Best clinical practice in the treatment of juvenile systemic sclerosis: expert panel guidance - the result of the International Hamburg Consensus Meeting December 2022

Foeldvari, Ivan; Torok, Kathryn S.; Antón, Jordi; Blakley, Michael; Constantin, Tamás; Cutolo, Maurizio; Denton, Christopher P.; Fligelstone, Kim; Hinrichs, Bernd; Li, Suzanne C.; Maillard, Susan; Marrani, Edoardo; Moinzadeh, Pia; Orteu, Catherine H.; Pain, Clare E.; Pauling, John D.; Pilkington, Clarissa; Rosser, Franziska; Smith, Vanessa; Furst, Daniel F.

doi:10.1080/1744666x.2023.2298354

Expert Review of Clinical Immunology

2024

DOI: 10.1080/1744666x.2023.2298354

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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

Ivan Foeldvari,

Kathryn S. Torok,

Jordi Antón

et al.

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Cited by 3 publications

References 127 publications

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Pulmonary manifestations of juvenile vs. adult systemic sclerosis: insights into pathophysiological and clinical features

Di Pasquale,

Caione,

Di Berardino

et al. 2024

Pediatric Pulmonology

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Juvenile systemic sclerosis (jSSc), the pediatric counterpart of systemic sclerosis (SSc), is a rare autoimmune disorder characterized by vasculopathy and fibrotic disorders. It ranks among the rheumatologic diseases with the highest rates of morbidity and mortality, predominantly impacting females. Although a universally accepted classification for jSSc remains elusive, a provisional classification proposed in 2007 integrates major and minor criteria, reflecting the involvement of diverse organs and tissues. Pulmonary manifestations are relatively common in jSSc, occurring in 36% to 55% of cases. Particularly lung complications include children s interstitial lung disease (chILD), pulmonary arterial hypertension (PAH) and nodules. The aim of this paper is to describe the main pulmonary manifestations of patients with jSSc in relation to SSc, highlighting fundamental pathophysiological, and clinical features based on the latest literature data.

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Pulmonary manifestations of juvenile vs. adult systemic sclerosis: insights into pathophysiological and clinical features

Di Pasquale,

Caione,

Di Berardino

et al. 2024

Pediatric Pulmonology

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The 2024 British Society for Rheumatology guideline for management of systemic sclerosis

Denton,

De Lorenzis,

Roblin

et al. 2024

Rheumatology

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This guideline was developed according to the British Society for Rheumatology Guidelines Protocol by a Guideline Development Group comprising healthcare professionals with expertise in SSc and people with lived experience, as well as patient organization representatives. It is an update of the previous 2015 SSc guideline. The recommendations were developed and agreed by the group and are underpinned by published evidence, assessed by systematic literature review and reinforced by collective expert opinion of the group. It considers all aspects of SSc including general management, treatment of organ-based complications, including cardiopulmonary, renal and gastrointestinal tract manifestations, as well as broader impact of disease. Whilst it is focused on adults with SSc we expect that the guideline will be relevant to people of all ages and expert input and review by paediatric rheumatologists and other relevant specialists considered where the guideline was, or may not be, applicable to young people with SSc and juvenile-onset disease. In addition to providing guidance on disease assessment and management the full guideline also considers service organization within the National Health Service and future approaches to audit of the guideline. The lay summary that accompanies this abstract can be found in Supplemental information 1.

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What are Juvenile-onset systemic sclerosis providers thoughts, experiences, and reasons for autologous stem cell transplant? Result of a multinational survey

Foeldvari,

Branton,

et al. 2024

Journal of Scleroderma and Related Disorders

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Objective: Juvenile-onset systemic sclerosis (jSSc) is a rare and life-threatening disease with no formal studies evaluating the indications for, access to, or benefits of autologous stem cell transplantation (ASCT). As a first step toward understanding pediatric jSSc specialist thoughts and experiences with ASCT, we conducted a multinational survey. Methods: An electronic survey was developed and distributed in November 2023 to members of the Pediatric Rheumatology European Society (PRES) and/or Childhood Arthritis and Rheumatology Research Alliance (CARRA) pediatric scleroderma workgroups. Results: Twenty-nine (69%) jSSc specialists completed the survey. All participants have considered or would consider ASCT referral for a jSSc patient. Nearly all respondents indicated disease-modifying anti-rheumatic drugs (DMARDs) should be trialed prior to ASCT referral, with most indicating two to four DMARDs. The most common reasons selected for referral were rapidly progressive disease (despite DMARD) (90%), followed by severe disease status (83%), and significant impact on quality of life (83%). All respondents selected pulmonary disease as an indication for referral, followed by cardiac (93%), gastrointestinal (72%), and skin disease (66%). While pulmonary and cardiac involvement were considered individually sufficient for referral for ASCT, only a minority considered musculoskeletal involvement (28%) sufficient on its own. Conclusion: This survey is the first explore thoughts and experience with ASCT for jSSc. Results indicate pediatric rheumatologists were aware of and would consider ASCT for their patients. Our results indicate there is likely some variability in clinical practice regarding who is referred for ASCT, and further research is needed to guide development of evidence-based clinical care guidelines.

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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

Cited by 3 publications

References 127 publications

Pulmonary manifestations of juvenile vs. adult systemic sclerosis: insights into pathophysiological and clinical features

Pulmonary manifestations of juvenile vs. adult systemic sclerosis: insights into pathophysiological and clinical features

The 2024 British Society for Rheumatology guideline for management of systemic sclerosis

What are Juvenile-onset systemic sclerosis providers thoughts, experiences, and reasons for autologous stem cell transplant? Result of a multinational survey

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