Childrens Interstitial Lung Disease Clinical Overview
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This document provides an overview of Children's Interstitial Lung Disease (ChILD). It discusses that ChILD is a heterogeneous group of rare lung disorders that cause damage to the alveolar walls. The prevalence and specific entities of ChILD differ from adult interstitial lung disease. ChILD can be difficult to diagnose due to its diversity and is associated with significant morbidity and mortality. Over time, diagnostic tools and understanding of ChILD have improved, leading to identification of genetic causes and targeted treatments. However, more research is still needed.
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Childrens Interstitial Lung Disease Clinical Overview
- 1. Children’s Interstitial Lung Disease (ChILD): Clinical Overview Leland L. Fan, MD 2/7/15
- 2. Disclosures • I receive royalties for writing topics for UpToDate
- 3. Interstitial Lung Disease (ILD), also known as diffuse lung disease, includes large, heterogeneous group of rare pulmonary disorders that cause derangements of the alveolar wall and disordered gas exchange
- 4. Alveolar Wall • Epithelial lining cells • Closely apposed capillary network • Delicate fibroelastic matrix
- 5. • Prevalence 0.13* 67-81# • Dominant Form None UIP • Unique Entities – Growth Abnormalilties Yes No – NEHI Yes No – PIG Yes No – SP-B Mutations Yes No ChILD vs Adult ILD Children Adults *OJRD. 2009;4:26 #AJRCCM 1994;150:967
- 6. Idiopathic Pulmonary Fibrosis (IPF)/ Usual Interstitial Pneumonitis (UIP) • Temporal Heterogeneity • Fibroblastic foci Highly lethal disease of older adults
- 7. UIP Pattern in Non-IPF Patients • Immune-mediated Disorders • Hypersensitivity Pneumonitis • Inborn Errors of Surfactant Metabolism
- 8. Desquamative Interstitial Pneumonia (DIP) • Benign form of adult ILD • Linked to smoking • Very responsive to steroids
- 9. • Nothing known at the time • Pediatricians assumed these were pediatric examples of ILD types described in adults • Numerous cases reported as DIP, UIP, IPF, and CFA Early Cases of ChILD
- 10. Mortality DIP 39%* 5%+ UIP 4%# 50-80%+ Children Adults # Zapletel. Pediatr Pulmonol 1985;1:154 # Hewitt. Arch Dis Child 1977;52:22 # Chetty. Ann Allergy 1987;58:336 # Steinkamp. Acta Paediatr Scand 1990;79:823 *Stillwell. Chest 1980;77:165 + King. AJRCCM 2005;172:268
- 12. UIP in Children 1 AJRCCM 2007;176:1120 2 Histopathol 2013 EPUP 3 Pediatrics 2013;132:684 # Cases # UIP ChILDRN1 378 0 Brompton2 203 1 Vanderbilt3 64 0
- 13. What we now know • True IPF/UIP probably does not exist in children • Most cases of pediatric DIP are related to inborn errors of surfactant metabolism
- 14. ChILD can be very difficult to diagnose and treat, and is associated with significant morbidity and mortality.
- 15. ChildRN Classification • Disorders more prevalent in infancy • Disorders of the immunocompetent host • Disorders related to systemic disease • Disorders of the immunocompromised host • Disorders masquerading as ILD • Unclassified Paediatr Respir Rev 2011;12:230
- 16. ChildRN Classification • Disorders more prevalent in infants –Diffuse developmental disorders –Growth abnormality –PIG –NEHI –Inborn error of surfactant metabolism
- 18. Inborn Errors of Surfactant Metabolism (IESM) • Abnormal production – SP-B – SP-C – ABCA3 – TTF-1 (NKX2-1) • Abnormal catabolism – CSF2RA – CSF2RB
- 19. SP-B SP-C ABCA3 TTF-1 CSF2RA CSF2RB Pathology PAP, DIP Abnormal lamellar bodies on EM PAP, DIP, CPI, NSIP. UIP PAP, DIP, CPI, NSIP, UIP Abnormal lamellar bodies on EM Alveolar simplification, DIP PAP Lethality +++++ +-++ +++ +-++ + IESM
- 20. An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy Geoffrey Kurland, Robin R. Deterding, James S. Hagood, Lisa R. Young, Alan S. Brody, Robert G. Castile, Sharon Dell, Leland L. Fan, Aaron Hamvas, Bettina C. Hilman, Claire Langston, Lawrence M. Nogee, and Gregory J. Redding; on behalf of the American Thoracic Society Committee on Childhood Interstitial Lung Disease (chILD) and the chILD Research Network Am J Respir Crit Care Med 2013;188:376
- 21. 6% 26% 9% 3% 10% 12% 15% 3% 5% 11% Diffuse developmental Growth abnormality NEHI PIG Surfactant dysfunction Normal host Immunocompromised Systemic Vascular Unclassified Infants (0-2)
- 22. 6% 26% 9% 3% 10% 12% 15% 3% 5% 11% Diffuse developmental Growth abnormality NEHI PIG Surfactant dysfunction Normal host Immunocompromised Systemic Vascular Unclassified 40% 22% 16% 1% 0% 2% 0% 7% 9% 3% Diffuse developmental Growth abnormality NEHI PIG Surfactant dysfunction Normal host Immunocompromised Systemic Masqueraders Unclassified Infants (0-2) Older Children (2-18)
- 24. • Systemic Disease 48 (12.7%) –Immune-mediated 39 (10.3%) •JIA 5 (1.3%) –NSIP 2 –Capillaritis 1 –Multicompartment disease 1 –Granulomatous pneumonia, likely infection 1
- 25. a systematic approach is essential to the evaluation of ChILD. With such a huge differential diagnosis,
- 26. Diagnostic Tools Infant PFTs HRCT VATS BAL
- 27. • Hct 7 • ANA neg • ANCA neg • AGMBA neg • UA neg 3 YO Girl
- 29. Alveolar Hemorrhage Syndromes 2010-2013 44 Pediatric Cases 2222 1414 88
- 30. 1970 1990 2000 1980 BAL VATS HRCT Molecular Genetics PFTs Infant PFTs CVCT OLB Progress In ChILD Biomarkers 2010 Diagnostic Tools
- 31. 1970 1990 2000 DIP, UIP, CFA 1980 BAL VATS HRCTSP-B Molecular Genetics PFTs Infant PFTs PIG ABCA3 SP-C LIP CVCT OLB Progress In ChILD Biomarkers 2010Growth CSF2RA, CSF2RB DNA repair defects TTF1 Diagnostic Tools PC, NEHI IPH
- 32. 1970 1990 2000 DIP, UIP, CFA 1980 SP-B PIG ABCA3 PC, NEHI SP-C LIP 2010Growth CSF2RA, CSF2RB DNA repair defects TTF1 Progress In ChILD IPH
- 33. 1970 1990 2000 DIP, UIP, CFA 1980 SP-B PIG ABCA3 PC, NEHI SP-C LIP 2010Growth CSF2RA, CSF2RB DNA repair defects TTF1 pulse steroids corticosteroids, hydroxychloroquine, azathioprine, cyclophosphamide IVIG cyclosporine lung transplant biologic agents tacrolimus, MMF macrolides Progress In ChILD Treatment plasmapheresis IPH
- 34. 1970 1990 2000 DIP, UIP, CFA 1980 SP-B PIG ABCA3 SP-C LIP 2010Growth CSF2RA, CSF2RB DNA repair defects TTF1 Progress In ChILD Controlled Clinical Trials PC, NEHI IPH
- 35. 2020 ChILD The Future ? 2030 2010 Regenerative medicine Gene sequencing
- 36. We’ve come a long way, Baby! It’s IPF.It’s IPF.