Pediatric Immune Mediated Interstitial Lung Disease
Conditions
Keywords
Pediatric, interstitial lung disease, immune mediated, IL-6, MM-7
Brief summary
Pediatric interstitial lung disease (ILD) represents a heterogeneous group of rare but potentially severe pulmonary disorders characterized by diffuse parenchymal involvement and impaired gas exchange. Among these, immune-mediated ILD constitutes a significant subgroup, often associated with autoimmune and inflammatory conditions, leading to progressive lung damage and substantial morbidity. Early diagnosis and accurate prognostication of pediatric ILD remain challenging due to overlapping clinical presentations, nonspecific radiological findings, and the lack of reliable biomarkers. Consequently, there is an increasing need to identify measurable biological indicators that can aid in both diagnosis and prediction of disease progression. Interleukin-6 (IL-6) is a pro-inflammatory cytokine that plays a central role in immune regulation and has been implicated in various inflammatory and autoimmune disorders. Elevated IL-6 levels have been associated with disease activity and severity in multiple pulmonary conditions. Similarly, matrix metalloproteinase-7 (MMP-7) is involved in extracellular matrix remodeling and has emerged as a promising biomarker in interstitial lung diseases, reflecting ongoing tissue injury and fibrosis. The combined assessment of IL-6 and MMP-7 may provide valuable insights into both inflammatory activity and fibrotic processes in immune-mediated ILD. This dual role suggests their potential utility not only as diagnostic markers but also as prognostic indicators for disease progression and clinical outcomes. This study aims to evaluate the diagnostic and prognostic utility of IL-6 and MMP-7 in children with immune-mediated interstitial lung disease, with the goal of improving early detection, risk stratification, and clinical management.
Interventions
DIAGNOSTIC_TESTIL-6 (interlukin 6)
Interleukin-6 (IL-6): measured using enzyme-linked immunosorbent assay (ELISA) -Matrix Metalloproteinase-7 (MM7): quantified using standardized ELISA kits
RADIATIONHigh-Resolution Computed Tomography (HRCT):
High-Resolution Computed Tomography (HRCT): Evaluation of: (ground-glass opacities, fibrosis, reticular patterns, bronchiectasis) Scoring system may be applied to quantify ILD severity
Pulmonary Function Tests (PFTs) Spirometry: (FVC, FEV1, FEV1/FVC ratio)
Sponsors
Sohag University
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
2 Years to 18 Years
Healthy volunteers
Yes
Inclusion criteria
* Children aged 2-18 years with immune-mediated interstitial lung disease, including: * Connective Tissue Disease-associated ILD (CTD-ILD), such as Juvenile Idiopathic Arthritis, according to ILAR Classification, 2019, Systemic Lupus Erythematosus, according to EULAR/ACR Classification, 2023, Juvenile Dermatomyositis, according to EULAR/ACR Classification for Juvenile Idiopathic Inflammatory Myopathies, 2017, Mixed Connective Tissue Disease, according to Kasukawa Criteria, 2019, and Systemic Sclerosis, according to ACR/EULAR Classification, 2013. * Pediatric vasculitis, according to EULAR/PRINTO/PReS Pediatric Vasculitis Classification Criteria, 2022 update. * Pediatric sarcoidosis, according to WASOG pediatric consensus, 2019 * Children with newly diagnosed or previously diagnosed cases in whom IL-6 and MMP- 7 biomarker levels can be obtained according to standardized procedures. * Children whose guardians have provided written informed consent, according to institutional ethical guidelines.
Exclusion criteria
* • ILD caused by infectious, environmental, drug-induced, or post-injury etiologies, rather than immune-mediated mechanisms, according to ERS/ATS chILD guidelines, 2025. * Patients with incomplete diagnostic documentation preventing confirmation according to recognized criteria, as per disease-specific guidelines mentioned above. * Children with other chronic lung diseases unrelated to immune-mediated pathology, such as congenital malformations or cystic fibrosis, according to pediatric pulmonology consensus, 2024. * Cases in which biomarker samples cannot be obtained or processed reliably, according to laboratory standards for IL-6 and MMP-7 measurement
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| diagnostic and prognostic utility of interleukin-6 (IL-6) and matrix metalloproteinase-7 (MMP-7) in children with immune-mediated interstitial lung disease (ILD) | 2 years | To evaluate the diagnostic and prognostic utility of interleukin-6 (IL-6) and matrix metalloproteinase-7 (MMP-7) in children with immune-mediated interstitial lung disease (ILD), including connective tissue diseases (CTD), vasculitis, and sarcoidosis. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| To compare IL-6 and MMP-7 levels among the subgroups | 2 years | To compare IL-6 and MMP-7 levels among the subgroups: CTD-associated ILD Vasculitis-associated ILD Sarcoidosis-associated ILD Healthy pediatric controls |
| To correlate IL-6 and MMP-7 levels with clinical severity, pulmonary function tests, and radiological extent of ILD. | 2 years | — |
| To assess the potential of IL-6 and MMP-7 as non-invasive biomarkers for early detection, disease monitoring, and prognosis in pediatric immune-mediated ILD. | 2 years | — |
Contacts
CONTACTlamiaa k morssi, assistant lecturer
Outcome results
None listed