eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Children's Interstitial Lung Disease (ChILD)

Author: James S Hagood, MD, Director, Pediatric Pulmonary Center, Associate Professor of Pediatrics, Cell Biology and Pathology, Department of Pediatrics, University of Alabama School of Medicine
Coauthor(s): Gulnur Com, MD, Fellow in Pediatric Pulmonology, Department of Pediatrics, Division of Pulmonary Medicine, University of Alabama School of Medicine; David J Vaughan, MBBCh, Consultant Pediatrician, Department of Pediatrics, Our Lady of Lourdes Hospital, Ireland; Daniel W Young, MD, Clinical Professor of Radiology, Clinical Asst Professor of Pediatrics, University of Alabama School of Medicine; Consulting Staff, Department of Radiology, The Children's Hospital of Alabama; President, Pediatric Radiology Associates, PC; 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
Contributor Information and Disclosures

Updated: Jan 16, 2008

Introduction

Background

Interstitial lung diseases (ILDs) in childhood are a diverse group of conditions that primarily involve the alveoli and perialveolar tissues and that lead to derangement of gas exchange, restrictive lung physiology, and diffuse infiltrates as seen on radiographs. Because ILDs usually involve the distal airspaces and the interstitium, the term diffuse infiltrative lung disease has been suggested. This nomenclature is more accurate than ILD, but children's interstitial lung disease (chILD) has become the preferred term. In 2004, the Rare Lung Disease Consortium, a network of clinical and research centers and patient support organizations, was formed to accelerate clinical research in rare lung diseases, including chILD.

As a result of the rarity of ILDs and the important differences between childhood ILD and ILDs that affect adults, a great deal of confusion regarding their nomenclature, classification, and management is observed. Idiopathic pulmonary fibrosis (IPF, also known as cryptogenic fibrosing alveolitis [CFA]), the most prominent adult ILD, mostly occurs after the fifth decade of life and is almost never seen in children. Unlike in adults, most ILDs in children are found to have an underlying cause. In addition, the clinical significance of the histologic classification differs between children and adults.

Usual interstitial pneumonitis (UIP), the pattern associated with IPF in adults, is rarely, if ever, described in children. Desquamative interstitial pneumonitis (DIP), which is associated with steroid responsiveness and a better prognosis in adults, has a poor prognosis in children, particularly in infants. Neuroendocrine cell hyperplasia in infancy (NEHI) and pulmonary interstitial glycogenesis (PIG) are histologic patterns unique to children.

Management of ILD in children also differs from that in adults. Correct diagnosis is critical, requiring a comprehensive search for possible underlying causes. Case reports describing unique presentations and anecdotal responses to various therapeutic interventions abound. Definitive management of ILDs, particularly those of unknown etiology, is unclear at present. The newly formed consortium of centers, perhaps in collaboration with centers worldwide, may facilitate use of standardized diagnostic criteria and develop a network for clinical trials.

Pathophysiology

Childhood ILD is not a disease but a group of disorders (see Causes). However, most ILDs share a common pathophysiologic feature, namely, structural remodeling of the distal airspaces, leading to impaired gas exchange. In general, this remodeling has been believed to be the sequela of persistent inflammation; however, more recently, the paradigm has shifted away from inflammation to one of tissue injury with aberrant wound healing resulting in collagenous fibrosis. Until recently, most research in this field has been based on adult histopathology and data from animal models.

Wound healing and fibrosis are complex pathophysiologic processes that involve numerous cell types and cellular processes, such as adhesion; migration; proliferation; apoptosis; and a vast array of soluble mediators, extracellular matrix (ECM) molecules, and signaling intermediates. Detailed discussion of the pathophysiology of lung fibrosis can be found in several excellent reviews.1,2,3 In chILD, these processes occur in an organ that is still developing, further complicating the pathophysiology.

Many types of ILD follow some type of injury to the distal airspaces, such as adenoviral infection or exposure to organic dust, resulting in damage to the epithelial or endothelial layers and the associated basement membrane. In an animal model of lung fibrosis using bleomycin, as well as in models of surfactant-protein (SP) abnormalities, apoptosis of the alveolar epithelium was demonstrated to be a key inciting event. Blood or plasma leaks into procoagulant-rich airspaces and clots, forming a provisional matrix that contains fibronectin, fibrin, and numerous mediators released from activated platelets and injured cells (the hyaline membranes of acute lung injury). This fibrinous exudate usually forms the base for subsequent repair and remodeling.

Fibroblasts, which are normally present in the attenuated interstitial spaces between alveoli and surrounding distal airways, are activated by exposure to plasma proteins and soluble mediators and migrate into the fibrinous wound matrix, where they proliferate and elaborate matrix molecules such as collagen. Fibroblasts also produce proteases, such as urokinase and collagenase (which degrade the matrix), and inhibitors of matrix degradation, such as tissue inhibitors of metalloproteinases (TIMPs).

Through production of cytokines and chemokines, such as interleukin (IL)-6, IL-8, and keratinocyte growth factor (KGF), fibroblasts signal inflammatory cells, endothelial cells, and type II pneumocytes, activating or modulating the other cellular events that follow lung injury. Recent data has indicated alternate origins of fibroblasts, such as circulating precursors known as fibrocytes, which hone in on injured tissues, and transdifferentiation of other cells, such as epithelial-mesenchymal transition (EMT).

Inflammation is present in most types of ILD, and many forms of ILD are triggered by inflammatory events, such as infection or hypersensitivity. Neutrophils and lymphocytes are prominent in bronchoalveolar lavage (BAL) samples in many types of ILD. In DIP, the airspaces are filled with cells that were once believed to be desquamated epithelium but which are, in fact, activated macrophages. The mediators released by inflammatory cells, particularly IL-1 and transforming growth factor (TGF)-beta, are potent activators of fibroblast-mediated remodeling. Almost every type of inflammatory cell, including eosinophils and mast cells, have been described in various types of ILD and can interact with fibroblasts and other parenchymal cells. Therefore, for some time, the degree or persistence of inflammation has been believed to dictate the degree of remodeling and fibrosis.

This inflammation hypothesis has recently been called into question for numerous reasons. Careful review of well-defined cases of UIP in adults has revealed little evidence of extensive inflammation. Numerous reports detail diseases with extensive airspace inflammation, such as hypersensitivity pneumonitis, that do not always progress to fibrosis. Animal models, particularly certain transgenic mice, have demonstrated that interstitial remodeling and fibrosis are possible without inflammation. Clinical markers of inflammation, such as BAL cellularity, scanning using gallium-67 citrate, and levels of circulating inflammatory mediators, have not been useful in determining disease activity or prognosis. Therefore, inflammation has a prominent, but not a central, role in lung remodeling and fibrosis.

A large number of other pathophysiologic events are increasingly recognized as having clinically significant effects on lung remodeling. Injury or stress in epithelial cells, resulting in stress signaling or programmed cell death, may be the initiating factor in many types of fibrosis, such as that associated with surfactant protein abnormalities or viral infection. Markers of angiogenesis have been prominent in several animal models of ILD and substantially affect outcomes. The ECM is a complex, biologically active structure that signals cells either by direct means or by means of its soluble breakdown products and that binds, sequesters, and presents growth factors and other mediators to cells. The ECM is altered in ILD, and alterations in the ECM may also have a causative role.

Resolution of fibrotic remodeling involves a complex series of orderly steps, including matrix breakdown and restructuring, reepithelialization, and apoptosis of fibroblasts and inflammatory cells. Proliferation of type II pneumocytes is seen in most types of ILD. The proliferation and migration of type II pneumocytes over the provisional wound matrix are believed to be crucial events in the resolution of fibrosis.

Fibrotic remodeling is responsible for most of the morbidity and mortality associated with ILD. Remodeling of distal airspaces results in hypoxemia. Persistent hypoxemia results in pulmonary hypertension and vascular remodeling, leading to cor pulmonale. The increased work of breathing associated with reduced compliance results in increased energy expenditure, which, combined with the effects of inflammatory mediators, can result in cachexia. Portions of the lung may be replaced by fibrotic septae between dilated airspaces, the so-called honeycomb changes of end-stage interstitial disease. Although the events described in the preceding paragraphs are necessary for repair of the injured lung, excessive activation or failure of resolution of any of these pathways can result in disabling fibrosis.

Frequency

United States

ILD is rare in children. Because of a lack of consensus on case definition (until recently), the broad differential diagnosis, and the lack of organized reporting systems (eg, a national database), determining the incidence or prevalence of ILDs is impossible. Cases tend to cluster in infancy, and 10-16% appear to be familial.

Most of the literature is composed of case reports and small series. One of the largest reported series is a combined retrospective and prospective study by Fan et al performed over a 15-year period at a leading referral center for ILD.4 The investigators reported 99 patients, in whom the case definition included respiratory symptoms lasting longer than one month, diffuse infiltrates depicted on chest radiography, and absence of known bronchopulmonary dysplasia (BPD), heart disease, malignancy, immunodeficiency, autoimmunity, cystic fibrosis (CF), aspiration, or acquired immunodeficiency syndrome (AIDS). A more recent retrospective study that attempted a relatively complete case ascertainment of children undergoing biopsy for ILD in 11 referral centers in the United States and Canada over a 5-year period (1999-2004) reported 187 cases in children younger than 2 years old.

International

A national survey of cases of chronic ILD in immunocompetent children aged 0-16 years in the United Kingdom and Ireland over a three year period (1995-1998) yielded an estimated prevalence of 3.6 per million.5

Mortality/Morbidity

The same factors that make estimating the incidence of ILD difficult make estimating its mortality rates difficult.

  • In the series of 99 patients discussed above, the probability of surviving 24, 48, or 60 months was 83%, 72%, and 64%, respectively.6 Mean survival interval from onset in this group of patients was 47 months. 
  • Factors associated with poor outcome included pulmonary hypertension at the time of diagnosis and a final diagnosis of DIP or pulmonary vascular disease.
  • Data from small series suggest that patients with DIP, particularly infants, have a poor prognosis. This finding is in marked contrast to observations in adult ILD, in whom DIP diagnosed histologically indicates steroid responsiveness and an improved prognosis. Certain diagnosed conditions, such as SP-B deficiency, do not respond to any interventions. Without lung transplantation, the prognosis is poor.

Race

No data are available in the pediatric literature concerning differences in racial incidence or prevalence.

Sex

There appears to be a slight male predominance (roughly 60:40) in reported cases of chILD. A male predominance is observed in IPF in adults.

Age

Most cases of chILD occur in infants, but presentation can occur throughout childhood and adolescence.

  • In one of the largest series, the mean age at onset was 43 months (range, 0-212 mo). The median age at onset was 8 months, but the median age at evaluation was 30 months. These data indicate that some clustering of ILD occurs in infancy, and that, as is seen in adults, the delay between the onset of symptoms and appropriate diagnostic evaluation is often lengthy.
  • The mean age of onset of IPF (UIP) is 57 years. This particular entity likely does not occur in children at all.
  • Recently identified pediatric ILD syndromes unique to infancy, including NEHI, PIG, and chronic pneumonitis of infancy, may present at or shortly after birth. Genetic abnormalities of SPs often cause severe symptoms during the newborn period (See Causes and Childhood ILD syndromes manifesting in infancy).

Clinical

History

Diagnosing interstitial lung disease (ILD) requires a high index of suspicion on the part of the physician. The delay between the onset of symptoms and ultimate diagnosis is often months to years. Respiratory symptoms can be subtle in infants and children, and primary care providers often treat ILD as asthma. A delay in referral can lead to clinically significant remodeling of the lung before diagnosis.

The clinical history varies substantially by age. The onset of disease is often insidious, with caregivers or patients unsure when the illness actually began. Occasionally, patients present with relatively few symptoms but with abnormal findings on chest radiographs or pulmonary function tests (PFTs).

  • Tachypnea and/or dyspnea
    • Tachypnea is present in most patients (75%), particularly in infants.
    • Younger infants manifest retractions, difficulty in feeding, and diaphoresis with feeding. Cyanosis may be evident during feeding or at rest.
    • Exercise intolerance is often noted in older children.
  • A cough that is described as dry and nonproductive is commonly present (75%) and can be the only symptom of ILD, even in the newborn.
  • Failure to thrive and weight loss are common symptoms that result from anorexia, difficulty in feeding, and increased energy expenditure from increased work of breathing. 
  • Hemoptysis may indicate the presence of a vasculitic process or a pulmonary hemorrhage syndrome.
  • Older children may report chest pain.
  • Fever may be present, suggesting infectious or inflammatory causes.
  • Wheezing occurs in 40% of patients, according to the history, and is present upon examination in as many as 20%.
  • A careful family history is critical because some forms of children’s interstitial lung disease (ChILD) may have a genetic basis, which may be associated with neonatal deaths, unexplained childhood respiratory disease, or ILD in adults (see Causes).

Physical

  • General physical findings
    • Growth retardation, signs of weight loss, and/or failure to thrive may be evident.
    • Hypoxemia on room air is common (87% of patients with saturation below 90% in one series).
    • Desaturation may occur during sleep, during feeding (infants), or with exercise (eg, 6-minute walk test in older children and adolescents).
  • Auscultation may reveal normal findings or dry crackles that sound like Velcro being pulled apart; these are present only in a subset of patients.
  • Signs consistent with pulmonary hypertension may be present. Examples include an active precordium, which signifies right ventricular hypertrophy and a loud pulmonary component to the second heart sound.
  • Cyanosis and clubbing are late manifestations of ILD.
  • Stigmata of collagen vascular diseases, vasculitides, and other systemic disorders should be carefully sought.
  • Deformity of the chest has been reported and may indicate lung hypoplasia, as well the effects of prolonged illness.

Causes

ILD in children can be classified into idiopathic disorders, those of known or suspected causes, and those associated with systemic diseases (see ILD associated with systemic diseases below). In the largest reported series in children, 19-27% of cases remain undetermined.6 Several important disorders present with chronic respiratory symptoms and findings of diffuse radiographic infiltrates and must be considered in the differential diagnosis.

Different strains of mice and rats can be sensitive or resistant to experimental models of ILD. This fact, as well as the occurrence of familial IPF in humans, suggests both genetic and environmental determinants for ILD.

A clinical classification of causes of childhood ILD is located below. The numbers in parentheses indicate percentage of final diagnoses in the largest reported series.6

Disorders with known causes

  • Infection (8-10%)
    • Viral infection (eg, adenoviral bronchiolitis obliterans[5%], cytomegaloviral [CMV] infection, infection with Epstein-Barr virus [EBV])
    • Bacterial infection (eg, pertussis or infection due to Legionella, Mycoplasma, Chlamydia, or Mycobacterium species) 
    • Fungal infection (eg, infection due to Histoplasma, Aspergillus, or Pneumocystis species) 
    • Parasitic infection (eg, visceral larva migrans)
  • Environmental conditions (13%)
    • Exposure to organic dusts (hypersensitivity pneumonitis [7-12%]) 
    • Exposure to inorganic particulates (eg, silica, asbestos, talc, zinc) 
    • Exposure to chemical fumes (eg, sulfuric acid, hydrochloric acid, methyl isocyanate)
    • Exposure to gases (eg, oxygen, chlorine, nitrogen dioxide [silo-filler disease], ammonia)
    • Exposure to radiation
  • Drugs
    • Use of antineoplastic agents (eg, cyclophosphamide, nitrosoureas, methotrexate [MTX], azathioprine, cytosine arabinoside, 6-mercaptopurine [6-MP], vinblastine, bleomycin, busulfan)
    • Use of other drugs or elements (eg, penicillamine, nitrofurantoin, gold)
  • Previous lung injury
    • Chronic aspiration pneumonitis (4-5%) 
    • Resolving acute respiratory distress syndrome (ARDS) 
    • BPD
  • Lymphoproliferative disorders (10%) 
  • Neoplasia (eg, lymphoma [1%], leukemia, Langerhans cell histiocytosis [LCH])
  • Metabolic disorders
    • Lysosomal storage disorders (eg, Gaucher disease, Niemann-Pick disease)
    • Degenerative disorders (eg, pulmonary microlithiasis [1%])
  • Immunodeficiency-associated ILD
Disorders with unknown causes
  • Undetermined (19-27%); also called nonspecific (but not nonspecific interstitial pneumonitis [NSIP]) cellular interstitial pneumonitis or chronic interstitial pneumonia
  • Pulmonary hemorrhage syndromes (idiopathic pulmonary hemosiderosis [5-8%])
  • DIP (4-8%)
  • Lymphocytic interstitial pneumonitis (LIP [6%]) (Known AIDS cases are excluded; LIP is often associated with HIV infection or AIDS but can be idiopathic.)
  • UIP (2-4%) (The accuracy of this diagnosis in children is highly questionable.)
  • Lymphangiomatosis (4%)
  • Nonadenoviral bronchiolitis obliterans (4%)
  • Sarcoidosis (2%)
  • Pulmonary alveolar proteinosis (PAP [2%]) (see below)  
  • Eosinophilic syndromes (2%) (chronic eosinophilic pneumonia, pulmonary infiltrates with eosinophilia)
  • Idiopathic bronchiolitis obliterans organizing pneumonia (BOOP), also called cryptogenic organizing pneumonia (COP) (This is primarily a disease of adults that presents subacutely in the fifth or sixth decades, although rare idiopathic cases are reported in children.) 
  • Bronchocentric granulomatosis (1%) 
  • Nonspecific interstitial pneumonia
  • Acute interstitial pneumonitis (AIP)
ILD associated with systemic diseases
  • Connective tissue diseases (2-4%) (juvenile rheumatoid arthritis [JRA], dermatomyositis/polymyositis, systemic sclerosis, systemic lupus erythematosus [SLE], ankylosing spondylitis, Sjögren syndrome, Behçet syndrome, mixed connective tissue disease)
  • Autoimmune diseases (antiglomerular basement membrane antibody disease) 
  • Pulmonary vasculitis (polyarteritis nodosa, Wegener granulomatosis, Churg-Strauss syndrome)
  • Liver disease (chronic active hepatitis, primary biliary cirrhosis)
  • Bowel disease (2%) (eg, ulcerative colitis, Crohn disease)
  • Amyloidosis
  • Neurocutaneous disorders (tuberous sclerosis, neurofibromatosis, ataxia-telangiectasia)
Bronchiolitis obliterans: This may be the histologic pattern associated with connective tissue disorders or other chronic inflammatory disorders, such as inflammatory bowel disease. It may be seen as a noninfectious pulmonary complication of bone marrow transplantation (associated with graft vs host disease [GVHD]) or lung transplantation and may be seen in association with malignancies. Bronchiolitis obliterans syndrome (BOS) is a clinical term that refers to irreversible airway obstruction (defined as a decrease in forced expiratory volume in 1 second [FEV1] of >20% from baseline) after lung transplantation, in the absence of other causes.

Disorders with presenting features similar to those of ILD

  • Pulmonary venoocclusive disorders (8-10%) (anomalous pulmonary venous return, pulmonary hemangiomatosis, hereditary hemorrhagic telangiectasia, alveolar capillary dysplasia, pulmonary venous stenosis/atresia) 
  • Proliferative and congenital vascular disorders (alveolar capillary dysplasia and misalignment of pulmonary veins) 
  • Heart disease (left ventricular failure, left-to-right shunts) 
  • CF 
  • Immunodeficiency
Forms of ILD most prevalent in infancy
  • Diffuse developmental disorders
    • Acinar dysplasia
    • Congenital alveolar dysplasia
    • Alveolar capillary dysplasia with pulmonary vein misalignment (This is associated with a poor prognosis.)
  • Growth abnormalities
    • Pulmonary hypoplasia
    • Chronic neonatal lung diseases (prematurity-related BPD and acquired chronic lung diseases in term infants)
    • Structural pulmonary changes with chromosomal abnormalities (eg, trisomy 21)
    • Abnormalities associated with congenital heart disease in otherwise healthy children
  • Specific conditions with unknown etiology
    • PIG 
    • NEHI
  • SP dysfunction mutations and related disorders
    • SFTPB genetic mutations (PAP as dominant histologic pattern; see below) 
    • SFTPC genetic mutations 
    • ABCA3 genetic mutations 
    • Granulocyte-macrophage colony stimulating factor (GM-CSF) receptor mutations
Genetic and/or familial disorders
  • Mutations in genes for SP-B, SP-C, and ABCA3
  • Familial hypocalciuric hypercalcemia
  • Lysinuric protein intolerance
  • Farber lipogranulomatosis
  • Hermansky-Pudlak syndrome
PAP is characterized by amorphous periodic acid-Schiff (PAS)-positive intra-alveolar lipoproteinaceous material. PAP can be associated with inherited abnormalities of surfactant metabolism that cause severe neonatal respiratory distress. Although most forms of PAP are either idiopathic or acquired, several conditions have been described in association with PAP, including lysinuric protein intolerance, congenital cellular immunodeficiency, AIDS, myeloid leukemias, sideroblastic anemia, and infections with Pneumocystis carinii, Nocardia species, and Histoplasma capsulatum.7 Mutations in genes that encode for SFTPB, ABCA3, and the beta chain of the receptor for GM-CSF (CSF2RB) have been found in the neonatal form of PAP. Other forms of PAP develop weeks to years after the newborn period and associated with a variable progression. 

The 4 major SPs are A, B, C, and D. The lung collectins (SP-A and SP-D) function as opsonins for pathogens and also function as immunomodulators that regulate the inflammatory response in the alveolar space. Their levels are elevated in adults with IPF, in adults with ILD with collagen vascular disease, and in adults withPAP.8 In children with ILD, SP-A and SP-D levels are correlated with some measures of disease severity.

SP-A deficiency was first described in animal models of BPD. Selman et al However, so far, no human infants with SP-A deficiency have been identified.

SP-B deficiency is inherited in an autosomal recessive manner. When it is homozygous, it is highly lethal during newborn period. The radiologic appearance is similar to that of hyaline membrane disease. Patients do not respond to surfactant replacement therapy, and many of them require extracorporeal membrane oxygenation (ECMO). They eventually require lung transplantation. Heterozygous family members of infants with SP-B deficiency were free of pulmonary symptoms and had normal lung function.9

Recently, familial pulmonary fibrosis has been associated with mutations in the SFTPC gene. SP-C deficiency can have variable clinical presentations, even in members of the same family.10,11 Its inheritance is autosomal dominant with variable penetrance. Patients can present with severe symptoms in the first few months of life, can present with symptoms of ILD in adulthood or they may remain asymptomatic. A recent study investigating a possible role of high-frequency SP-C variants in common pediatric disorders demonstrated that SP-C variants represent a risk factor for the development of severe respiratory syncytial virus (RSV) infection

Mutations of ABCA3, the gene that encodes for a transmembrane protein that transports substances across biologic membranes and that has been localized to the lamellar bodies, are inherited in an autosomal recessive fashion. Mutations in ABCA3 gene may be the most common genetic cause of neonatal lung disease.

In 2004, Shulenin et al described 21 infants with severe neonatal surfactant deficiency with an unknown etiology; mutations in ABCA3 were identified in 16 of 21 patients.13 The exact function of the ABCA3 protein is unknown, but it is critical for the lipid transport into lamellar bodies and proper surfactant function.14 The clinical picture in this condition varies; it might be lethal in newborns, but some patients have a more protracted course, and some are living as adolescents with ILD.15,16  

The findings that the complete absence of ABCA3 function results in severe surfactant deficiency and that some mutations may result in milder lung disease in the neonatal period indicates that ABCA3 may be a candidate gene for more common lung diseases such as neonatal respiratory distress syndrome (RDS) in premature infants.17

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References
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Further Reading

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Keywords

children’s interstitial lung disease, ChILD, ILD, diffuse infiltrative lung disease, pulmonary disease, lung disease, interstitial disease, idiopathic pulmonary fibrosis, IPF, nonidiopathic interstitial pulmonary fibrosis, usual interstitial pneumonia, UIP, desquamative interstitial pneumonia, DIP, bronchiolitis obliterans with interstitial pneumonia, BIP, lymphoid interstitial pneumonia, lymphocytic interstitial pneumonia, LIP, giant cell interstitial pneumonia, giant-cell interstitial pneumonia, GIP, respiratory bronchiolitis interstitial lung disease, RBILD

nonspecific interstitial pneumonia, NSIP, bronchiolitis obliterans organizing pneumonia, BOOP, cryptogenic organizing pneumonia, COP, cryptogenic fibrosing alveolitis, CFA, pulmonary histiocytosis X, eosinophilic granuloma, Langerhans cell histiocytosis, LCH, acute interstitial pneumonia, AIP, idiopathic BOOP, nonclassifiable ILD, neuroendocrine cell hyperplasia of infancy, NEHI, pulmonary interstitial glycogenosis, PIG, idiopathic interstitial pneumonia, cryptogenic fibrosing alveolitis, chronic pneumonitis of infancy, cellular interstitial pneumonitis

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Contributor Information and Disclosures

Author

James S Hagood, MD, Director, Pediatric Pulmonary Center, Associate Professor of Pediatrics, Cell Biology and Pathology, Department of Pediatrics, University of Alabama 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, Fellow in Pediatric Pulmonology, Department of Pediatrics, Division of Pulmonary Medicine, University of Alabama School of Medicine
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 W Young, MD, Clinical Professor of Radiology, Clinical Asst Professor of Pediatrics, University of Alabama School of Medicine; Consulting Staff, Department of Radiology, The Children's Hospital of Alabama; President, Pediatric Radiology Associates, PC
Daniel W Young, MD 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 of Clinical Pathologists and College of American Pathologists
Disclosure: Nothing to disclose.

Medical Editor

Susanna A McColley, MD, Director of Cystic Fibrosis Center, Divisions of Pediatric Pulmonary and Critical Care, Associate Professor, Department of Pediatrics, Children's Memorial Medical Center of Chicago, Northwestern University
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: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Associate Professor, Department 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, American Heart Association, and American Thoracic Society
Disclosure: Nothing to disclose.

Chief Editor

Michael R Bye, MD, Attending Physician, Pediatric Pulmonary Medicine, Columbia University Medical Center; Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons
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: Merck Honoraria Speaking and teaching

 
 
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