Round Table Discussion: Children's Interstitial Lung Disease

Abstract

Robin R. Deterding: We have the opportunity today to talk about children’s interstitial lung disease (chILD) with experts in that fi eld. My hope is that we can have a lively discussion about what we know is important in chILD and what we see coming on the horizon. I would like to begin our discussion with a question: Why is it important to recognize that chILD differs from i nterstitial lung disease (ILD) in adults? Lisa, would you like to give your thoughts about this? Lisa R. Young: I think this is an extremely important question, about which we have learned a lot in the past sev-eral years. A major reason for the importance of distinguish-ing chILD from adult ILD has to do with what patients read and think about with respect to these 2 diagnoses. A family that turns to the Internet in the belief that it has a member affected by ILD can become very quickly frightened, for ex-ample, in reading about the prognosis in idiopathic pulmo-nary fi brosis (IPF), a common form of ILD in adults but one that does not occur in children. Robin R. Deterding: Alan, what are your thoughts as a radiologist? Alan S. Brody: I agree with Lisa, and I think that some things that look like but are not the same as adult IPF may be a problem for the radiologist as well as for the clinician who does not deal with chILD, as well as for the families of patients. The 2 conditions should not be treated in the same way; they do not have the same prognosis. We did not know that years ago, but today it is very clear, as Lisa observed, that IPF, the most common adult form of ILD, does not occur in children, and if someone gives a child that diagnosis, we can tell the family right away that it is not correct, that there is more work to do to understand their child’s ILD. The prognosis of chILD is very variable and often much better than adult IPF. Lisa R. Young: We’re also learning that the etiology of chILD is often very different from the types of ILD seen in adults, and we think that understanding the eti-ology of the childhood disorder is a key step toward its treatment. James S. Hagood: I agree. For adults and physicians car-ing for adults with ILD, by far the largest category of disease is IPF, which does not appear to occur in children. And while many secondary forms of ILD and pulmonary fi brosis may be similar to what we see in older children and adolescents with rheumatic disease or hypersensitivity pneumonitis in terms of specifi c causes, the prognosis and management may differ in children. Robin R. Deterding: All of these are excellent observa-tions. The only thing I would add is that ILD in infants and children may improve or transform into a different type of lung disease, that may be similar to or different than adult ILD, as they grow older. It is very diffi cult to know what may happen until we can correctly classify these various condi-tions and follow them over time. I would like to discuss the presentations of ILD in chil-dren. What are your experiences with this, and when might you think you are dealing with a child with ILD? Lisa R. Young: The children I see generally have 2 dif-ferent categories of ILD. One of them has a very dramatic presentation, such as respiratory failure, and this generally presents in neonates at birth. I fi nd it can be more diffi cult to identify ILD in older children, which may be insidious in onset. Children may present with progressive exercise intol-erance marked only by a gradual slowing and falling behind their friends or siblings in their physical activities. In babies, the symptoms of this may include trouble with feeding, and may look like refl ux or other feeding problems, but may in-stead be related to their diffi culty with breathing. Alan S. Brody: As a radiologist, most of the children sent to me are referred, and for a number of reasons, but there is almost always some concern about the presence of lung