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Sequential pulmonary function measurements during treatment of infantile chronic interstitial pneumonitis
1Division of Respiratory Physiology, Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
High-dose methylprednisolone pulses and hydroxychloroquine effectively treat infantile chronic interstitial pneumonitis. Respiratory system compliance improved significantly, correlating with clinical recovery in infants.
Area of Science:
- Pediatric Pulmonology
- Pharmacological Treatments
- Respiratory Physiology
Background:
- Infantile chronic interstitial pneumonitis (ICIP) presents a diagnostic and therapeutic challenge.
- Limited effective treatment options exist for severe cases of ICIP in infants.
Observation:
- Three infants diagnosed with histologically confirmed ICIP were treated with intravenous methylprednisolone pulses.
- Adjunctive daily hydroxychloroquine therapy was administered to some patients.
- Pulmonary function was assessed using multiple and end-inspiratory occlusion techniques.
Findings:
- All three infants demonstrated clinical improvement following treatment.
- Significant increases in respiratory system compliance (60-100%) were observed, correlating with clinical recovery.
- Passive respiratory resistance and time constant did not consistently reflect clinical status changes.
Implications:
- High-dose methylprednisolone and hydroxychloroquine represent an effective therapeutic strategy for infantile chronic interstitial pneumonitis.
- Respiratory system compliance serves as a reliable indicator of treatment response and clinical status in affected infants.
Abstract:
Three infants with histologically confirmed chronic interstitial pneumonitis were treated with monthly intravenously administered high doses of methylprednisolone with or without daily hydroxychloroquine therapy. We applied the multiple occlusion technique to measure the static respiratory system compliance, and the end-inspiratory occlusion technique to measure passive respiratory system compliance, resistance, and time constant. When assessed by clinical criteria and pulmonary function measurements, all three patients showed improvement with this treatment. Clinical improvement was associated with an increase in respiratory system compliance as measured by both techniques (60% to 100% increase in all patients). The passive respiratory resistance and the time constant did not closely reflect the clinical course. We conclude (1) that high doses (pulses) of methylprednisolone and daily oral doses of hydroxychloroquine are effective in the treatment of infantile chronic interstitial pneumonitis and (2) that the respiratory system compliance, measured by both pulmonary function techniques, correlates well with the response to treatment and change in clinical status.