Children's Interstitial Lung Disease (ChILD) Follow-up

  • Author: James S Hagood, MD; Chief Editor: Michael R Bye, MD   more...
 
Updated: Apr 2, 2012
 

Further Inpatient Care

  • Admit patients to the hospital for diagnostic workup and initial therapy, pulse courses of IV corticosteroids, management of superimposed infections, and management of any serious adverse drug reactions.
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Further Outpatient Care

  • A pediatric pulmonologist should regularly follow up patients with interstitial lung disease (ILD).
  • The patient's oxygen saturation, nutritional status, and incidence of adverse drug reactions should be monitored at each visit.
  • PFTs and imaging studies should be used to monitor disease progression and the patient's response to treatment.
  • Echocardiography should be repeated to assess for the development of pulmonary hypertension or cor pulmonale.
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Inpatient & Outpatient Medications

  • New therapies, particularly use of cytotoxic or immunosuppressive drugs, should be initiated in the hospital.
  • Long-term therapeutic agents are usually administered and monitored on an outpatient basis.
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Transfer

  • Transfer may be necessary for further diagnostic workup or lung transplantation.
  • Pretransplantation evaluation should be initiated before end-stage disease develops to allow sufficient time for evaluation and donor identification.
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Complications

  • Superinfection can be life threatening, particularly if the patient is receiving immunosuppressive medications. Immunosuppressive drugs can mask signs and symptoms of infection. Prevention and careful monitoring are crucial.
  • Drug toxicity causes much of the morbidity associated with ILD. Again, prevention and monitoring are the keys to management.
  • Hemoptysis may occur in some types of ILD and suggests vasculitis or venoocclusive disease as possible underlying causes.
  • Death is usually the result of respiratory failure or cor pulmonale and right heart failure.
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Prognosis

  • Mortality rates as high as 90% have been reported in children who develop ILD when younger than 1 year (predominantly DIP); other studies have reported much better survival with conservative management.
  • Fan and Kozinetz reviewed the outcomes of 99 children with ILD over 15 years.[5] Survival rates at 24, 48, and 60 months after the appearance of initial symptoms were 83%, 72%, and 64%, respectively. Patients with histopathologic DIP and pulmonary vascular disorders have a prognosis worse than this.
  • Familial IPF manifesting in the neonatal period is associated with a high mortality rate.
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Patient Education

  • Stress the importance of compliance with medication and nutritional regimens, rehabilitation, and regular follow-up visits.
  • Carefully instruct patients and parents about the need to report possible adverse effects of medications and to monitor for signs and symptoms of superinfection.
  • Counsel patients and caregivers of patients with hypersensitivity pneumonitis to avoid precipitating exposures.
  • Strongly advise smoking cessation and prevention, and inform patients and caregivers about specific support programs.
  • Encourage involvement in support groups for rare disorders such as the Children’s Interstitial Lung Disease (chILD) Foundation
  • Caregivers and patients should receive education and counseling appropriate for families of children with chronic respiratory diseases, including financial counseling and transplantation preparedness.
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Contributor Information and Disclosures
Author

James S Hagood, MD  Professor of Pediatrics and Chief, Division of Respiratory Medicine, Department of Pediatrics, University of California, San Diego, School of Medicine and Rady Children's Hospital of San Diego

James S Hagood, MD is a member of the following medical societies: American Thoracic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Gulnur Com, MD  Pediatric Pulmonologist, University of Arkansas for Medical Sciences Children's Hospital

Gulnur Com, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Cystic Fibrosis Foundation

Disclosure: Nothing to disclose.

David J Vaughan  MB, MRCPI, MB, BCh, Consultant Pediatrician, Department of Pediatrics, Our Lady of Lourdes Hospital, Ireland

David J Vaughan is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Daniel William Young, MD, FACR  Clinical Professor of Radiology, Clinical Professor of Pediatrics, University of Alabama School of Medicine; Active Staff, Department of Pediatric Imaging, Children's Hospital of Alabama; Vice-President, Pediatric Radiology Associates, PC

Daniel William Young, MD, FACR is a member of the following medical societies: Alpha Omega Alpha, American College of Radiology, Radiological Society of North America, and Society for Pediatric Radiology

Disclosure: Nothing to disclose.

Elizabeth C Mroczek-Musulman, MD  Clinical Associate Professor of Pathology, Associate Pathologist, Department of Pathology, University of Alabama Schools of Medicine and Dentistry, The Children's Hospital of Alabama

Elizabeth C Mroczek-Musulman, MD is a member of the following medical societies: American Society for Clinical Pathology and College of American Pathologists

Disclosure: Nothing to disclose.

Lisa R Young, MD  Associate Professor of Allergy, Immunology, and Pulmonary Medicine, Department of Pediatrics, and Allergy, Pulmonary, and Critical Care Medicine, Department of Medicine, Vanderbilt University School of Medicine

Lisa R Young, MD is a member of the following medical societies: American Thoracic Society, Central Society for Clinical Research, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Susanna A McColley, MD  Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Director of Cystic Fibrosis Center, Head, Division of Pulmonary Medicine, Children's Memorial Medical Center of Chicago

Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, and American Thoracic Society

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Honoraria Consulting; Boston Scientific Consulting fee Consulting; Gilead Honoraria Speaking and teaching; Caremark Consulting fee Consulting; Vertex Pharmaceuticals Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Heidi Connolly, MD  Associate Professor of Pediatrics and Psychiatry, University of Rochester School of Medicine and Dentistry; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center

Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Mary E Cataletto, MD  Director of Children's Sleep Services, Winthrop Sleep Disorders Center; Professor of Clinical Pediatrics, State University of New York at Stony Brook

Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians

Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD  Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

The authors are greatly indebted to the Rare Lung Disease Consortium, the members of the Children's Interstitial Lung Disease (chILD) collaborative, the chILD Foundation, and the children and families who struggle daily with interstitial lung disease (ILD).

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Interstitial lung disease (ILD) due to ABCA3 gene mutations. (A) High-resolution CT (HRCT) scan from a 4-month-old infant with ABCA3 mutations. The CT scan was performed with controlled ventilation under general anesthesia. Diffuse bilateral ground glass opacities and thickened interlobular septae are present. This "crazy paving" pattern suggests alveolar proteinosis or ILD due to genetic mutations affecting surfactant function and metabolism. (B) Histopathology (hematoxylin and eosin) shows diffuse alveolar septal thickening with uniform prominent type II cell hyperplasia. Accumulations of alveolar macrophages and granular proteinosis are also present in the alveolar spaces.(C) Electron microscopy demonstrates abnormal lamellar bodies with dense inclusions (arrows).
Pulmonary interstitial glycogenosis (PIG). (A) Lung histopathology from a 5-week-old infant shows diffuse interstitial widening and cellularity with bland-appearing vacuolated foamy cells that contain glycogen (periodic acid-Schiff [PAS] stain). These cells seen in PIG are strongly immunoreactive with vimentin (not shown). Pigmented alveolar macrophages were an additional finding in this infant with history of meconium aspiration.(B) Electron microscopy demonstrates that these mesenchymal cells contain abundant monoparticulate glycogen.
Neuroendocrine cell hyperplasia of infancy (NEHI) (A) Chest high-resolution CT (HRCT) scanning (at total lung capacity) in a 6-month-old infant with tachypnea, hypoxemia, and failure to thrive. Sharply defined areas of ground glass opacity are seen most prominent in the right middle lobe and lingual. Diffuse air-trapping was seen on expiratory images (not shown). No additional abnormalities were identified.(B) Hematoxylin and eosin staining of the lung biopsy reveals near-normal lung architecture. (C) Bombesin immunostaining reveals increased numbers of neuroendocrine cells.
Follicular bronchiolitis (A) Chest high-resolution CT (HRCT) scan from a 6-year-old infant with common variable immunodeficiency with history of anemia, thrombocytopenia, recurrent pneumonia, chronic cough, and exercise intolerance. Mosaic attenuation is present diffusely throughout the lungs. Extensive hilar and mediastinal lymphadenopathy is also present. Air-trapping was seen on expiratory images (not shown). (B) Lung histopathology demonstrates severe airway-centric lymphocytic inflammation with reactive follicles, which infiltrates and obscures most bronchioles.
Bronchiolitis obliterans. (A) Chest CT scanning from an 8-year-old demonstrates irregular large mosaic regions of ground-glass opacity and air-trapping, as well as the presence of peribronchial thickening and bronchiectasis. (B) Pathology demonstrates focal areas of fibrosis with polypoid plugs of fibroblastic cells and fibrin filling distal bronchioles and airspaces (hematoxylin and eosin).
Nonspecific interstitial pneumonitis. (A) Chest high-resolution CT (HRCT) scanning from a 10-year-old with systemic sclerosis and progressive exercise intolerance. (B) Lung biopsy showed multiple abnormalities including a relatively diffuse interstitial process with mild chronic inflammation, abundant fibroblastic tissue and patchy dense interstitial fibrosis. Accumulation of alveolar macrophages is seen in the airspaces, with rare foci of organizing pneumonia. Pulmonary arteries demonstrated focal intimal hyperplasia and medial hypertrophy, and the pleura contains patchy chronic inflammation. This overall constellation of findings is generally classified as mixed cellular and fibrotic nonspecific interstitial pneumonia (NSIP) and is a pattern most commonly seen in the setting of underlying collagen vascular disease.
 
 
 
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