Childhood Interstitial Lung Disease (ChILD) Treatment & Management

Updated: Feb 21, 2020
  • Author: Rebekah J Nevel, MD, MSCI; Chief Editor: Girish D Sharma, MD, FCCP, FAAP  more...
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Treatment

Medical Care

The heterogeneity of disease etiology and the lack of randomized clinical trials make offering specific recommendations regarding treatment of childhood interstitial lung disease (chILD) impossible. If the process is secondary to an underlying condition, patients should be treated for the underlying disease.

General treatment recommendations

General recommendations include supportive care with nutritional, oxygen, and ventilator support as needed. Management of comorbid conditions, including atopic phenotypes, sleep apnea, dysphagia, and aspiration, is encouraged. More specific systemic disease treatments, such as immunomodulatory and immunosuppressive medications, are appropriate in patients with chILD related to vasculitis and connective tissue diseases. [70, 71]

The same principles that apply to all children with chronic pulmonary diseases apply to those with interstitial lung disease (ILD). These include meticulous attention to growth and nutrition, immunizations (including respiratory syncytial virus [RSV], influenza, and pneumococcal prophylaxis), and treatment of secondary infections.

Treatment with bronchodilators, inhaled steroids, or both may be appropriate if any component of airway reactivity is demonstrated on pulmonary function testing (PFT). However, this therapy has not been proven to modify the clinical course of most types of ILD.

Oxygen therapy, either continuously or during sleep, may be necessary to provide symptomatic relief and to decrease the risk or halt the progression of pulmonary hypertension and cor pulmonale related to alveolar hypoxia.

Active and passive smoking, as well as exposure to electronic cigarette vapor, should be avoided. Smoking cessation should be actively pursued for caregivers who smoke.

Avoiding contact with persons who have symptomatic respiratory tract infections, especially during periods of high respiratory viral activity, is prudent.

Many medications have been used to treat different forms of ILD. No therapeutic regimen has been subjected to the rigors of a randomized control trial in the pediatric population. Numerous broad treatment strategies have been attempted, including anti-inflammatory and immunomodulatory medications (eg, steroids, hydroxychloroquine, intravenous immunoglobulin [IVIG], cytotoxic agents, immunosuppressive therapies, and biologic agents).

Treatment of specific conditions

Hypersensitivity pneumonitis is the most treatable condition among chILDs, with removal of the offending agent as an initial and definitive step in therapy. Fan et al reported 86 cases of pediatric hypersensitivity pneumonitis that had an excellent response to steroids. [57] Other conditions that have demonstrated a response to steroids in selected cases include nonspecific interstitial pneumonia (NSIP), lymphocytic interstitial pneumonia (LIP), cryptogenic organizing pneumonitis (COP), eosinophilic pneumonia syndromes, sarcoidosis, pulmonary hemosiderosis, and ILD associated with connective tissue disease. [31]

Treatment of specific conditions resulting in ILD includes antiviral agents against cytomegalovirus (CMV) and Epstein-Barr virus (EBV), antiretroviral therapy in addition to prednisolone for AIDS-associated LIP, a surgical approach for lymphangiomatosis, therapeutic bronchoalveolar lavage (BAL) for pulmonary alveolar proteinosis (PAP), proton pump inhibitor (PPI) therapy and gastric fundoplication for gastroesophageal reflux (GER)-associated chronic aspiration, and non-oral feeding for primary aspiration.

In patients with associated pulmonary arterial hypertension (PAH), sildenafil and anticoagulant therapy (if PAH is related to chronic thromboembolic disease) should be considered.

In patients with congenital PAP due to granulocyte-macrophage colony-stimulating factor (GM-CSF) receptor mutation or acquired receptor dysfunction secondary to autoantibody formation, subcutaneous or inhaled GM-CSF treatment has been reported to be beneficial. [72, 73] Therapeutic whole lung lavage may be beneficial in autoimmune or GM-CSF–associated PAP, but its application in cases of alveolar proteinosis associated with an underlying ILD diagnosis has not been evaluated in clinical trials and may be of limited utility, except in rare cases. [74]

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Surgical Care

Surgical consultation is usually sought for diagnostic biopsy.

Patients with end-stage progressive forms of ILD, severe neonatal lung disease associated with SFTPB or ABCA3 mutations, and some pulmonary veno-occlusive diseases may be candidates for lung or heart/lung transplantation. These patients are considered on an individual basis at the few centers specializing in pediatric lung transplantation.

The 2016 International Society for Heart and Lung Transplantation (ISHLT) Thoracic Transplant Registry Report shows that worldwide more than 100 pediatric lung transplantations are performed annually, although the majority of lung transplant centers perform fewer than 5 transplantations each year. [75]  According to the 2018 ISHLT Thoracic Transplant Registry Report, the median post-transplantation survival is 7 years. [76]

Rama et al compared children with diffuse lung disease, cystic fibrosis, and pulmonary vascular disorders who were undergoing lung transplantation. Children with diffuse lung disease had higher pre-transplant morbidity (lower forced expiratory volume in 1 second [FEV1], pulmonary hypertension, and hypercapnia). They also required more invasive ventilation and intensive care compared with patients with cystic fibrosis. No difference was noted in survival or in the frequency of infections and lymphoproliferative disease after transplantation. [77]

For some diseases, such as neonatal surfactant protein B (SP-B) and alveolar capillary dysplasia, lung transplantation remains the only effective treatment. The proportion of ChILD- and other rare disease–related lung transplants at pediatric transplant centers is likely to increase as the life expectancy and prognosis of patients with cystic fibrosis continue to improve.

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Consultations

All children with ILD should be treated in consultation with a pediatric pulmonologist. In addition, referral to or telephone consultation with a center that has clinicians who specialize in chILD is advised.

Because cardiovascular diseases can masquerade as ILD, all patients should have an echocardiogram and may benefit from the involvement of a pediatric cardiologist. A pediatric rheumatologist should be involved in the management of ILD associated with connective tissue disease. For ILD associated with immunodeficiency and/or immune dysregulation, the involvement of a pediatric immunologist is advised.

Consult a pediatric radiologist regarding the interpretation of imaging studies. Consultation with a pathologist is recommended before tissue is obtained to ensure that adequate specimens are collected and that they are correctly processed. In many cases, consideration should be given to consultation with a pathologist who is knowledgeable about chILD diagnoses.

In addition, consider consultation with an infectious disease specialist and a transplantation specialist. The pretransplantation evaluation should be initiated before end-stage disease develops to allow sufficient time for assessment and donor identification.

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Diet

No specific diet is necessary in most cases. In ILD associated with gastrointestinal disorders, dietary management should be planned in consultation with a gastroenterologist and an experienced dietitian.

As for patients with any chronic disease, patients with chILD should receive sufficient kilojoules to maintain adequate growth. ChILDs are associated with an increased risk of failure to thrive. Decreased lung compliance increases the work of breathing and energy expenditure. Energy supplementation should be undertaken with consideration to the added difficulty in handling high carbohydrate loads with chronic lung disease.

Consult a nutritionist experienced in the management of chronic pulmonary conditions in children. Young infants with feeding difficulties resulting from dyspnea may require a transpyloric or gastrostomic feeding tube.

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Activity

Activity may be limited by the patient's degree of dyspnea. Oxygen saturation during exercise should be measured. A prescribed, monitored exercise program may be beneficial to prevent deconditioning in older children. Conditions that may exacerbate pulmonary symptoms (high levels of ozone or other environmental pollutants) should be avoided. Patients with hypersensitivity pneumonitis should be removed from exposure to the precipitating substances (eg, birds, organic dusts). Air travel or travel to high altitudes must be carefully planned in patients with arterial desaturation.

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