Childhood Interstitial Lung Disease (ChILD) Medication

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

Medication Summary

The primary treatment focus in childhood interstitial lung disease (chILD) remains supportive care, including supplemental oxygen and ventilatory support, nutrition optimization, appropriate immunizations, and treatment of pulmonary exacerbations. No clinical trials of medications for chILD have been conducted to date.

Corticosteroids have been the mainstay of therapy in most children and adults with interstitial lung disease (ILD), despite little conclusive evidence of their efficacy. The medications commonly used for pharmacotherapy in chILD and common adverse effects are reviewed below. [70]

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Glucocorticoids

Class Summary

These agents elicit anti-inflammatory properties and cause profound and varied metabolic effects. They modify the immune response of the body to diverse stimuli. Suppression of immune-mediated alveolitis and repair mechanisms may reduce the progression of fibrosis. Data from small studies suggest that pulse administration with intravenous (IV) corticosteroids may improve survival and lessen toxicity compared with prolonged courses of oral steroids. Adverse effects of chronic systemic steroid treatment include adrenal suppression, decreased bone density and growth, glaucoma, and immunosuppression. Regular screenings such as annual dual-energy x-ray absorptiometry (DEXA) scans, blood pressure medications, and ophthalmologic evaluations are recommended. [70]

Prednisone/Prednisolone (Deltasone, Meticorten, Orasone, Sterapred)

Most widely used agent, particularly for usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), and hypersensitivity pneumonitis. May decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear (PMN) leukocyte activity. Typical dosing is 1-2 mg/kg/day until disease symptoms are controlled, then is decreased to the lowest effective dose. Alternating days on therapy may reduce the risk of adverse effects.

IV Methylprednisolone (Solu-Medrol)

Decreases inflammation by suppressing migration of PMN leukocytes and reversing increased capillary permeability. Typically given in high-pulse doses (10-30 mg/kg/day) for 3 consecutive days monthly, then dosing frequency is reduced once the clinical course stabilizes.

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Immunomodulating and immunosuppressive agents

Class Summary

Immunomodulatory drugs may be used as second-line therapy if a response to corticosteroids has not occurred, if a steroid-sparing effect is desired, or as an adjunctive agent to steroids in severe or rapidly progressive disease. Immunomodulatory medications used in patients with chILD include azathioprine, methotrexate, cyclophosphamide, cyclosporine, and mycophenolate mofetil.

Additionally, the biologic treatments most commonly used in chILDs include granulocyte-macrophage colony-stimulating factor (GM-CSF) for pulmonary alveolar proteinosis (PAP) due to alveolar macrophage neutralizing autoantibodies; monoclonal antibodies, such as rituximab, for autoimmune-related lung diseases; intravenous immunoglobulin (IVIG) for several off-label indications, including immune-mediated alveolar hemorrhage syndromes; and tumor necrosis factor (TNF)-alpha inhibitors, interleukin-1 (IL-1) antagonists, and IL-6 antagonists for some immune-mediated disorders, including lung disease related to juvenile idiopathic arthritis. [31, 78, 70]

For the use of these and other immunomodulatory medications and biologic agents, especially in the setting of systemic disorders such as connective tissue diseases, and vasculitides and immune dysregulation, consultation with a rheumatologist or an immunologist is recommended.

Hydroxychloroquine (Plaquenil)

Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions. In addition to its anti-inflammatory properties, hydroxychloroquine is thought to inhibit intracellular processing of the precursor of surfactant protein C (SP-C), which may be the mechanism of action in SP-C deficiency. Hydroxychloroquine sulfate 200 mg equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate. Dose and duration not tested in controlled trials, but case reports describe children receiving 6-10 mg/kg/day for years. Adverse effects of hydroxychloroquine include possible cardiomyopathy and conduction disorders (including QT prolongation), photosensitivity, retinal and corneal toxicity, and liver function abnormalities. Thus, regular ophthalmologic evaluations and liver enzyme monitoring are recommended.

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Macrolides

Azithromycin (Zithromax, Zmax)

In other chronic lung diseases, azithromycin has been used regularly for anti-inflammatory and immunomodulatory effects. The typical dosing is 10 mg/kg up to 500 mg per dose 3 times per week. Adverse effects include risk of QT prolongation, pyloric stenosis (in early infancy), and gastrointestinal effects. Additionally, a study of azithromycin for prevention of bronchiolitis obliterans syndrome after bone marrow transplantation was terminated early because of the increased risk of hematologic relapses and worse airflow decline–free survival in the azithromycin group.

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