Infants With Childhood Interstitial Lung Disease Need Expert Management

August 06, 2013

By Will Boggs, MD

NEW YORK (Reuters Health) Aug 06 - Childhood interstitial lung disease (chILD) in infancy differs from adult ILD and requires specialized evaluation and management, the American Thoracic Society says in a new guideline.

Only recently has chILD been defined with any clarity, and most of the novel diagnostic entities disproportionately affect infants, according to the guideline, published August 1 in the American Journal of Respiratory and Critical Care Medicine.

"Clinicians need to have knowledge of a potential diagnosis, know how to pursue the diagnosis, and then carry out the studies necessary to make a diagnosis," Dr. Geoffrey Kurland from Children's Hospital of Pittsburgh, Pennsylvania, told Reuters Health by email.�

"By bringing these newer entities to the attention of our colleagues, we hope to familiarize them with these diagnoses," he said.�"The paper gives a suggested methodology to pursue these diagnostic entities."

Dr. Kurland and colleagues on a multidisciplinary panel convened to develop evidence-based guidelines on the classification, diagnosis, and management of ILD in children, focusing on neonates and infants <2 years of age. They ultimately had to rely on observational evidence and clinical experience because there were no controlled clinical trials on which to base their advice.

According to the panel, once the common diseases that cause DLD are excluded, at least three of the following four criteria must be present to make the diagnosis of chILD syndrome: respiratory symptoms; respiratory signs; hypoxemia; and diffuse abnormalities on a chest radiograph or CT scan.

Diagnostic testing should then aim to determine the exact chILD diagnosis. This can include echocardiography, thin section CT scanning, infant pulmonary function testing, and flexible bronchoscopy with bronchoalveolar lavage, although half of these are only weak recommendations.

When these measures fail to identify a cause for chILD, surgical lung biopsy is indicated, the guideline says.

Pediatric lung transplantation may be required for severe, life-threatening chILD diseases, but other treatments lack good evidence. Certainly, supportive and preventive care is appropriate, and families should receive education and support from care providers.

More research is needed to determine the natural history and clinical phenotypes of specific chILD diagnoses and to identify optimal methods of clinical evaluation and management.

"We know a fair amount about the clinical appearance of some of these entities, know more about the molecular basis of a few of these entities, and have little understanding of the natural history, targeted treatment, or outcome of many of them!" Dr. Kurland said.�"I, for one, hope that this Statement sparks more interest in chILD syndrome entities; this will result ultimately in a much better understanding of these diseases.�We are already anticipating that the next ATS Statement on this topic will show these advances."

Coauthor Dr. Robin Deterding from Children's Hospital Colorado and University of Colorado in Aurora told Reuters Health by email, "I would just like to mention that these children and their families endure significant quality of life impacts as they struggle with these diseases that have high morbidity and mortality and major variance in expertise and evaluation strategies around the world."

"These guidelines provide a foundation for common language, more consistent care, and starting points for critically needed future studies. Families, clinicians, and researchers should benefit," he added.

Dr. Don Hayes from The Ohio State University College of Medicine in Columbus, who has studied interstitial lung disease but was not involved in the new work, called it a "comprehensive and critical review of our current knowledge."

"These various groups of diseases are so rare that a large pediatric referral hospital may only see a few cases per year; thus it's very difficult to perform large research studies to expand our knowledge of the pathophysiology of these diseases and develop new treatments," he told Reuters Health by email.

"Despite limitations in the area of research, an organized approach in the diagnosis and treatment of infants with interstitial lung disease is badly needed in order to approach these diseases in a systematic way to improve diagnostic testing and treatment while also improving our understanding of the pathophysiology," Dr. Hayes said. "It's been proven in other rare diseases that a larger and more unified method to diagnose and treat patients leads to advancement in their medical care."


Am J Resp Crit Care Med 2013;188:376-394.


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