Interstitial lung disease in children: a multi-center survey on diagnostic approach

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.