s Disease (cSjD) is an underdiagnosed phenomenon with clinical and pathophysiological nuances in contrast to Sjögren’s Disease (SjD) in the adult population. While adults typically experience... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - August 13, 2023 Category: Rheumatology Authors: Kristin Drew, Ren é Kronlage, Seunghee Cha, Akaluck Thatayatikom and Thomas Schrepfer Tags: Case Report Source Type: research

Impact of SARS-CoV-2 on the clinical presentation of juvenile idiopathic inflammatory myopathies
Growing evidence suggests that infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may trigger idiopathic inflammatory myopathies (IIM). Few studies have described individual juvenile I... (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - August 11, 2023 Category: Rheumatology Authors: Jessica Perfetto, Donna A. Yoo, Carolina Y. Tamashiro, Megan M. Perron, Natalia Vasquez-Canizares and Dawn M. Wahezi Tags: Research article Source Type: research

Monoarticular juvenile idiopathic arthritis as a distinct clinical entity A proof-of-concept study
Currently, monoarticular Juvenile Idiopathic Arthritis (monoJIA) is included in the ILAR classification as oligoarticular subtype although various aspects, from clinical practice, suggest it as a separate entity. (Source: Pediatric Rheumatology)
Source: Pediatric Rheumatology - August 10, 2023 Category: Rheumatology Authors: Francesco Zulian, Beatrice Pierobon, Maria Elisabetta Zannin, Caterina Politi, Fabio Vittadello, Alessandra Meneghel, Francesca Tirelli and Giorgia Martini Tags: Research article Source Type: research