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Interstitial lung disease
Interstitial lung disease
What is interstitial lung disease in children?
Diffuse interstitial lung diseases (DILDs) in children comprise a group of rare lung diseases that affect the deep lung tissue (called the interstitium).
They are characterised by inflammation and sometimes fibrosis, which may impair oxygen exchange.
In children, these diseases have specific features compared with adults. They are often associated with:
• genetic abnormalities (particularly those involving pulmonary surfactant);
• disorders of lung development;
• infectious, environmental or autoimmune causes.
Symptoms may appear in early childhood and vary according to severity:
• persistent respiratory distress;
• chronic cough;
• reduced blood oxygen levels (hypoxaemia);
• shortness of breath during exertion or feeding in infants;
• significant fatigue;
• growth retardation;
• sometimes digital clubbing (enlargement of the fingertips), a sign of chronic disease.
Diagnosis is based on a specialist, multidisciplinary assessment, including:
• a detailed review of the medical and family history;
• a thorough clinical examination;
• lung imaging (high-resolution chest CT scan);
• pulmonary function tests;
• genetic analyses;
• sometimes bronchoscopy or a lung biopsy.
Early diagnosis is essential to adapt management and limit disease progression.
Care at the Children’s Hospital
At the Children’s Hospital, the management of interstitial lung diseases is based on a specialised, individualised and multidisciplinary approach, involving in particular the paediatric pulmonology, genetics, nutrition and rehabilitation teams.
Comprehensive and personalised care
Each child receives follow-up tailored to their clinical profile, including:
• regular paediatric pulmonology consultations;
• respiratory function assessment;
• monitoring of growth and nutritional status;
• coordination with other specialists as required.
Treatments offered
General measures
• Oxygen therapy in cases of hypoxaemia, to improve comfort and limit complications;
• Nutritional support to promote growth;
• Respiratory physiotherapy where necessary;
• Vaccinations (influenza, pneumococcus, RSV) to prevent infections;
• Therapeutic education to help families manage the disease on a daily basis.
Drug treatments
Depending on the cause and progression:
• Corticosteroids (first-line treatment for certain inflammatory forms);
• Immunosuppressants (azathioprine, mycophenolate) to limit inflammation;
• Antifibrotic agents (currently under study in children) to slow progression;
• Antibiotic therapy in certain cases to prevent infections.
Lung transplantation
In the most severe forms, lung transplantation may be considered.
Early assessment at a specialised centre makes it possible to anticipate this option when necessary.
Follow-up and research
Regular follow-up is essential in order to:
• adjust treatments;
• monitor disease progression;
• prevent complications.
H.U.B. actively participates in international research, notably through the chILD-EU European registry, and in clinical trials aimed at developing new therapies adapted to children.
Advice for parents
Monitor symptoms on a daily basis
• Be alert to the onset or worsening of shortness of breath.
• Monitor fatigue, eating and weight gain.
• Note any changes in the cough or breathing pattern.
Facilitate your child’s breathing
• Avoid exposure to tobacco smoke and pollutants.
• Air out the home regularly.
• Follow the prescribed treatments, including oxygen therapy if necessary.
Prevent infections
• Follow the recommended vaccination schedule.
• Limit contact with people who are ill during high-risk periods.
• Encourage good hand hygiene.
Support growth and energy
• Ensure an appropriate and sufficient diet.
• Offer smaller, more frequent meals if your child tires easily.
• Ask the team for advice if there are feeding difficulties.
Support your child on a daily basis
• Adapt activities to your child’s respiratory capacity.
• Encourage gentle, regular physical activity if possible.
• Take your child’s rhythm and need for rest into account.
When should you contact the team again?
• worsening shortness of breath;
• reduced appetite or weight loss;
• unusual fatigue;
• respiratory infection;
• difficulty following the treatments at home.
