Interstitial Lung Disease In Adults Research Articles

Olipudase alfa enzyme replacement therapy reverses interstitial lung disease in adults with acid sphingomyelinase deficiency: Long-term pulmonary outcomes of the ASCEND trial

Olipudase alfa enzyme replacement therapy reverses interstitial lung disease in adults with acid sphingomyelinase deficiency: Long-term pulmonary outcomes of the ASCEND trial

Diffuse alveolar haemorrhage in children: an international multicentre study

Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. A retrospective, descriptive multicentre follow-up study initiated from the European network for translational research in children's and adult interstitial lung disease (Cost Action CA16125) and chILD-EU CRC (the European Research Collaboration for Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before the age of 18 years. Data of 124 patients from 26 centres (15 counties) were submitted, of whom 117 patients fulfilled the inclusion criteria. Diagnoses were idiopathic pulmonary haemosiderosis (n=35), DAH associated with autoimmune features (n=20), systemic and collagen disorders (n=18), immuno-allergic conditions (n=10), other childhood interstitial lung diseases (chILD) (n=5), autoinflammatory diseases (n=3), DAH secondary to other conditions (n=21) and nonspecified DAH (n=5). Median (IQR) age at onset was 5 (2.0-12.9) years. Most frequent clinical presentations were anaemia (87%), haemoptysis (42%), dyspnoea (35%) and cough (32%). Respiratory symptoms were absent in 23%. The most frequent medical treatment was systemic corticosteroids (93%), hydroxychloroquine (35%) and azathioprine (27%). Overall mortality was 13%. Long-term data demonstrated persistent abnormal radiology and a limited improvement in lung function. Paediatric DAH is highly heterogeneous regarding underlying causes and clinical presentation. The high mortality rate and number of patients with ongoing treatment years after onset of disease underline that DAH is a severe and often chronic condition. This large international study paves the way for further prospective clinical trials that will in the long term allow evidence-based treatment and follow-up recommendations to be determined.

Living Donor Lobar Lung Transplant for a Patient With Lung Disease Caused by ABCA3 Gene Mutations: A Case Report

Recessive gene mutations in ABCA3 cause lethal neonatal respiratory distress, and pediatric and adult interstitial lung disease. The effectiveness of medical treatments is limited and a subset of such patients will eventually require lung transplantation. A 20 months old boy developed interstitial lung disease and was treated with hydroxychloroquine, which had a significant effect. Sequence analysis of ABCA3 gene revealed newly discovered compound heterozygous mutations. His respiratory dysfunction gradually progressed over years and he underwent living-donor lobar lung transplantation (LDLLT) at 8 years of age with his parents serving as bilateral lobar donors. The parents had been genetically examined beforehand and found to be carriers who had one allele with an ABCA3 gene mutation and the other with no mutation. The recipient has been well without chronic lung allograft dysfunction and his parents have been enjoying healthy social lives for 7 years since the operations. LDLLT appears to be a valid option for selected children with ABCA3 gene mutations who are too ill to wait for cadaveric lung transplantation. When relatives of the recipient with ABCA3 gene mutation are deemed potential donors for LDLLT, sequence analyses of the donors are indispensable to exclude the possibility that they are late-onset patients of this recessive hereditary disease.

Familial interstitial lung disease associated with surfactant protein C gene mutation in adults: report of two cases and literature review

Objective: To improve the understanding of clinical manifestations, imaging findings, diagnosis and treatment of surfactant protein C gene (SFTPC) mutation associated with familial interstitial lung disease in adults. Methods: Two cases of adult SFTPC gene mutation associated with familial interstitial lung disease diagnosed in the Affiliated Hospital of Medical School of Ningbo University were analyzed retrospectively, and the literature was reviewed. The literatures were retrieved with "family interstitial lung disease" "SFTPC gene" "surface protein C gene" "SFTPC gene mutation associated with familial international lung disease" and "surface protein C gene mutation associated with familial international lung disease" in PubMed, Embase, Ovid, Wanfang database and China National Knowledge Infrastructure (CNKI). Results: There were two patients with familial interstitial lung diseases(one male and one female) with an average age of 27.5 years. Ⅱ-2 patient had symptoms of dry cough and shortness of breath, and Ⅱ-1 patient had no symptoms. There were multiple cysts and fine reticular shadows in both cases. Ⅱ-2 patient had multiple ground glass opacities in both lower lungs. TheⅡ-2 patient was diagnosed with usual interstitial pneumonia (UIP) by transbronchial lung cryobiopsy. A total of 35 patients were included in this literature review, including 20 males, with an average age of 33.5 years. Of all the patients, the clinical symptoms were described in 30 patients. The main manifestations were shortness of breath (22/30), dry cough (18/30), clubbing finger (12/30), and 30% (9/30) of them were found by chest computerized tomography (CT) without symptoms. There were 17 cases with detailed description of chest CT imaging. The most common chest CT findings were multiple intralobular reticular opacities (17/17), multiple cysts (12/17) and ground glass opacities (7/17). The main histopathological pattern was UIP (24/26). Conclusions: The main clinical manifestations of SFTPC gene mutation associated with familial interstitial lung disease in adults are shortness of breath, dry cough and clubbing fingers. The main manifestations are multiple cysts and intralobular reticular opacities in combination with multiple ground glass opacities. There is no specific drug in the treatment at present and early treatment with hydroxychloroquine may have better curative effect. When the imaging findings show multiple cysts and intralobular reticular opacities in combination with multiple ground glass opacities, especially the age of onset is less than 50 years old, this disease should be considered.

Transition of patients with interstitial lung disease from paediatric to adult care

The European Cooperation in Science and Technology (COST) is a funding organisation for the creation of research networks, called COST Actions. COST Action 16125 was dedicated to European network for translational research in children's and adult interstitial lung disease. Working Group 5 of the COST Action CA16125 focussed on the problems of transition of children with interstitial lung diseases from paediatric to adult care. Results of this survey performed among members of the Working Group and some affiliated specialists showed highly variable and inadequate current system of transitions of these patients to adult care in most centres. In most centres there is no established and consistent procedure to guarantee appropriate transfer of information and prepare the patient for transition to a new specialist and a new team. Immediate action is therefore required. The Working Group has prepared a model procedure for the transition, based on protocols from several centres with established pathways, either for childhood interstitial lung disease or other chronic respiratory diseases.

Hermansky-Pudlak syndrome-2 alters mitochondrial homeostasis in the alveolar epithelium of the lung

BackgroundMitochondrial dysfunction has emerged as an important player in the pathogenesis of idiopathic pulmonary fibrosis (IPF), a common cause of idiopathic interstitial lung disease in adults. Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder that causes a similar type of pulmonary fibrosis in younger adults, although the role of mitochondrial dysfunction in this condition is not understood.MethodsWe performed a detailed characterization of mitochondrial structure and function in lung tissues and alveolar epithelial cells deficient in the adaptor protein complex 3 beta 1 (Ap3b1) subunit, the gene responsible for causing subtype 2 of HPS (HPS-2).ResultsWe observed widespread changes in mitochondrial homeostasis in HPS-2 cells, including the acquisition of abnormally shaped mitochondria, with reduced number of cristae, and markedly reduced activity of the electron transport chain and the tricarboxylic acid cycle. We also found that mitochondrial redox imbalance and activity of the mitochondrial unfolded protein response were dysregulated in HPS-2 cells and this associated with various other changes that appeared to be compensatory to mitochondrial dysfunction. This included an increase in glycolytic activity, an upregulation in the expression of mitochondrial biogenesis factors and enhanced activation of the energy-conserving enzyme AMP-activated protein kinase.ConclusionIn summary, our findings indicate that mitochondrial function is dramatically altered in HPS-2 lung tissues, suggesting dysfunction of this organelle might be a driver of HPS lung disease.

Performance Characteristics of Pulmonary Function Tests for the Detection of Interstitial Lung Disease in Adults With Early Diffuse Cutaneous Systemic Sclerosis.

Interstitial lung disease (ILD) is the leading cause of death in patients with systemic sclerosis (SSc). Although pulmonary function tests (PFTs) are commonly used to screen for ILD in patients with SSc, studies have shown that they lack sensitivity for the detection of ILD in general SSc cohorts. This study was undertaken to assess the performance characteristics of PFTs for the detection of ILD in patients with early diffuse cutaneous SSc (dcSSc), a population at high risk for the development of ILD. We performed a retrospective cohort study of patients enrolled in the Prospective Registry of Early Systemic Sclerosis at 11 sites in the US between April 2012 and January 2019. Patients were included if they underwent spirometry and high-resolution computed tomography (HRCT) of the chest. We calculated the performance characteristics of PFTs for the detection of ILD on HRCT. The study included 212 patients, 54% of whom had radiographic ILD. For the detection of ILD on HRCT imaging, a forced vital capacity (FVC) <80% predicted had a sensitivity of 63%. The combination of FVC <80% predicted or diffusing capacity for carbon monoxide (DLco) <80% predicted improved the sensitivity to 85%. An FVC <80% predicted had a negative predictive value (NPV) of 61%, while the combination of FVC <80% predicted or DLco <80% predicted had an NPV of 70%. PFTs alone are an inadequate screening tool for the diagnosis of ILD in patients with early dcSSc. HRCT should be part of the ILD screening algorithm in patients with dcSSc.

Serum KL-6 level as a biomarker of interstitial lung disease in childhood connective tissue diseases: a pilot study.

Krebs von den Lungen-6 (KL-6) has been described as a promising biomarker in the diagnosis and determining the severity of interstitial lung disease in adults with connective tissue disease. This study was performed to determine whether the serum KL-6 level is useful as a biomarker for detecting the interstitial lung disease (ILD) in pediatric cases of connective tissue disease (CTD). In total, 88 patients [36 patients with systemic juvenile idiopathic arthritis (sJIA), 27 patients with juvenile systemic lupus erythematosus (JSLE), 14 patients with juvenile dermatomyositis (JDM), and 11 patients with juvenile systemic sclerosis (JSSc)] and 68 healthy controls were included in this study. Age, sex, and duration of CTD and ILD (if any) were recorded. Blood samples from all the patients and controls were examined by ELISA. Eleven of the 88 patients with CTD (12.5%) had ILD and all of them were symptomatic. Subgroup analysis indicated that eight patients had JSSc, two had JSLE, and one had systemic JIA. The median serum KL-6 level was 1450.5U/mL (interquartile range (IQR) 742.9-2603.2) in the CTD with ILD group, 415.9U/mL (IQR 233.4-748.4) in the CTD without ILD group, and 465.9U/mL (IQR 273.6-1036) in the control group. KL-6 levels were significantly higher in the CTD with ILD group than the CTD without ILD group and the control group (p = 0.003). At a cut-off of 712.5 U/ml identified by ROC curve, serum KL-6 yielded a sensitivity of 81% and specificity of 72% for CTD with ILD group. There was no significant difference in serum KL-6 level among the disease subgroups (sJIA, JSLE, JSSc, JDM), in either the CTD with ILD group or the CTD without ILD group (p > 0.05). In conclusion, KL-6 is a useful biomarker of CTD with ILD in pediatric patients.

Abandoning developmental silos: what can paediatricians and adult interstitial lung disease physicians learn from each other?

Interstitial lung disease (ILD) consists of a large and heterogeneous group of disorders that are classified together because of similar clinical, radiographic, physiologic or pathologic manifestations. Overall, although there is overlap between adult and childhood ILD (chILD), there are many differences in disease causes and prevalences. In the last few years, our understanding of adult ILD pathobiology has improved substantially. This is particularly true for idiopathic pulmonary fibrosis, the most common of the idiopathic interstitial pneumonias, wherein recently developed guideline documents provide recommendations for the diagnosis and clinical management of patients. For chILD, similar guidelines are yet to be developed. However, complications and long-term pulmonary outcomes of paediatric disease are better appreciated, which make the implementation of a successful transition program from paediatric to adult care an urgent need. Similarly important is the development of guidelines on performance and interpretation of genetic testing in affected and unaffected relatives of familial cases and in children of adult-onset ILD patients. Lung transplantation appears to be as successful as in adult patients for end-stage disease. Paediatric pulmonologists should engage with the adult multidisciplinary teams and benefit from their much more extensive experience. Childhood and adult ILD share a number of aspects, which give children and adult ILD specialists exciting opportunities to collaborate and learn from each other. Such collaborative effort between child and adult ILD experts is crucial for successful future development in the field.

Association of anti-nuclear matrix protein 2 antibody with complications in patients with idiopathic inflammatory myopathies: A meta-analysis of 20 cohorts

BackgroundSeveral complications like calcinosis, interstitial lung disease (ILD) or malignancy, are primary causes leading to poor outcomes in idiopathic inflammatory myopathies (IIM) patients. Specific antibodies might help to indicate the occurrence or absence of these complications. ObjectiveThe aim of this study was to evaluate the association of anti-nuclear matrix protein 2 antibody (anti-NXP2) with calcinosis, ILD and malignancy in IIM patients. MethodsTwo investigators independently searched literature about the relation of anti-NXP2 with calcinosis, ILD, malignancy in IIM patients in PubMed, EMBASE, Web of Science databases, then selected eligible articles and extracted data from the included studies. The association between anti-NXP2 and these complications was assessed by odds ratios (OR) and 95% confidence intervals (95% CI). Further quantitative meta-analysis, subgroup analysis, sensitivity analysis and publication bias analysis were conducted with STATA 14.0 software (Stata Corp.; College Station, Texas, USA). A fixed-effects model (the Mantel-Haenszel method) was employed when I2 < 25%, otherwise a random-effects model (the Mantel-Haenszel method) was used. ResultsTwenty cohorts with 3064 IIM patients were included in this meta-analysis, among which 9 were about calcinosis in adults, 6 about calcinosis in juvenile patients, 9 about ILD in adults, 3 about ILD in juvenile patients, while 13 about malignancy in adult patients. Anti-NXP2 was more common in patients with calcinosis than those without calcinosis (pooled OR = 4.00, 95% CI: 2.65–6.06 in adults; pooled OR = 1.62, 95% CI: 1.14–2.30 in juvenile patients). On the contrary, this antibody was less common in adult patients with ILD than those without ILD (pooled OR: 0.33, 95% CI: 0.19–0.56). No significant difference concerning the incidence of anti-NXP2 antibody was found in IIM patients between those with and without cancer (pooled OR = 1.42, 95% CI: 0.69–2.91). ConclusionThe present study indicates that anti-NXP2 autoantibody is a risk factor for development into calcinosis both in adult and juvenile patients, while a protective factor for ILD in adult patients. Anti-NXP2 had no relation with malignancy in adult patients.

Lavage bronchoalvéolaire de l’enfant et de l’adulte, rôle du pathologiste

Bronchoalveolar lavage (BAL) is a common diagnostic tool in the exploration of children and adult interstitial lung disease. First of all we will develop the technical aspects and transport modalities of the BAL specimen, then the role of the pathologist will be discussed. At last we will examine the key diseases of the children and adults. Accurate clinical data are mandatory to a good BAL analysis.

Variants of the ABCA3 gene might contribute to susceptibility to interstitial lung diseases in the Chinese population

ATP-binding cassette A3 (ABCA3) is a phospholipid carrier that is mainly expressed in the alveolar epithelium. Biallelic mutations of ABCA3 has been associated with fatal respiratory distress syndrome and interstitial lung disease (ILD) in children. However, whether variations in ABCA3 have a role in the development of adult ILD, including idiopathic pulmonary fibrosis (IPF), remains to be addressed. In this study, we screened for germline variants of ABCA3 by exons-sequencing in 30 patients with sporadic IPF and in 30 matched healthy controls. Eleven missense variants, predominantly in heterozygous, were found in 13 of these patients, but only two missenses in 2 healthy controls. We then selected four of the detected missense variants (p.L39V, p.S828F, p.V968M and p.G1205R) to performed cohort analysis in 1,024 ILD patients, containing 250 IPF and 774 connective tissue disease-ILD (CTD-ILD) patients, and 1,054 healthy individuals. Our results showed that the allele frequency of p.G1205R, but not p.L39V, was significantly higher in ILD patients than in healthy controls. However, no additional subject carrying the variant p.S828F or p.V968M was detected in the cohort analysis. These results indicate that the heterozygous ABCA3 gene variants may contribute to susceptibility to diseases in the Chinese population.

Deciphering the mechanism of Q145H SFTPC mutation unmasks a splicing defect and explains the severity of the phenotype.

Mutations in the gene encoding surfactant protein C (SFTPC) have led to a broad range of phenotypes from neonatal respiratory distress syndrome to adult interstitial lung disease. We previously identified the c.435G>C variant in the SFTPC gene associated with fatal neonatal respiratory distress syndrome in an infant girl. Although this variation is predicted to change glutamine (Q) at position 145 to histidine (H), its position at the last base of exon 4 and the severity of the phenotype suggested that it might also induce a splicing defect. To test this hypothesis, we used hybrid minigene, biochemical and immunofluorescence tools to decipher the molecular mechanism of the mutation. Immunoblotting and confocal imaging showed similar maturation and localization of wild-type and Q145H proteins, but hybrid minigene analysis showed complete exon 4 skipping. Since the exon 4 is in frame, a putative truncated protein of 160 amino acids would be produced. We have shown that this truncated protein had an altered intracellular trafficking and maturation. The c.435G>C mutation is deleterious not because of its amino acid substitution but because of its subsequent splicing defect and should be referred to as r.325_435del and p.Leu109_Gln145del. The absence of residual full-length transcripts fully explained the severity of the phenotype we observed in the infant.

The radiology of diffuse interstitial pulmonary disease in children: pearls, pitfalls and new kids on the block in 2015.

Diffuse interstitial lung disease in children differs markedly from interstitial lung disease in adults and is a distinct entity. The childhood interstitial lung disease (ChILD) classification, devised in 2010 separates conditions into those occurring in infancy, and those not specific to infants, the later group containing many conditions related to systemic diseases (including connective tissue diseases and depositional/storage disorders), and conditions occurring in immunocompromised children. In this article, we briefly review normal lung growth and development. We discuss our preferred technique for imaging the lungs with computed tomography in children, and review the recent literature regarding the radiological appearance of various ChILD. We illustrate this with cases from our institution and emphasize the more recently recognised conditions including pleuroparenchymal fibroelastosis and filamin A deficiency-related lung disease.

Clinical and ultrastructural spectrum of diffuse lung disease associated with surfactant protein C mutations.

Genetic defects of surfactant metabolism are associated with a broad range of clinical manifestations, from neonatal respiratory distress syndrome to adult interstitial lung disease. Early therapies may improve symptoms but diagnosis is often delayed owing to phenotype and genotype variability. Our objective was to characterize the cellular/ultrastructural correlates of surfactant protein C (SP-C) mutations in children with idiopathic diffuse lung diseases. We sequenced SFTPC - the gene encoding SP-C - SFTPB and ABCA3, and analyzed morphology, ultrastructure and SP expression in lung tissue when available. We identified eight subjects who were heterozygous for SP-C mutations. Median age at onset and clinical course were variable. None of the mutations were located in the mature peptide-encoding region, but were either in the pro-protein BRICHOS or linker C-terminal domains. Although lung morphology was similar to other genetic surfactant metabolism disorders, electron microscopy studies showed specific anomalies, suggesting surfactant homeostasis disruption, plus trafficking defects in the four subjects with linker domain mutation and protein misfolding in the single BRICHOS mutation carrier in whom material was available. Immunolabeling studies showed increased proSP-C staining in all cases. In two cases, amyloid deposits could be identified. Immunochemistry and ultrastructural studies may be useful for diagnostic purposes and for genotype interpretation.

The importance of understanding epidemiology in order to inform financial decisions: A lesson from the Scottish Home Oxygen Service

To ensure that decisions on the future planning of the Scottish Home Oxygen Service reflect population needs by examining the epidemiology of the main conditions that require home oxygen therapy and trends in their management. Analysis of routinely available vital event and health service data supplemented by published literature. Use of linked data to provide person-based analyses. Consideration of trends in key risk factors, disease incidence, prevalence and mortality for chronic neonatal lung disease, cystic fibrosis, chronic interstitial lung disease in adults and chronic obstructive pulmonary disease. Examination of trends in management of these conditions including hospital admissions, length of stay and re-admissions. The prevalence of all the conditions studied has increased in Scotland over recent years due to a combination of increased incidence, increased survival, more active case finding and demographic changes. There have been changes in management with trends towards shorter hospital stays. The clinical need for home oxygen therapy is likely to continue to increase over the next 10-20 years. It will encompass all age groups and a complex range of conditions. Public health needs to be proactive in providing relevant needs assessment information to ensure that planning within financial constraints is appropriately informed on population needs.

Round Table Discussion: Children's Interstitial Lung Disease

Robin R. Deterding: We have the opportunity today to talk about children’s interstitial lung disease (chILD) with experts in that fi eld. My hope is that we can have a lively discussion about what we know is important in chILD and what we see coming on the horizon. I would like to begin our discussion with a question: Why is it important to recognize that chILD differs from i nterstitial lung disease (ILD) in adults? Lisa, would you like to give your thoughts about this? Lisa R. Young: I think this is an extremely important question, about which we have learned a lot in the past sev-eral years. A major reason for the importance of distinguish-ing chILD from adult ILD has to do with what patients read and think about with respect to these 2 diagnoses. A family that turns to the Internet in the belief that it has a member affected by ILD can become very quickly frightened, for ex-ample, in reading about the prognosis in idiopathic pulmo-nary fi brosis (IPF), a common form of ILD in adults but one that does not occur in children. Robin R. Deterding: Alan, what are your thoughts as a radiologist? Alan S. Brody: I agree with Lisa, and I think that some things that look like but are not the same as adult IPF may be a problem for the radiologist as well as for the clinician who does not deal with chILD, as well as for the families of patients. The 2 conditions should not be treated in the same way; they do not have the same prognosis. We did not know that years ago, but today it is very clear, as Lisa observed, that IPF, the most common adult form of ILD, does not occur in children, and if someone gives a child that diagnosis, we can tell the family right away that it is not correct, that there is more work to do to understand their child’s ILD. The prognosis of chILD is very variable and often much better than adult IPF. Lisa R. Young: We’re also learning that the etiology of chILD is often very different from the types of ILD seen in adults, and we think that understanding the eti-ology of the childhood disorder is a key step toward its treatment. James S. Hagood: I agree. For adults and physicians car-ing for adults with ILD, by far the largest category of disease is IPF, which does not appear to occur in children. And while many secondary forms of ILD and pulmonary fi brosis may be similar to what we see in older children and adolescents with rheumatic disease or hypersensitivity pneumonitis in terms of specifi c causes, the prognosis and management may differ in children. Robin R. Deterding: All of these are excellent observa-tions. The only thing I would add is that ILD in infants and children may improve or transform into a different type of lung disease, that may be similar to or different than adult ILD, as they grow older. It is very diffi cult to know what may happen until we can correctly classify these various condi-tions and follow them over time. I would like to discuss the presentations of ILD in chil-dren. What are your experiences with this, and when might you think you are dealing with a child with ILD? Lisa R. Young: The children I see generally have 2 dif-ferent categories of ILD. One of them has a very dramatic presentation, such as respiratory failure, and this generally presents in neonates at birth. I fi nd it can be more diffi cult to identify ILD in older children, which may be insidious in onset. Children may present with progressive exercise intol-erance marked only by a gradual slowing and falling behind their friends or siblings in their physical activities. In babies, the symptoms of this may include trouble with feeding, and may look like refl ux or other feeding problems, but may in-stead be related to their diffi culty with breathing. Alan S. Brody: As a radiologist, most of the children sent to me are referred, and for a number of reasons, but there is almost always some concern about the presence of lung

Infants and Young Children with Children's Interstitial Lung Disease

Though interstitial lung disease (ILD) can occur at any age in children, disorders more common in infancy and young children have received increased attention as an important group that is disproportionally affected, linked to lung development and lung injury, and represents disorders not seen in adult ILD. Identifying those children with potential children's ILD (chILD) and establishing a specific chILD diagnosis has evolved and is critical for pediatric pulmonologists, neonatologists, radiologists, and pathologists to recognize. Specific disorders more common in infancy include diffuse developmental disorders, growth abnormalities, pulmonary interstitial glycogenosis, neuroendocrine cell hyperplasia of infancy, and surfactant mutation dysfunction mutations. The presentation, evaluation, treatment, and clinical course are discussed for each of these specific disorders and other categories less common in infants and young children are briefly mentioned. Resources for physicians and families are also reviewed.

Introducing Pediatric Allergy, Immunology, and Pulmonology

Pediatric Asthma, Allergy & Immunology is now Pediatric Allergy, Immunology, and Pulmonology. Welcome to our first issue under our new name! The name change makes explicit the tradition of Pediatric Asthma, Allergy, & Immunology of publishing articles relevant to both allergy/immunology and pulmonology. Bringing together pediatric allergy/immunology and pulmonology is highly relevant. There are many areas—such as asthma—where allergy/immunology and pulmonology overlap. There are many other areas where understanding of the allergy, immunology, and pulmonology leads to a more complete understanding of the disease process. This journal will provide comprehensive coverage to further the understanding, and optimize the treatment, of some of our most common and costly chronic illnesses in children. This journal will publish original translational, clinical, epidemiologic, public health, and quality improvement research, reviews, and commentaries. Interviews with leading scientists will describe our current history. Periodic themed issues or sections will review rapidly changing and/or under-recognized areas. Roundtable discussions will elucidate current expert opinion and experience in evolving controversial areas of clinical management of pulmonary, allergic, and immunologic diseases of childhood. This issue is a special issue on children's interstitial lung disease (also called diffuse lung disease, as it often involves more than just the interstitium). We are dedicating this issue to Dr. Bettina Hilman, who served as editor for this journal from 1999 to 2009. As Dr. Hilman pointed out years ago, children's interstitial lung disease may not simply be a smaller version of adult interstitial lung disease.1 The rapid evolution of the field of interstitial lung disease of children bears this out. New forms of interstitial lung disease have been identified in children that have not been previously recognized in adults. Other interstitial lung diseases have been found to have a different prognosis and/or course in children as compared with adults. Interstitial lung diseases are one of many disease processes that cross the lines between pulmonology, allergy, and immunology. Etiologies may be genetic (such as surfactant protein abnormalities), allergic (such as hypersensitivity pneumonitis), and/or immunologic (such as lymphoid interstitial pneumonitis). I feel privileged to bring this collection of articles together in this journal. I am particularly grateful to the guest editors, Robin Deterding and Leland Fan, whose insight, vision, and willingness to take on yet another project made this issue possible. I am grateful to the authors who have developed this field and contributed such excellent reviews. I look forward to the contributions that this journal can make to the fields of pediatric allergy, immunology, and pulmonology.

Usual Interstitial Pneumonia in an Adolescent With ABCA3 Mutations*

Many diverse and frequently idiopathic disorders cause interstitial lung disease (ILD) in children. Although the histologic patterns of ILD in children and adults share similar features, important differences exist in etiology, clinical manifestations, and outcome. Usual interstitial pneumonia (UIP) is the most frequent histologic pattern in adult ILD; however, the characteristic histologic features of UIP have yet to be demonstrated in a child. We report a 15-year-old boy with the UIP pattern of pulmonary fibrosis who had mutations in the adenosine triphosphate-binding-cassette-A3 gene. Discovery of how genetic mutations of proteins involved in surfactant biosynthesis lead to progressive fibrosis will have implications for the understanding of the pathogenesis and clinical manifestations of ILD in both adults and children.