The burden of extracutaneous manifestations in juvenile localized scleroderma: A literature review

IF 9.2 1区医学 Q1 IMMUNOLOGY

Autoimmunity reviews Pub Date : 2025-04-04 DOI:10.1016/j.autrev.2025.103812

Ilaria Liguoro , Gabriele Simonini , Giorgia Martini

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引用次数: 0

Abstract

Objectives

Juvenile Localized Scleroderma (JLS) is an autoimmune disease leading to fibrosis of skin and subcutaneous tissues affecting children, that is characterized by extracutaneous manifestations (ECM) in about 20 % of patients. JLS and ECM can cause severe disabilities, potentially impacting patients' quality of life (QoL). We aimed to systematically review studies reporting ECM in young patients with JLS.

Methods

Pubmed, Cochrane and Scopus databases were approached to identify studies evaluating ECM in children with LS. Selected papers focusing on QoL and multidisciplinary approach were separately analysed.

Results

At the end of the selection process, 15 papers (encompassing 3604 children) focused on the description of ECM were included. Overall, ECM were reported in 958/3604 (26.5 %) children, and the 3 most frequent ones were musculoskeletal (24 %), neurological (10.3 %) and odontostomatological (7.6 %). Six papers (435 patients) focusing on QoL in children with JLS resulted comparable. Three studies focusing on the role of a multidisciplinary team in the management of children and adolescents with JLS and ECM were also selected (216 children).

Conclusions

Almost one-third of patients with JLS may present several clinical problems other than skin lesions that should be managed by a multidisciplinary team. However, evidence on the efficacy of a multispecialty management is still lacking. The impact of ECM on QoL of these patients may be underestimated, as no specifically developed assessment tool has been applied so far, but recently proposed overall disease severity and disease-specific patient-reported outcome measures may improve the evaluation of this important clinical aspect.

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来源期刊

Autoimmunity reviews 医学-免疫学

CiteScore

24.70

自引率

4.40%

发文量

164

审稿时长

21 days

期刊介绍： Autoimmunity Reviews is a publication that features up-to-date, structured reviews on various topics in the field of autoimmunity. These reviews are written by renowned experts and include demonstrative illustrations and tables. Each article will have a clear "take-home" message for readers. The selection of articles is primarily done by the Editors-in-Chief, based on recommendations from the international Editorial Board. The topics covered in the articles span all areas of autoimmunology, aiming to bridge the gap between basic and clinical sciences. In terms of content, the contributions in basic sciences delve into the pathophysiology and mechanisms of autoimmune disorders, as well as genomics and proteomics. On the other hand, clinical contributions focus on diseases related to autoimmunity, novel therapies, and clinical associations. Autoimmunity Reviews is internationally recognized, and its articles are indexed and abstracted in prestigious databases such as PubMed/Medline, Science Citation Index Expanded, Biosciences Information Services, and Chemical Abstracts.