Childhood diffuse parenchymal lung diseases: We need a new classification

Childhood diffuse parenchymal lung diseases (DPLD) are a heterogeneous group of respiratory disorders of both known and unknown causes that share common histological features. To date, there is not an exact consensus about the terminology, classification, therapy and follow up of this disease because of its rarity and wide clinical spectrum.

In this study, we tried to classify our DPLD patients according to the last classification scheme (chILD Network Classification).

The files of the children diagnosed with DPLD at our university hospital between 1974 and 2012 were retrospectively investigated. Clinical features, laboratory, radiological and histopathological findings, therapy and follow-up outcomes of these patients were recorded and evaluated according to the actual information and definitions.

We described 130 DPLD patients, the largest childhood DPLD series from a single center, classified in 16 distinct groups according to their diagnosis. Our largest group in this serie is pulmonary hemosiderosis (28.5%); idiopathic interstitial pneumonias, pulmonary hemosiderosis, sarcoidosis and lipid storage diseases with lung involvement represent the 70% of the diagnoses. When we classified our patients according to the chILD Network Classification; patients with idiopathic interstitial pneumonia older than 2 years, idiopathic pulmonary hemosiderosis, pulmonary alveolar microlithiasis and diffuse chondroid malformation of the lung stayed out of this classification.

To ensure a consensus about the therapy and follow up, we have to make revisions and reorganisations on the DPLD classification which was proposed in 2007. We need a new childhood DPLD classification that will cover all these disease groups.