The retrospective review of case-records involved seven patients (4 boys) with median age of 26 months, diagnosed between January 2011 to September 2014 at Institute of Child Health, Kolkata. The presentations were variable with symptoms like pallor (n=3), poor weight gain (n=4), cough and respiratory distress during acute bleeds (n=3), and unexplained iron deficiency anemia (n=7). Six children had bilateral patchy infiltrates on Chest X-ray. Diagnosis was confirmed by detection of hemosiderin-laden macrophages in bronchoalveolar lavage (BAL) in 4 children, and in gastric aspirate in 3 children; secondary causes of hemosiderosis were excluded. Anti-nuclear antibodies, Anti-nuclear cytoplasmic antibodies and Direct Coomb’s were negative in all the patients.

All patients were prescribed a milk-free diet, and were treated with oral prednisolone (1–1.5 mg/kg/day) and hydroxychloroquine (HCQ). One child needed pulse methylprednisolone at presentation because of inadequate response to oral steroids. As the first three patients had recurrence of pulmonary bleed on tapering steroids, they were treated with azathioprine, which was subsequently routinely prescribed after 2 to 4 weeks of initiation of treatment when gradual tapering of steroid was started. One child unresponsive to azathioprine was induced by intravenous monthly cyclophosphamide pulses for 6 months followed by azathioprine. On follow-up (average duration 3 years, 10 months), there was no recurrence. After remission for more than two years, azathioprine was gradually tapered off with continuation of hydroxychloroquine.

One of the limitations of current observations is that the diagnosis of IPH was not confirmed by lung biopsy However, in the presence of hemosiderin-laden macrophages in BAL or in gastric aspirate/sputum along with chronic pulmonary symptoms, a diagnosis of IPH can be made [2,3]. Small sample size and lack of a control group were other major limitations.

There are no evidence-based recommendations regarding the treatment of IPH [1,3-7]. In this series of patients, we used prednisolone for induction and maintained remission with early addition of HCQ plus azathioprine/cyclophosphamide. None of them showed any recurrence or any major side effect of immunosuppression.

Prognosis of pulmonary hemosiderosis seems to have improved over time. While two decades ago the mean survival was 3　years from diagnosis, recent data show 5-year survival in 86% of cases [8]. The significant improvement is possibly due to the early initiation and long-term use of immunosuppressive therapy.

Contributors: PP: conceptualized the study. All authors were involved in patient management and data collection. All five authors have contributed to drafting of manuscript.

Funding: None; Competing interest: None stated.

1. Taytard J, Nathan N, Blic J, Fayon M, Epaud R, Deschildre A, et al. New insights into pediatric idiopathic pulmonary hemosiderosis: The French RespiRare cohort. Orphanet J Rare Dis. 2013;8:161.

2. Nuesslein TG, Teig N, Rieger CH. Pulmonary haemosiderosis in infants and children. Pediatr Respir Rev. 2006;7:45-8.

3. Kabra SK, Bhargava S, Lodha R, Satyavani A, Walia M. Idiopathic pulmonary hemosiderosis: Clinical profile and follow up of 26 children. Indian Pediatr. 2007;44:333-8.

4. Bulucea C, Sorin D. Idiopathic pulmonary hemosiderosis in children: A Romanian experience. Pediatrics. 2008;121:158-9.

5. Le Clainche L, Le Bourgeois M, Fauroux B, Forenza N, Dommergues JP, Desbois JC, et al. Long term outcome of idiopathic pulmonary hemosiderosis in children. Medicine. 2000;79:318-26.

6. Saeed MM, Woo MS, MacLaughlin EF, Margetis MF, Keens TG. Prognosis in pediatric idiopathic pulmonary hemosiderosis. Chest. 1999;116:721-5.

7. Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J. 2004;24:162-70.