Ivan Foeldvari, Kathryn S Torok, Jordi Antón, Michael Blakley, Tamás Constantin, Maurizio Cutolo, Christopher P Denton, Kim Fligelstone, Bernd Hinrichs, Suzanne C Li, Susan Maillard, Edoardo Marrani, Pia Moinzadeh, Catherine H Orteu, Clare E Pain, John D Pauling, Clarissa Pilkington, Franziska Rosser, Vanessa Smith, Daniel F Furst
{"title":"治疗幼年系统性硬化症的最佳临床实践:专家组指南--2022 年 12 月汉堡国际共识会议的成果。","authors":"Ivan Foeldvari, Kathryn S Torok, Jordi Antón, Michael Blakley, Tamás Constantin, Maurizio Cutolo, Christopher P Denton, Kim Fligelstone, Bernd Hinrichs, Suzanne C Li, Susan Maillard, Edoardo Marrani, Pia Moinzadeh, Catherine H Orteu, Clare E Pain, John D Pauling, Clarissa Pilkington, Franziska Rosser, Vanessa Smith, Daniel F Furst","doi":"10.1080/1744666X.2023.2298354","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently there is only one consensus treatment guideline concerning skin, pulmonary and vascular involvement for jSSc, the jSSc SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) initiative, which was based on data procured up to 2014. Therefore, an update of these guidelines, with a more recent literature and expert experience, and extension of the guidance to more aspects of the disease is needed.</p><p><strong>Areas covered: </strong>Treatment options were reviewed, and opinions were provided for most facets of jSSc including general management, some of which differs from adult systemic sclerosis, such as the use of corticosteroids, and specific organ involvement, such as skin, musculoskeletal, pulmonary, and gastroenterology.</p><p><strong>Expert opinion: </strong>We are suggesting the treat to target strategy to treat early to prevent cumulative disease damage in jSSc. Conclusions are derived from both expert opinion and available literature, which is mostly based on adult systemic sclerosis (aSSc), given shared pathophysiology, extrapolation of results from aSSc studies was judged reasonable.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":null,"pages":null},"PeriodicalIF":3.9000,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Best clinical practice in the treatment of juvenile systemic sclerosis: expert panel guidance - the result of the International Hamburg Consensus Meeting December 2022.\",\"authors\":\"Ivan Foeldvari, Kathryn S Torok, Jordi Antón, Michael Blakley, Tamás Constantin, Maurizio Cutolo, Christopher P Denton, Kim Fligelstone, Bernd Hinrichs, Suzanne C Li, Susan Maillard, Edoardo Marrani, Pia Moinzadeh, Catherine H Orteu, Clare E Pain, John D Pauling, Clarissa Pilkington, Franziska Rosser, Vanessa Smith, Daniel F Furst\",\"doi\":\"10.1080/1744666X.2023.2298354\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. 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Best clinical practice in the treatment of juvenile systemic sclerosis: expert panel guidance - the result of the International Hamburg Consensus Meeting December 2022.
Introduction: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently there is only one consensus treatment guideline concerning skin, pulmonary and vascular involvement for jSSc, the jSSc SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) initiative, which was based on data procured up to 2014. Therefore, an update of these guidelines, with a more recent literature and expert experience, and extension of the guidance to more aspects of the disease is needed.
Areas covered: Treatment options were reviewed, and opinions were provided for most facets of jSSc including general management, some of which differs from adult systemic sclerosis, such as the use of corticosteroids, and specific organ involvement, such as skin, musculoskeletal, pulmonary, and gastroenterology.
Expert opinion: We are suggesting the treat to target strategy to treat early to prevent cumulative disease damage in jSSc. Conclusions are derived from both expert opinion and available literature, which is mostly based on adult systemic sclerosis (aSSc), given shared pathophysiology, extrapolation of results from aSSc studies was judged reasonable.
期刊介绍:
Expert Review of Clinical Immunology (ISSN 1744-666X) provides expert analysis and commentary regarding the performance of new therapeutic and diagnostic modalities in clinical immunology. Members of the International Editorial Advisory Panel of Expert Review of Clinical Immunology are the forefront of their area of expertise. This panel works with our dedicated editorial team to identify the most important and topical review themes and the corresponding expert(s) most appropriate to provide commentary and analysis. All articles are subject to rigorous peer-review, and the finished reviews provide an essential contribution to decision-making in clinical immunology.
Articles focus on the following key areas:
• Therapeutic overviews of specific immunologic disorders highlighting optimal therapy and prospects for new medicines
• Performance and benefits of newly approved therapeutic agents
• New diagnostic approaches
• Screening and patient stratification
• Pharmacoeconomic studies
• New therapeutic indications for existing therapies
• Adverse effects, occurrence and reduction
• Prospects for medicines in late-stage trials approaching regulatory approval
• Novel treatment strategies
• Epidemiological studies
• Commentary and comparison of treatment guidelines
Topics include infection and immunity, inflammation, host defense mechanisms, congenital and acquired immunodeficiencies, anaphylaxis and allergy, systemic immune diseases, organ-specific inflammatory diseases, transplantation immunology, endocrinology and diabetes, cancer immunology, neuroimmunology and hematological diseases.