Children's Interstitial Lung Disease (ChILD) Treatment & Management

  • Author: James S Hagood, MD; Chief Editor: Michael R Bye, MD   more...
 
Updated: Sep 11, 2009
 

Medical Care

The multiple possible diagnostic entities and lack of randomized clinical trials make offering specific recommendations regarding treatment of children’s interstitial lung disease (ChILD) impossible. If the process is secondary to an underlying condition, patients should be treated for the underlying disease.

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 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 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 should be avoided. Smoking cessation should be actively pursued for caregivers who smoke.
  • 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 medications (eg, steroids, cytotoxic agents, immunosuppressive therapies), collagen synthesis inhibitors, antifibrotic agents, hydroxychloroquine, intravenous immunoglobulin (IVIG), antioxidants, and cytokine inhibitors.
  • Hypersensitivity pneumonitis is the most treatable condition among chILDs. Fan et al (2004) reported 86 cases of pediatric hypersensitivity pneumonitis that had an excellent response to steroids.[34] Other steroid-responsive conditions include NSIP, LIP, COP, eosinophilic pneumonia syndromes, sarcoidosis, pulmonary hemosiderosis, and ILD associated with connective tissue disease.[6]
  • Treatment of specific conditions resulting in ILD includes antiviral agents against CMV and EBV, antiretroviral therapy in addition to prednisolone for AIDS-associated LIP, surgical approach for lymphangiomatosis, therapeutic BAL for PAP, and PPI and Nissen fundoplication for GER-associated chronic aspiration. Reports indicate that infliximab (an inhibitor of tumor necrosis factor [TNF]-alpha) may be beneficial for ILD associated with rheumatoid arthritis.[44] Several studies have demonstrated successful use of subcutaneous treatments with GM-CSF in adults with PAP.[6]
  • In patients with associated PAH, sildenafil and/or anticoagulant therapy should be considered.
  • In patients with congenital PAP due to GM-CSF receptor mutation or acquired receptor dysfunction secondary to autoantibody formation, subcutaneous or inhaled GM-CSF treatment has been reported to be beneficial.[45] ,[46]
Next

Surgical Care

  • Surgical consultation is usually sought for diagnostic biopsy (see Procedures).
  • Patients with end-stage idiopathic forms of ILD, severe lung disease associated with SFTPB or ABCA3 mutations, as well as 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.
  • In children, the establishment of lung transplantation has been slower than in adults. Only 5% of all patients receiving transplants for this reason have been younger than 18 years. For some diseases, such as SP-B and ABCA3 deficiencies and alveolar capillary dysplasia, lung transplantation remains the only effective treatment.
  • Huddleston et al (2002) reported a 77% overall survival rate for the first year after transplantation in children.45 The 3- and 5-year survival declined to 63% and 54%, respectively. The authors observed no statistical relationship between pretransplantation diagnoses and long-term survival. The same authors reported 19 infants younger than 6 months who underwent lung transplantation: Seven had SP-B deficiency, 4 had PAP of other etiology, 3 had congenital interstitial pneumonitis, 2 had alveolar-capillary dysplasia, and 10 had pulmonary vascular disease.
Previous
Next

Consultations

  • Pediatric pulmonologist: All children with ILD should be treated in consultation with a pediatric pulmonologist.
  • Pediatric ILD specialist: In addition, referral to or telephone consultation with a center with clinicians specializing in childhood ILD is advised.
  • Pediatric cardiologist: As a result of the existence of cardiovascular diseases masquerading as ILD, all patients should see a pediatric cardiologist.
  • Pediatric rheumatologist: A pediatric rheumatologist should be involved in the management of ILD associated with connective tissue disease.
  • Pediatric radiologist: Consult a pediatric radiologist regarding interpretation of imaging studies.
  • In addition, consider consultation with the following specialists:
    • Infectious disease specialist
    • Immunologist
    • Rheumatologist
    • Transplantation specialist
  • Pathologist: Consultation with a pathologist is recommended before tissue is obtained to ensure that adequate specimens are collected and that they are correctly processed. Consider consultation with a pathologist knowledgeable about ChILD.
Previous
Next

Diet

No specific diet is necessary. However, as with patients with any chronic disease, patients with ChILD should receive sufficient kilojoules to maintain adequate growth. 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.

Previous
Next

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.

Previous
Proceed to Medication
 
 
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

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, MBBCh  Consultant Pediatrician, Department of Pediatrics, Our Lady of Lourdes Hospital, Ireland

David J Vaughan, MBBCh 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  Assistant Professor, Pediatric Pulmonary Medicine and Pulmonary Critical Care and Sleep Medicine, University of Cincinnati; Director of Pediatric Rare Lung Diseases Program and Consulting Physician, Cincinnati Children's Hospital Medical Center; Consulting Physician, University Hospital, Cincinnati

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

Disclosure: Nothing to disclose.

Specialty Editor Board

Susanna A McColley, MD  Associate Professor, Department 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; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Heidi Connolly, MD  Associate Professor of Pediatrics and Psychiatry, University of Rochester; 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  Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital

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.

References

  1. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med. Apr 1998;157(4 Pt 1):1301-15. [Medline].

  2. Selman M, King TE, Pardo A, American Thoracic Society, European Respiratory Society and American College of Chest Physicians. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med. Jan 16 2001;134(2):136-51. [Medline].

  3. Thannickal VJ, Toews GB, White ES, Lynch JP 3rd, Martinez FJ. Mechanisms of pulmonary fibrosis. Annu Rev Med. 2004;55:395-417. [Medline].

  4. Fan LL, Kozinetz CA, Wojtczak HA, Chatfield BA, Cohen AH, Rothenberg SS. Diagnostic value of transbronchial, thoracoscopic, and open lung biopsy in immunocompetent children with chronic interstitial lung disease. J Pediatr. Oct 1997;131(4):565-9. [Medline].

  5. Deutsch GH, Young LR, Deterding RR, et al. Diffuse lung disease in young children: application of a novel classification scheme. Am J Respir Crit Care Med. Dec 1 2007;176(11):1120-8. [Medline].

  6. Dinwiddie R, Sharief N, Crawford O. Idiopathic interstitial pneumonitis in children: a national survey in the United Kingdom and Ireland. Pediatr Pulmonol. Jul 2002;34(1):23-9. [Medline].

  7. Doan ML, Guillerman RP, Dishop MK, et al. Clinical, radiological and pathological features of ABCA3 mutations in children. Thorax. Apr 2008;63(4):366-73. [Medline].

  8. Fan LL, Mullen AL, Brugman SM, Inscore SC, Parks DP, White CW. Clinical spectrum of chronic interstitial lung disease in children. J Pediatr. Dec 1992;121(6):867-72. [Medline].

  9. Young LR, Nogee LM, Barnett B, Panos RJ, Colby TV, Deutsch GH. Usual interstitial pneumonia in an adolescent with ABCA3 mutations. Chest. Jul 2008;134(1):192-5. [Medline].

  10. de Blic J. Pulmonary alveolar proteinosis in children. Paediatr Respir Rev. Dec 2004;5(4):316-22. [Medline].

  11. Sano H, Kuroki Y. The lung collectins, SP-A and SP-D, modulate pulmonary innate immunity. Mol Immunol. Feb 2005;42(3):279-87. [Medline].

  12. Yusen RD, Cohen AH, Hamvas A. Normal lung function in subjects heterozygous for surfactant protein-B deficiency. Am J Respir Crit Care Med. Feb 1999;159(2):411-4. [Medline].

  13. Nogee LM, Dunbar AE 3rd, Wert SE, Askin F, Hamvas A, Whitsett JA. A mutation in the surfactant protein C gene associated with familial interstitial lung disease. N Engl J Med. Feb 22 2001;344(8):573-9. [Medline].

  14. Chibbar R, Shih F, Baga M, et al. Nonspecific interstitial pneumonia and usual interstitial pneumonia with mutation in surfactant protein C in familial pulmonary fibrosis. Mod Pathol. Aug 2004;17(8):973-80. [Medline].

  15. Shulenin S, Nogee LM, Annilo T, Wert SE, Whitsett JA, Dean M. ABCA3 gene mutations in newborns with fatal surfactant deficiency. N Engl J Med. 2004;350(13) Mar 25:1296-303. [Medline].

  16. Prestridge A, Wooldridge J, Deutsch G, et al. Persistent tachypnea and hypoxia in a 3-month-old term infant. J Pediatr. Nov 2006;149(5):702-6. [Medline].

  17. Hartl D, Griese M. Interstitial lung disease in children -- genetic background and associated phenotypes. Respir Res. 2005;6(1):32. [Medline]. [Full Text].

  18. Bullard JE, Wert SE, Whitsett JA, Dean M, Nogee LM. ABCA3 mutations associated with pediatric interstitial lung disease. Am J Respir Crit Care Med. Oct 15 2005;172(8):1026-31. [Medline].

  19. Bullard JE, Wert SE, Nogee LM. ABCA3 deficiency: neonatal respiratory failure and interstitial lung disease. Semin Perinatol. Dec 2006;30(6):327-34. [Medline].

  20. Kobayashi I, Ono S, Kawamura N, et al. KL-6 is a potential marker for interstitial lung disease associated with juvenile dermatomyositis. J Pediatr. Feb 2001;138(2):274-6. [Medline].

  21. Al-Salmi QA, Walter JN, Colasurdo GN, et al. Serum KL-6 and surfactant proteins A and D in pediatric interstitial lung disease. Chest. Jan 2005;127(1):403-7. [Medline].

  22. Satoh H, Kurishima K, Ishikawa H, Ohtsuka M. Increased levels of KL-6 and subsequent mortality in patients with interstitial lung diseases. J Intern Med. Nov 2006;260(5):429-34. [Medline].

  23. Owens C. Radiology of diffuse interstitial pulmonary disease in children. Eur Radiol. Mar 2004;14 Suppl 4:L2-12. [Medline].

  24. Copley SJ, Coren M, Nicholson AG, Rubens MB, Bush A, Hansell DM. Diagnostic accuracy of thin-section CT and chest radiography of pediatric interstitial lung disease. AJR Am J Roentgenol. Feb 2000;174(2):549-54. [Medline].

  25. Brody AS. Imaging considerations: interstitial lung disease in children. Radiol Clin North Am. Mar 2005;43(2):391-403. [Medline].

  26. Long FR, Castile RG. Technique and clinical applications of full-inflation and end-exhalation controlled-ventilation chest CT in infants and young children. Pediatr Radiol. 2001;31(6):413-22. [Medline].

  27. Lynch DA, Hay T, Newell JD Jr, Divgi VD, Fan LL. Pediatric diffuse lung disease: diagnosis and classification using high-resolution CT. AJR Am J Roentgenol. Sep 1999;173(3):713-8. [Medline].

  28. Brody AS, Crotty EJ. Neuroendocrine cell hyperplasia of infancy (NEHI) [clinical image]. Pediatr Radiol. Dec 2006;36(12):1328. [Medline].

  29. Jensen SP, Lynch DA, Brown KK, Wenzel SE, Newell JD. High-resolution CT features of severe asthma and bronchiolitis obliterans. Clin Radiol. Dec 2002;57(12):1078-85. [Medline].

  30. Meyer KC. The role of bronchoalveolar lavage in interstitial lung disease. Clin Chest Med. Dec 2004;25(4):637-49, v. [Medline].

  31. Langston C, Patterson K, Dishop MK, Askin F, Baker P. A protocol for the handling of tissue obtained by operative lung biopsy: recommendations of the chILD pathology co-operative group. Pediatr Dev Pathol. May-Jun 2006;9(3):173-80. [Medline].

  32. American Thoracic Society, European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. Feb 2000;161(2 Pt 1):646-64. [Medline]. [Full Text].

  33. Deterding RR, Pye C, Fan LL, Langston C. Persistent tachypnea of infancy is associated with neuroendocrine cell hyperplasia. Pediatr Pulmonol. Aug 2005;40(2):157-65. [Medline].

  34. Fan LL, Deterding RR, Langston C. Pediatric interstitial lung disease revisited. Pediatr Pulmonol. Nov 2004;38(5):369-78. [Medline].

  35. Kinane BT, Mansell AL, Zwerdling RG, Lapey A, Shannon DC. Follicular bronchitis in the pediatric population. Chest. Oct 1993;104(4):1183-6. [Medline].

  36. Hull J, Chow CW, Robertson CF. Chronic idiopathic bronchiolitis of infancy. Arch Dis Child. Dec 1997;77(6):512-5. [Medline].

  37. Canakis AM, Cutz E, Manson D, O'Brodovich H. Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease. Am J Respir Crit Care Med. Jun 1 2002;165(11):1557-65. [Medline].

  38. Fan LL, Langston C. Pediatric interstitial lung disease: children are not small adults [editorial]. Am J Respir Crit Care Med. 2002;165(11):1466-7. [Medline]. [Full Text].

  39. Mallory GB Jr. Surfactant proteins: role in lung physiology and disease in early life. Paediatr Respir Rev. Jun 2001;2(2):151-8. [Medline].

  40. Garcia CK, Raghu G. Inherited interstitial lung disease. Clin Chest Med. Sep 2004;25(3):421-33, v. [Medline].

  41. Grutters JC, du Bois RM. Genetics of fibrosing lung diseases. Eur Respir J. May 2005;25(5):915-27. [Medline]. [Full Text].

  42. Fan LL, Kozinetz CA, Deterding RR, Brugman SM. Evaluation of a diagnostic approach to pediatric interstitial lung disease. Pediatrics. Jan 1998;101(1 Pt 1):82-5. [Medline].

  43. Hamman L, Rich AR. Acute diffuse interstitial fibrosis of the lungs. Bull Johns Hopkins Hosp. 1944;74:177-212.

  44. Vassallo R, Thomas CF. Advances in the treatment of rheumatic interstitial lung disease. Curr Opin Rheumatol. May 2004;16(3):186-91. [Medline].

  45. Wylam ME, Ten R, Prakash UB, Nadrous HF, Clawson ML, Anderson PM. Aerosol granulocyte-macrophage colony-stimulating factor for pulmonary alveolar proteinosis. Eur Respir J. Mar 2006;27(3):585-93. [Medline].

  46. Venkateshiah SB, Yan TD, Bonfield TL, et al. An open-label trial of granulocyte macrophage colony stimulating factor therapy for moderate symptomatic pulmonary alveolar proteinosis. Chest. Jul 2006;130(1):227-37. [Medline].

  47. Rosen DM, Waltz DA. Hydroxychloroquine and surfactant protein C deficiency. N Engl J Med. Jan 13 2005;352(2):207-8. [Medline].

  48. Awasthi S, Coalson JJ, Yoder BA, Crouch E, King RJ. Deficiencies in lung surfactant proteins A and D are associated with lung infection in very premature neonatal baboons. Am J Respir Crit Care Med. Feb 2001;163(2):389-97. [Medline].

  49. Balasubramanyan N, Murphy A, O'Sullivan J, O'Connell EJ. Familial interstitial lung disease in children: response to chloroquine treatment in one sibling with desquamative interstitial pneumonitis. Pediatr Pulmonol. Jan 1997;23(1):55-61. [Medline].

  50. Bokulic RE, Hilman BC. Interstitial lung disease in children. Pediatr Clin North Am. Jun 1994;41(3):543-67. [Medline].

  51. Coren ME, Nicholson AG, Goldstraw P, Rosenthal M, Bush A. Open lung biopsy for diffuse interstitial lung disease in children. Eur Respir J. Oct 1999;14(4):817-21. [Medline].

  52. Crouch E. Pathobiology of pulmonary fibrosis. Am J Physiol. Oct 1990;259(4 Pt 1):L159-84. [Medline].

  53. Desmarquest P, Tamalet A, Fauroux B, et al. Chronic interstitial lung disease in children: response to high-dose intravenous methylprednisolone pulses. Pediatr Pulmonol. Nov 1998;26(5):332-8. [Medline].

  54. Dinwiddie R. Treatment of interstitial lung disease in children. Paediatr Respir Rev. Jun 2004;5(2):108-15. [Medline].

  55. Du Bois RM. Interferon gamma-1b for the treatment of idiopathic pulmonary fibrosis. N Engl J Med. Oct 21 1999;341(17):1302-4. [Medline].

  56. Fan LL, Kozinetz CA. Factors influencing survival in children with chronic interstitial lung disease. Am J Respir Crit Care Med. Sep 1997;156(3 Pt 1):939-42. [Medline].

  57. Fan LL, Langston C. Chronic interstitial lung disease in children. Pediatr Pulmonol. Sep 1993;16(3):184-96. [Medline].

  58. Fan LL, Lung MC, Wagener JS. The diagnostic value of bronchoalveolar lavage in immunocompetent children with chronic diffuse pulmonary infiltrates. Pediatr Pulmonol. Jan 1997;23(1):8-13. [Medline].

  59. Hacking D, Smyth R, Shaw N, Kokia G, Carty H, Heaf D. Idiopathic pulmonary fibrosis in infants: good prognosis with conservative management. Arch Dis Child. Aug 2000;83(2):152-7. [Medline].

  60. Hilman BC. Diagnosis and treatment of ILD. Pediatr Pulmonol. Jan 1997;23(1):1-7. [Medline].

  61. Hilman BC, Amaro-Galvez R. Diagnosis of interstitial lung disease in children. Paediatr Respir Rev. Jun 2004;5(2):101-7. [Medline].

  62. Huddleston CB, Bloch JB, Sweet SC, de la Morena M, Patterson GA, Mendeloff EN. Lung transplantation in children. Ann Surg. Sep 2002;236(3):270-6. [Medline].

  63. Huddleston CB, Sweet SC, Mallory GB, Hamvas A, Mendeloff EN. Lung transplantation in very young infants. J Thorac Cardiovasc Surg. Nov 1999;118(5):796-804. [Medline].

  64. Kerem E, Bentur L, England S, et al. Sequential pulmonary function measurements during treatment of infantile chronic interstitial pneumonitis. J Pediatr. Jan 1990;116(1):61-7. [Medline].

  65. Kurland G, Michelson P. Bronchiolitis obliterans in children. Pediatr Pulmonol. Mar 2005;39(3):193-208. [Medline].

  66. Leslie KO. Pathology of interstitial lung disease. Clin Chest Med. Dec 2004;25(4):657-703, vi. [Medline].

  67. Liebow AA. Definition and classification of interstitial pneumonias in human pathology. Prog Respir Res. 1975;8:1-33.

  68. Nogee LM, de Mello DE, Dehner LP, Colten HR. Brief report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis. N Engl J Med. Feb 11 1993;328(6):406-10. [Medline].

  69. Osika E, Muller MH, Boccon-Gibod L, et al. Idiopathic pulmonary fibrosis in infants. Pediatr Pulmonol. Jan 1997;23(1):49-54. [Medline].

  70. Puthothu B, Krueger M, Heinze J, Forster J, Heinzmann A. Haplotypes of surfactant protein C are associated with common paediatric lung diseases. Pediatr Allergy Immunol. Dec 2006;17(8):572-7. [Medline].

  71. Raghu G, Brown KK, Bradford WZ, et al. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med. Jan 8 2004;350(2):125-33. [Medline].

  72. Selman M, Lin HM, Montano M, et al. Surfactant protein A and B genetic variants predispose to idiopathic pulmonary fibrosis. Hum Genet. Nov 2003;113(6):542-50. [Medline].

  73. Sharief N, Crawford OF, Dinwiddie R. Fibrosing alveolitis and desquamative interstitial pneumonitis. Pediatr Pulmonol. Jun 1994;17(6):359-65. [Medline].

  74. Sondheimer HM, Lung MC, Brugman SM, Ikle DN, Fan LL, White CW. Pulmonary vascular disorders masquerading as interstitial lung disease. Pediatr Pulmonol. Nov 1995;20(5):284-8. [Medline].

  75. Stillwell PC, Norris DG, O'Connell EJ, Rosenow EC 3rd, Weiland LH, Harrison EG Jr. Desquamative interstitial pneumonitis in children. Chest. Feb 1980;77(2):165-71. [Medline].

  76. Ziesche R, Hofbauer E, Wittmann K, Petkov V, Block LH. A preliminary study of long-term treatment with interferon gamma-1b and low-dose prednisolone in patients with idiopathic pulmonary fibrosis. N Engl J Med. Oct 21 1999;341(17):1264-9. [Medline].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.