Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS)
Background: Juvenile systemic sclerosis (jSSc) is a systemic inflammatory and fibrotic autoimmune disease. Adult guidelines recommend obtaining a screening high-resolution computed tomography scan (CT) at diagnosis. As these recommendations are adopted as standard of care for jSSc, increased screeni...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-06-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/13/12/2103 |
| _version_ | 1797595289611141120 |
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| author | Jonathan C. Li Sameh Tadros Franziska Rosser Kathryn S. Torok |
| author_facet | Jonathan C. Li Sameh Tadros Franziska Rosser Kathryn S. Torok |
| author_sort | Jonathan C. Li |
| collection | DOAJ |
| description | Background: Juvenile systemic sclerosis (jSSc) is a systemic inflammatory and fibrotic autoimmune disease. Adult guidelines recommend obtaining a screening high-resolution computed tomography scan (CT) at diagnosis. As these recommendations are adopted as standard of care for jSSc, increased screening with CT may lead to increased detection of nodules. The implications of nodules identified in jSSc are unclear and unreported. Methods: A retrospective chart review was performed on the prospectively enrolled National Registry for Childhood-Onset Scleroderma (NRCOS) cohort over an enrollment period of 20 years. Clinical associations with presence of nodules and nodule characteristics were investigated. Results: In this jSSc cohort, the prevalence of pulmonary nodules was 31% (<i>n</i> = 17 of 54). Nodule characteristics were heterogeneous, and most displayed stability over time. More participants with nodules had structural esophageal abnormalities, restriction, and reduced diffusing capacity on lung function tests, and follow-up imaging. Most participants had multiple nodules, and although most nodules were <5 mm, most participants had at least one nodule >5 mm. Conclusions: Pulmonary nodules are seen in children with jSSc and may be related to more severe disease and/or esophageal dysfunction. More work is needed to provide guidance on radiologic follow-up and clinical management of pulmonary nodules in jSSc. |
| first_indexed | 2024-03-11T02:35:18Z |
| format | Article |
| id | doaj.art-e26ab3b568264de191304f1c150ae3df |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-11T02:35:18Z |
| publishDate | 2023-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-e26ab3b568264de191304f1c150ae3df2023-11-18T10:01:07ZengMDPI AGDiagnostics2075-44182023-06-011312210310.3390/diagnostics13122103Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS)Jonathan C. Li0Sameh Tadros1Franziska Rosser2Kathryn S. Torok3UPMC Internal Medicine and Pediatrics Residency Program, University of Pittsburgh, Pittsburgh, PA 15224, USADepartment of Pediatrics, Division of Radiology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, USADepartment of Pediatrics, Division of Pulmonary Medicine, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, USADepartment of Pediatrics, Division of Rheumatology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, USABackground: Juvenile systemic sclerosis (jSSc) is a systemic inflammatory and fibrotic autoimmune disease. Adult guidelines recommend obtaining a screening high-resolution computed tomography scan (CT) at diagnosis. As these recommendations are adopted as standard of care for jSSc, increased screening with CT may lead to increased detection of nodules. The implications of nodules identified in jSSc are unclear and unreported. Methods: A retrospective chart review was performed on the prospectively enrolled National Registry for Childhood-Onset Scleroderma (NRCOS) cohort over an enrollment period of 20 years. Clinical associations with presence of nodules and nodule characteristics were investigated. Results: In this jSSc cohort, the prevalence of pulmonary nodules was 31% (<i>n</i> = 17 of 54). Nodule characteristics were heterogeneous, and most displayed stability over time. More participants with nodules had structural esophageal abnormalities, restriction, and reduced diffusing capacity on lung function tests, and follow-up imaging. Most participants had multiple nodules, and although most nodules were <5 mm, most participants had at least one nodule >5 mm. Conclusions: Pulmonary nodules are seen in children with jSSc and may be related to more severe disease and/or esophageal dysfunction. More work is needed to provide guidance on radiologic follow-up and clinical management of pulmonary nodules in jSSc.https://www.mdpi.com/2075-4418/13/12/2103juvenile systemic sclerosispulmonary noduleschildhood lung diseaseesophageal abnormalitieshigh-resolution computed tomography scan (HRCT)interstitial lung disease (ILD) |
| spellingShingle | Jonathan C. Li Sameh Tadros Franziska Rosser Kathryn S. Torok Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS) Diagnostics juvenile systemic sclerosis pulmonary nodules childhood lung disease esophageal abnormalities high-resolution computed tomography scan (HRCT) interstitial lung disease (ILD) |
| title | Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS) |
| title_full | Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS) |
| title_fullStr | Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS) |
| title_full_unstemmed | Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS) |
| title_short | Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS) |
| title_sort | pulmonary nodules in juvenile systemic sclerosis a case series from the national registry for childhood onset scleroderma nrcos |
| topic | juvenile systemic sclerosis pulmonary nodules childhood lung disease esophageal abnormalities high-resolution computed tomography scan (HRCT) interstitial lung disease (ILD) |
| url | https://www.mdpi.com/2075-4418/13/12/2103 |
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