Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS)

Li, Jonathan C.; Tadros, Sameh; Rosser, Franziska; Torok, Kathryn S.

doi:10.3390/diagnostics13122103

Cited by 4 publications

(1 citation statement)

References 30 publications

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“…Associations between esophageal damage and respiratory dysfunction in children with jSSc have also been described. An accompanying complication can be aspiration pneumonia following esophageal dysmotility associated with jSSc ( 17 , 32 , 95 – 97 ). It should be mentioned that chest x-rays do not show pathological aspects except in the late stages of ILD, thus being included in the category of insensitive diagnostic methods.…”

Section: Types Of Pulmonary Damagementioning

confidence: 99%

Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses

Lupu,

Sasaran,

Jechel

et al. 2024

Front. Immunol.

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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.

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Section: Types Of Pulmonary Damagementioning

confidence: 99%

Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses

Lupu,

Sasaran,

Jechel

et al. 2024

Front. Immunol.

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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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Approach to Pulmonary Nodules in Connective Tissue Disease

Gaffney,

Murphy

2024

Semin Respir Crit Care Med

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The assessment of pulmonary nodules is a common and often challenging clinical scenario. This evaluation becomes even more complex in patients with connective tissue diseases (CTDs), as a range of disease-related factors must also be taken into account. These diseases are characterized by immune-mediated chronic inflammation, leading to tissue damage, collagen deposition, and subsequent organ dysfunction. A thorough examination of nodule features in these patients is required, incorporating anatomic and functional information, along with patient demographics, clinical factors, and disease-specific knowledge. This integrated approach is vital for effective risk stratification and precise diagnosis. This review article addresses specific CTD-related factors that should be taken into account when evaluating pulmonary nodules in this patient group.

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Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS)

Cited by 4 publications

References 30 publications

Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses

Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses

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

Approach to Pulmonary Nodules in Connective Tissue Disease

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