Clinical spectrum of chronic interstitial lung disease in children L e l a n d L. Fan, MD, Ann L. W. Mullen, RN, MS, Susan M. Brugman, MD, S t e p h e n C. Inscore, MD, D a v i d P. Parks, MD, a n d Carl W. White, MD From the Divisionof Pediatric Pulmonology, Natio~nalJewish Center for Immunology and Respiratory Medicine, Denver, Colorado, the Department of Pediatrics, Universityof Colorado Health Sciences Center, Denver, and the Department of Pediatrics, Brooke Army Medical Center, Fort Sam Houston, Texas
To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, m a d e in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortality rates. (J PEDIATR1992;121:867"72) Interstitial lung disease refers to a group of chronic pulmonary disorders characterized by derangements of alveolar walls and loss of alveolar capillary units. Although more than 100 different disorders have been identified, the majority of patients have ILD of unknown cause.l The term "chronic diffuse infiltrative lung disease" has been advocated because involvement of alveolar air spaces is often present as well. 2 Recognizing its limitations, we will use the more familiar term, ILD. Usual interstitial pneumonitis, also known as idiopathic pulmonary fibrosis and cryptogenic fibrosing alveolitis, is one of the most common forms of idiopathic ILD in adults. Another idiopathic disorder is desquamative interstitial pneumonitis, which is characterized by hyperptasia of alve-
Supported in part (Dr. White) by a Grant-in-Aid and an Established Investigator Award from the American Heart Association National Center, and National Institutes of Health SCOR I P50 HL 46481-01. Submitted for publication Dec. 30, 1991; accepted July 28, 1992. Reprint requests: Leland L. Fan, MD, National Jewish Center for Immunology and Respiratory Medicine, 1400 Jackson, Denver, CO 80206.
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olar-lining cells and accumulation of mononuclear cells in the alveolar spaces. Some investigators believe that DIP and U1P represent different stages of the same disease process) Considerable information has accumulated about the pathogenesis of idiopathic pulmonary fibrosis, and there is also improved understanding of the clinical aspects of this condition in adults. 46 In children, ILD appears to be rare, but our current knowledge is fragmented. 7, 8 It remains to be determined
DIP FEV1 FVC ILD LIP UIP
Desquamativeinterstitial pneumonitis Forcedexpiratory volume in 1 second Forcedvital capacity Interstitial lung disease Lymphocyticinterstitial pneumonitis Usual interstitial pneumonitis
whether the information gained from adult investigations can be applied to pediatric ILD. To our knowledge, there have been no systematic studies of the clinical spectrum of ILD in children. Therefore we reviewed retrospectively our experience with pediatric ILD during the past 12 years to describe ( 1) the clinical findings of children with these disorders, (2) the diagnostic value of routine and invasive tests,
867
868
Fan et al.
The Journal o f Pediatrics December 1992
T a b l e I. Background data on 48 children with ILD
T a b l e II. Presenting symptoms and signs
Patients
Gender Male Female Year of initial evaluation 1980-1983 1984-1987 1988-1991 Age at onset (yr) 10 Duration of symptoms (yr) 3 Previous bronchodilator use
Patients
No.
%
23 25
48 52
9 14 25
19 29 52
22 11 9 6
46 23 19 13
27 8 13 17
56 17 27 35
(3) the variety of diagnoses encountered, (4) therapy, and (5) outcome.
Symptoms Cough Tachypnea Dyspnea Cyanosis Exercise intolerance Frequent respiratory infection Retractions Wheezing Hemoptysis Signs Rales Tachypnea Retractions Weight
To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, m a d e in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortality rates. (J PEDIATR1992;121:867"72) Interstitial lung disease refers to a group of chronic pulmonary disorders characterized by derangements of alveolar walls and loss of alveolar capillary units. Although more than 100 different disorders have been identified, the majority of patients have ILD of unknown cause.l The term "chronic diffuse infiltrative lung disease" has been advocated because involvement of alveolar air spaces is often present as well. 2 Recognizing its limitations, we will use the more familiar term, ILD. Usual interstitial pneumonitis, also known as idiopathic pulmonary fibrosis and cryptogenic fibrosing alveolitis, is one of the most common forms of idiopathic ILD in adults. Another idiopathic disorder is desquamative interstitial pneumonitis, which is characterized by hyperptasia of alve-
Supported in part (Dr. White) by a Grant-in-Aid and an Established Investigator Award from the American Heart Association National Center, and National Institutes of Health SCOR I P50 HL 46481-01. Submitted for publication Dec. 30, 1991; accepted July 28, 1992. Reprint requests: Leland L. Fan, MD, National Jewish Center for Immunology and Respiratory Medicine, 1400 Jackson, Denver, CO 80206.
9/20/41361
olar-lining cells and accumulation of mononuclear cells in the alveolar spaces. Some investigators believe that DIP and U1P represent different stages of the same disease process) Considerable information has accumulated about the pathogenesis of idiopathic pulmonary fibrosis, and there is also improved understanding of the clinical aspects of this condition in adults. 46 In children, ILD appears to be rare, but our current knowledge is fragmented. 7, 8 It remains to be determined
DIP FEV1 FVC ILD LIP UIP
Desquamativeinterstitial pneumonitis Forcedexpiratory volume in 1 second Forcedvital capacity Interstitial lung disease Lymphocyticinterstitial pneumonitis Usual interstitial pneumonitis
whether the information gained from adult investigations can be applied to pediatric ILD. To our knowledge, there have been no systematic studies of the clinical spectrum of ILD in children. Therefore we reviewed retrospectively our experience with pediatric ILD during the past 12 years to describe ( 1) the clinical findings of children with these disorders, (2) the diagnostic value of routine and invasive tests,
867
868
Fan et al.
The Journal o f Pediatrics December 1992
T a b l e I. Background data on 48 children with ILD
T a b l e II. Presenting symptoms and signs
Patients
Gender Male Female Year of initial evaluation 1980-1983 1984-1987 1988-1991 Age at onset (yr) 10 Duration of symptoms (yr) 3 Previous bronchodilator use
Patients
No.
%
23 25
48 52
9 14 25
19 29 52
22 11 9 6
46 23 19 13
27 8 13 17
56 17 27 35
(3) the variety of diagnoses encountered, (4) therapy, and (5) outcome.
Symptoms Cough Tachypnea Dyspnea Cyanosis Exercise intolerance Frequent respiratory infection Retractions Wheezing Hemoptysis Signs Rales Tachypnea Retractions Weight
