Recombinant interferon gamma lb and low dose steroid in two pediatric cases of nonspecific interstitial pneumonia
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Donato Rigante1, Angelica Bibiana Delogu, Gilda Federico
1Department of Pediatric Sciences, Università Cattolica Sacro Cuore, Policlinico A. Gemelli, Largo A. Gemelli, 8 00168 Rome, Italy. icple@rm.unicatt.it
Insights
Recombinant interferon gamma-1b did not stabilize or cure nonspecific interstitial pneumonia in two young girls unresponsive to steroids. This therapy showed no significant benefit in controlling this rare pediatric lung disease.
Area of Science:
- Pediatric Pulmonology
- Immunology
- Rare Diseases
Background:
- Nonspecific interstitial pneumonia (NSIP) is a rare lung disease.
- Steroids are a common treatment for NSIP.
- Limited treatment options exist for steroid-unresponsive pediatric NSIP.
Observation:
- Two young girls, aged 11 at diagnosis, presented with NSIP.
- Both patients showed insufficient response to low-dose steroid therapy.
- A 1-year trial of subcutaneous recombinant interferon gamma-1b with steroids was administered.
Findings:
- Interferon gamma-1b did not stabilize or cure NSIP in these patients.
- No significant improvement was observed in controlling or reversing the disease.
- The combination therapy failed to demonstrate therapeutic efficacy.
Implications:
- Recombinant interferon gamma-1b is not a recommended therapy for steroid-unresponsive pediatric NSIP.
- Further research is needed to identify effective treatments for this condition.
- This study highlights the challenges in managing rare pediatric interstitial lung diseases.
Abstract:
We report two young girls, the first almost 18 years old and the second 12 1/2 years old, affected with nonspecific interstitial pneumonia, both diagnosed at the age of 11 by open lung biopsy and both being treated with low-dose steroid. Due to insufficient response to conventional therapy and based on positive therapeutic results following the use of subcutaneous recombinant interferon gamma-1b in fibrosing interstitial pneumonias of adults, they were given a 1-year trial of subcutaneous recombinant interferon gamma-lb in association with a steroid. Our experience with these two young patients suggests that interferon gamma-lb cannot be considered as stabilizing or a curative therapy to control or reverse nonspecific interstitial pneumonia unresponsive to steroids alone.
