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

IF 2.7 3区医学 Q1 PEDIATRICS

Pediatric Pulmonology Pub Date : 2024-11-15 DOI:10.1002/ppul.27347

Gabriele Di Pasquale, Nicholas Caione, Alessio Di Berardino, Giulio Di Donato

引用次数: 0

Abstract

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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幼年系统性硬化症与成年系统性硬化症的肺部表现：病理生理学和临床特征研究。

幼年系统性硬化症（jSSc）是系统性硬化症（SSc）的儿科对应疾病，是一种罕见的自身免疫性疾病，以血管病变和纤维化紊乱为特征。它是发病率和死亡率最高的风湿病之一，主要影响女性。虽然 jSSc 的分类仍未得到普遍认可，但 2007 年提出的临时分类综合了主要标准和次要标准，反映了不同器官和组织的受累情况。肺部表现在 jSSc 中较为常见，占病例的 36% 至 55%。肺部并发症主要包括儿童间质性肺病（chILD）、肺动脉高压（PAH）和肺结节。本文旨在描述 jSSc 患者的主要肺部表现与 SSc 的关系，并根据最新的文献数据强调基本的病理生理学和临床特征。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Pulmonology 医学-呼吸系统

CiteScore

6.00

自引率

12.90%

发文量

468

审稿时长

3-8 weeks

期刊介绍： Pediatric Pulmonology (PPUL) is the foremost global journal studying the respiratory system in disease and in health as it develops from intrauterine life though adolescence to adulthood. Combining explicit and informative analysis of clinical as well as basic scientific research, PPUL provides a look at the many facets of respiratory system disorders in infants and children, ranging from pathological anatomy, developmental issues, and pathophysiology to infectious disease, asthma, cystic fibrosis, and airborne toxins. Focused attention is given to the reporting of diagnostic and therapeutic methods for neonates, preschool children, and adolescents, the enduring effects of childhood respiratory diseases, and newly described infectious diseases. PPUL concentrates on subject matters of crucial interest to specialists preparing for the Pediatric Subspecialty Examinations in the United States and other countries. With its attentive coverage and extensive clinical data, this journal is a principle source for pediatricians in practice and in training and a must have for all pediatric pulmonologists.