Idiopathic pulmonary hemosiderosis: clinical profile and follow up of 26 children

Abstract

Objectives: To describe the clinical details and follow up of children with idiopathic pulmonary hemosiderosis.

Design: Retrospective case series.

Setting: Pediatric chest clinic of a tertiary care hospital.

Subjects: Children diagnosed as suffering from idiopathic pulmonary hemosiderosis (IPH).

Methods: Charts of patients diagnosed as IPH were reviewed for clinical features and treatment regimen. Diagnosis was based on presence of iron deficiency anemia, chest radiography and demonstration of hemosiderin laden macrophages in bronchoalveolar lavage (BAL), gastric aspirate, or sputum. Treatment consisted of oral prednisolone, hydroxychloroquine (HCQ) and inhaled corticosteroids (ICS).

Results: The common clinical features in 26 children with IPH (mean age 75 months) included: cough, breathlessness, fever, hemoptysis and wheezing in 26 (100%), 22 (85%), 19 (73%),15 (58%) and 14 (54%) children, respectively. Clubbing, hepatomegaly and splenomegaly was seen in 16 (62%), 15 (58%) and 10 (38%) children, respectively. Hemosiderin laden macrophages were documented in BAL and gastric aspirate in 92% and 30% patients, respectively. Symptoms did not recur in 17 patients who received prednisolone and HCQ initially. 5 patients had recurrence of symptoms and required short courses of oral prednisolone, 4 patients required frequent courses of prednisolone and were started on azathioprine. Older age, longer duration of illness, history of hemoptysis and jaundice were associated with poor response.

Conclusion: Treatment with prednisolone and hydroxychloroquine followed by inhaled corticosteroids may improve survival in children with IPH.