PDF(Pubmed)
Abstract：
Connective tissue represents the support matrix and the connection between tissues and organs. In its composition, collagen, the major structural protein, is the main component of the skin, bones, tendons and ligaments. Especially at the pediatric age, its damage in the context of pathologies such as systemic lupus erythematosus, scleroderma or dermatomyositis can have a significant negative impact on the development and optimal functioning of the body. The consequences can extend to various structures (e.g., joints, skin, eyes, lungs, heart, kidneys). Of these, we retain and reveal later in our manuscript, mainly the respiratory involvement. Manifested in various forms that can damage the chest wall, pleura, interstitium or vascularization, lung damage in pediatric systemic inflammatory diseases is underdeveloped in the literature compared to that described in adults. Under the threat of severe evolution, sometimes rapidly progressive and leading to death, it is necessary to increase the popularization of information aimed at physiopathological triggering and maintenance mechanisms, diagnostic means, and therapeutic directions among medical specialists. In addition, we emphasize the need for interdisciplinary collaboration, especially between pediatricians, rheumatologists, infectious disease specialists, pulmonologists, and immunologists. Through our narrative review we aimed to bring up to date, in a concise and easy to assimilate, general principles regarding the pulmonary impact of collagenoses using the most recent articles published in international libraries, duplicated by previous articles, of reference for the targeted pathologies.
摘要：
结缔组织代表支持基质以及组织和器官之间的连接。在其组成中，胶原蛋白，主要的结构蛋白，是皮肤的主要组成部分，骨头,肌腱和韧带。尤其是在儿科时代，它在诸如系统性红斑狼疮等病理背景下的损害，硬皮病或皮肌炎可能对身体的发育和最佳功能产生重大负面影响。后果可以扩展到各种结构(例如，接头,皮肤,眼睛,肺,心,肾脏)。其中,我们在手稿中保留并揭示，主要是呼吸道的参与。表现为各种形式，可能会损坏胸壁，胸膜,间质或血管形成，与成人相比，文献中小儿全身性炎症性疾病的肺损伤尚不发达。在严重进化的威胁下，有时迅速进步并导致死亡，有必要增加针对病理生理触发和维持机制的信息的普及，诊断手段，以及医学专家之间的治疗指导。此外,我们强调需要跨学科合作，尤其是儿科医生之间，风湿病学家,传染病专家,肺病学家,和免疫学家。通过我们的叙事回顾，我们旨在更新最新信息，在一个简洁和易于吸收，使用国际图书馆发表的最新文章，关于胶原酶对肺部影响的一般原则，重复以前的文章，有针对性的病理参考。
