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Long-term outcome of idiopathic pulmonary hemosiderosis in children
L Le Clainche1, M Le Bourgeois, B Fauroux
1Hôpital Necker-Enfants Malades, Paris, France.
Insights
Idiopathic pulmonary hemosiderosis (IPH) in children can lead to long-term survival, with 80% experiencing mild or no respiratory issues. Presence of antineutrophil cytoplasm antibodies (ANCA) indicates a poorer prognosis.
Area of Science:
- Pediatric Pulmonology
- Rheumatology
- Immunology
Background:
- Idiopathic pulmonary hemosiderosis (IPH) is a rare condition causing lung bleeding in children.
- Long-term outcomes and prognostic factors for pediatric IPH require further elucidation.
Purpose of the Study:
- To analyze the long-term outcomes of pediatric idiopathic pulmonary hemosiderosis.
- To identify potential predictors of prognosis in IPH patients.
Main Methods:
- Retrospective analysis of 15 pediatric patients diagnosed with IPH.
- Assessment of respiratory outcomes, immune disorders, and radiological findings.
- Correlation of clinical and biological data with long-term survival.
Main Results:
- Mean age at IPH onset was 5 years, with a mean follow-up of 17.2 years.
- 80% of patients (12/15) experienced mild or no respiratory problems, maintaining a normal life.
- Four patients developed immune disorders (rheumatoid polyarthritis or celiac disease).
- Severe outcomes included pulmonary fibrosis (2 patients) and severe asthma (1 patient).
- Antineutrophil cytoplasm antibodies (ANCA) or other autoantibodies were associated with poor prognosis.
Conclusions:
- Long-term survival is achievable for children with idiopathic pulmonary hemosiderosis.
- The presence of ANCA or other autoantibodies is a significant negative prognostic factor.
- No other clinical or biological factors reliably predicted prolonged survival in this cohort.
Abstract:
We retrospectively analyzed the long-term outcome of idiopathic pulmonary hemosiderosis (IPH) in 15 children. IPH started at a mean age of 5 years, and the mean duration of follow-up was 17.2 years (range, 10-36 yr). Four patients developed immune disorders, 3 cases of rheumatoid polyarthritis or rheumatoid polyarthritis-like diseases and 1 case of celiac disease. Respiratory outcome showed that 3 patients had severe symptoms: 2 patients developed severe pulmonary fibrosis resulting in major chronic respiratory insufficiency, and 1 patient had severe asthma. Twelve patients (80%) had mild or no respiratory problems and were able to lead a normal life. According to chest X-ray and pulmonary function test data, 4 patients had normal chest X-ray and no evidence of restrictive syndrome, 6 patients had an interstitial pattern on chest X-ray and evidence of restrictive pattern, 1 patient had an interstitial pattern but normal lung function, and 1 patient had a normal chest X-ray but evidence of mixed obstructive and restrictive pattern. Our results show that long-term survival is possible in patients with IPH. Factors of poor prognosis seem to be the presence of antineutrophil cytoplasm antibodies (ANCA) or other autoantibodies. No other clinical or biological predictive factors for prolonged survival were found.