Objective: To describe the clinical spectrum and factors associated with poor short-term outcomes in children with interstitial lung disease (ILD).
Design: Retrospective chart review
Setting: Pediatric Chest Clinic of a tertiary care hospital
Methodology: We retrieved information regarding clinical course and laboratory features of all children diagnosed as ILD between January 1999 and February 2010. Disease severity was assessed using ILD score based on clinical features and SpO2 at the time of initial evaluation. Outcome was assessed after 3 months of initial diagnosis as improved or death/no improvement in symptoms.
Results: 90 children (median age, 6.8 years; 62% boys) were diagnosed to have ILD during this period. 46 children were classified as having definite ILD while 44 had possible ILD. The commonest clinical features at presentation were cough (82.2%), dyspnea (80%), pallor (50%), and crackles (45.6%). 3 children (3.3%) died while 21 (23%) showed no improvement in clinical status on follow-up at 3 months. A higher ILD score (RR 3.72, 95% CI 1.4, 9.9) and lower alkaline phosphatase levels (median [IQR]: 205 [175.2] vs. 360 [245.7]; P=0.006) were found to be significantly associated with worse outcomes.
Conclusion: The common clinical features of ILD in our study included breathlessness, cough and hypoxemia. A working diagnosis of ILD can be made with the help of imaging, bronchoscopy, or lung biopsy. A simple score based on clinical findings and pulse-oximetry might predict those children with poor short-term outcome.