ArticleRheumatology international2020
Serum KL-6 level as a biomarker of interstitial lung disease in childhood connective tissue diseases: a pilot study.
Article in Rheumatology international, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 2 of them syntheses that pooled it.
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Who cites it
18 citing papers in PubMed, 2 syntheses or guidelines pooled it, 24 citations in OpenAlex.
- KL-6 as an Immunological Biomarker Predicts the Severity, Progression, Acute Exacerbation, and Poor Outcomes of Interstitial Lung Disease: A Systematic Review and Meta-Analysis.Frontiers in immunology · 2021Pooled it
- Pooled it
- Case Report: Treatment of refractory lung disease in systemic juvenile idiopathic arthritis with cyclophosphamide and rituximab combination therapy.Frontiers in immunology · 2026Article
- Pulmonary Involvement in Systemic Lupus Erythematosus: A Potentially Overlooked Condition.Biomedicines · 2025Review
- Pediatric Pulmonology 2024 Year in Review: Rare and Diffuse Lung Disease.Pediatric pulmonology · 2025Review
- An update on diagnosis and treatments of childhood interstitial lung diseases.Breathe (Sheffield, England) · 2025Review
- Lung involvement is a risk factor for treatment resistance in patients with polymyositis and dermatomyositis.Archives of rheumatology · 2025Article
- Initial Respiratory System Involvement in Juvenile Idiopathic Arthritis with Systemic Onset Is a Marker of Interstitial Lung Disease: The Results of Retrospective Cohort Study Analysis.Journal of clinical medicine · 2024Article
- Protein profiling in systemic sclerosis patients with different pulmonary complications using proteomic antibody microarray.Arthritis research & therapy · 2024Article
- Emerging Treatments for Childhood Interstitial Lung Disease.Paediatric drugs · 2024Article
- Undercover lung damage in pediatrics - a hot spot in morbidity caused by collagenoses.Frontiers in immunology · 2024Review
- Ocular sarcoidosis in adults and children: update on clinical manifestation and diagnosis.Journal of ophthalmic inflammation and infection · 2023Review
- Clinical Presentation and Treatment of Juvenile Idiopathic Arthritis Combined with Lung Disease: A Narrative Review.Rheumatology and therapy · 2023Review
- Serum KL-6 as a Biomarker of Progression at Any Time in Fibrotic Interstitial Lung Disease.Journal of clinical medicine · 2023Article
- Article
- Serum B-cell activating factor and lung ultrasound B-lines in connective tissue disease related interstitial lung disease.Frontiers in medicine · 2022Article
- The value of KL-6 in the diagnosis and assessment of interstitial lung disease.American journal of translational research · 2021Article
- Pulmonary involvement in systemic sclerosis: exploring cellular, genetic and epigenetic mechanisms.Rheumatology international · 2020Review
Corrections and comments
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Krebs von den Lungen-6 (KL-6) has been described as a promising biomarker in the diagnosis and determining the severity of interstitial lung disease in adults with connective tissue disease. This study was performed to determine whether the serum KL-6 level is useful as a biomarker for detecting the interstitial lung disease (ILD) in pediatric cases of connective tissue disease (CTD). In total, 88 patients [36 patients with systemic juvenile idiopathic arthritis (sJIA), 27 patients with juvenile systemic lupus erythematosus (JSLE), 14 patients with juvenile dermatomyositis (JDM), and 11 patients with juvenile systemic sclerosis (JSSc)] and 68 healthy controls were included in this study. Age, sex, and duration of CTD and ILD (if any) were recorded. Blood samples from all the patients and controls were examined by ELISA. Eleven of the 88 patients with CTD (12.5%) had ILD and all of them were symptomatic. Subgroup analysis indicated that eight patients had JSSc, two had JSLE, and one had systemic JIA. The median serum KL-6 level was 1450.5 U/mL (interquartile range (IQR) 742.9-2603.2) in the CTD with ILD group, 415.9 U/mL (IQR 233.4-748.4) in the CTD without ILD group, and 465.9 U/mL (IQR 273.6-1036) in the control group. KL-6 levels were significantly higher in the CTD with ILD group than the CTD without ILD group and the control group (p = 0.003). At a cut-off of 712.5 U/ml identified by ROC curve, serum KL-6 yielded a sensitivity of 81% and specificity of 72% for CTD with ILD group. There was no significant difference in serum KL-6 level among the disease subgroups (sJIA, JSLE, JSSc, JDM), in either the CTD with ILD group or the CTD without ILD group (p > 0.05). In conclusion, KL-6 is a useful biomarker of CTD with ILD in pediatric patients.
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