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Interstitial lung disease in children: a multicentre survey on diagnostic approach
A Barbato1, C Panizzolo, A Cracco
1Dept of Paediatrics, University of Padua, Italy.
Insights
Diagnosing pediatric interstitial lung disease (ILD) often requires invasive methods. Bronchoalveolar lavage (BAL) was the most common invasive technique used in children with ILD.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Rare Diseases
Background:
- Chronic interstitial lung disease (ILD) is uncommon in children and has a poor prognosis.
- The diagnostic pathway for pediatric ILD involves various invasive and non-invasive methods.
- Understanding current diagnostic practices is crucial for improving patient outcomes.
Purpose of the Study:
- To investigate the diagnostic methods most frequently employed for interstitial lung disease (ILD) in pediatric patients.
- To assess the utilization of both non-invasive and invasive techniques in the diagnosis of pediatric ILD.
- To identify the most common invasive procedures used in the diagnosis of ILD in children.
Main Methods:
- A survey questionnaire was distributed to 187 centers within the European Respiratory Society Paediatric Assembly.
- The questionnaire gathered data on the use of non-invasive methods (history, physical exam, labs, PFTs, radiology) and invasive techniques (BAL, TBB, OLB, VAT, FNAB).
- Responses were received from 38 centers, detailing the diagnostic approaches for 131 children with ILD.
Main Results:
- Non-invasive techniques alone led to a diagnosis in only 3.8% of patients.
- Histological assessment through biopsy was performed in 74.8% of children.
- Bronchoalveolar lavage (BAL) was the most frequently used invasive technique (63.3%), followed by open lung biopsy (OLB) (48.8%).
Conclusions:
- A definitive diagnosis of ILD, based on etiological and/or histological findings, was achieved in 89% of the studied pediatric patients.
- Invasive diagnostic procedures, particularly BAL, are essential for diagnosing ILD in children.
- The study highlights the reliance on invasive methods for confirming pediatric ILD diagnoses.
Abstract:
Chronic interstitial lung disease (ILD) is a rare disorder in the paediatric age group, with a poor prognosis. The diagnostic approach to ILD is based on more or less invasive methods. This study was implemented to verify which methods are the most often used in children. Questionnaires (333) were sent to members of the European Respiratory Society Paediatric Assembly belonging to 187 European and non-European centres. Questions concerned the use of noninvasive diagnostic methods, e.g. history taking, physical examination, routine laboratory tests, respiratory function tests and radiology (chest radiography, high-resolution computed tomography (HRCT)), and the use of invasive techniques such as bronchoalveolar lavage (BAL), transbronchial biopsy (TBB), open lung biopsy (OLB), video-assisted thoracoscopic biopsy (VAT) and HRCT with fine-needle aspiration biopsy (FNAB). Thirty eight centres returned the questionnaires and 131 children with ILD were studied. A diagnosis of ILD was achieved in five (3.8%) patients using noninvasive techniques alone. Using the various biopsy methods, histological assessment was performed on a total of 98 (74.8%) children. The most frequently used invasive technique both alone and in combination was BAL (83, 63.3%), followed by OLB (64, 48.8%), TBB (26, 19.8%) and VAT (11, 8.4%); FNAB was used in one patient. In conclusion a diagnosis of interstitial lung disease was reached on the basis of aetiological and/or histological findings in 117 (89%) of the 131 patients studied.