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Orphanet Journal of Rare Diseases

, 5:22

First Online: 20 August 2010Received: 31 July 2009Accepted: 20 August 2010DOI: 10.1186-1750-1172-5-22

Cite this article as: Clement, A., Nathan, N., Epaud, R. et al. Orphanet J Rare Dis 2010 5: 22. doi:10.1186-1750-1172-5-22


Interstitial lung disease ILD in infants and children comprises a large spectrum of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. These disorders are characterized by inflammatory and fibrotic changes that affect alveolar walls. Typical features of ILD include dyspnea, diffuse infiltrates on chest radiographs, and abnormal pulmonary function tests with restrictive ventilatory defect and-or impaired gas exchange. Many pathological situations can impair gas exchange and, therefore, may contribute to progressive lung damage and ILD. Consequently, diagnosis approach needs to be structured with a clinical evaluation requiring a careful history paying attention to exposures and systemic diseases. Several classifications for ILD have been proposed but none is entirely satisfactory especially in children. The present article reviews current concepts of pathophysiological mechanisms, etiology and diagnostic approaches, as well as therapeutic strategies. The following diagnostic grouping is used to discuss the various causes of pediatric ILD: 1 exposure-related ILD; 2 systemic disease-associated ILD; 3 alveolar structure disorder-associated ILD; and 4 ILD specific to infancy. Therapeutic options include mainly anti-inflammatory, immunosuppressive, and-or anti-fibrotic drugs. The outcome is highly variable with a mortality rate around 15%. An overall favorable response to corticosteroid therapy is observed in around 50% of cases, often associated with sequelae such as limited exercise tolerance or the need for long-term oxygen therapy.

AbbreviationsARDSAcute respiratory distress syndrome

AECAlveolar epithelial cells

ATSAmercican Thoracic Society

ASAnkylosing spondylitis


anti-CCPAnticyclic citrullinated peptide

anti-GBMAnti-glomerular basement membrane

Jo1Anti-histidyl-t-RNA synthetase

ANCAAnti-neutrophil cytoplasmic antibody

ANAAntinuclear antibodies


SaO2Arterial oxygen saturation

ABCA3ATP-binding cassette, sub-family A, member 3

BiPBinding immunoglobulin protein

BALBronchoalveolar lavage

CASRCalcium-sensing receptor

CGDChronic granulomatous disease

COPDChronic obstructive pulmonary disease

CSSChurg-Strauss syndrome

CTDConnective tissue disorders



DIPDesquamative interstitial pneumonia

DADDiffuse alveolar damage

DAHDiffuse alveolar hemorrhage

DLCODiffusing capacity of the lung for carbon monoxide

EREndoplasmic reticulum


EMTEpithelial-mesenchymal transition

EBVEpstein-Barrr virus

ERSEuropean Respiratory Society

FRCFunctional residual capacity

SFTPBGene coding for SP-B

SFTPCGene coding for SP-C

GM-CSFGranulocyte-macrophage colony-stimulating factor

HSPHenoch-Schönlein purpura

HRCTHigh-resolution computed tomography

HIVHuman immunodeficiency virus

HPHypersensitivity pneumonitis


ILDInterstitial lung disease

KL-6Kerbs von Lungren 6

LIPLymphocytic interstitial pneumonia


MPAMicroscopic polyangiitis

MCTDMixed connective tissue disease

NCHINeuroendocrine cell hyperplasia of infancy

NSIPNon-specific interstitial pneumonia


PAPPulmonary alveolar proteinosis

PFTPulmonary function testing

PIGPulmonary interstitial glycogenosis

PNECPulmonary neuroendocrine cells

RVResidual volume

RSVRespiratory syncitial virus

RARheumatoid arthritis


SRPSignal recognition particle

SSSjögren syndrome

SmSmith antigen

SPSurfactant proteins

SLESystemic lupus erythematosus

SScSystemic sclerosis

TTF-1Thyroid transcription factor 1

TLCTotal lung capacity

TLCOTransfer factor of the lung for carbon monoxide

TGFTransforming Growth Factor

UIPUsual interstitial pneumonia

WGWegener-s granulomatosis

Electronic supplementary materialThe online version of this article doi:10.1186-1750-1172-5-22 contains supplementary material, which is available to authorized users.

Annick Clement, Nadia Nathan contributed equally to this work.

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Autor: Annick Clement - Nadia Nathan - Ralph Epaud - Brigitte Fauroux - Harriet Corvol


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