Idiopathic pulmonary haemosiderosis in childhood

Abstract

This report describes a case involving a boy in his middle childhood with a history of recurrent iron deficiency anaemia, accompanied by respiratory distress, tachypnoea and cyanosis. The respiratory distress improved with pulse doses of methylprednisolone and respiratory support using high-flow oxygen via heated humidified nasal cannula. During each admission, respiratory distress was resolved after administering pulse methylprednisolone. The child was admitted five times with such episodes in the paediatric intensive care unit (PICU). X-rays and high-resolution CT of the chest performed during respiratory distress showed diffuse ground-glass opacities and multiple areas of consolidation. After excluding sepsis, autoimmune diseases and vasculitis, a bronchoscopic alveolar lavage was conducted, revealing haemosiderin-laden macrophages. Having ruled out other causes of diffuse alveolar haemorrhage, this case was identified as idiopathic pulmonary haemosiderosis (IPH). Following discharge from the PICU and resolution of respiratory distress, the child was started on maintenance immunosuppression with prednisolone at 15 mg/day.

Keywords: Medical management; Paediatric intensive care; Pediatrics; Radiology; Respiratory system.

Figure 1. An X-ray of the chest of the child with idiopathic pulmonary haemosiderosis before giving methylprednisolone. There is diffuse opacification seen throughout both lung fields.

Figure 2. An X-ray of the chest of the child with idiopathic pulmonary haemosiderosis after giving methylprednisolone. X-ray shows clearance of opacities.

Figure 3. High-resolution CT (HRCT) chest (coronal section) of the child with idiopathic pulmonary haemosiderosis. HRCT scan reveals multiple areas of consolidation with areas of ground-glass opacities with superimposed interstitial septal thickening diffusely in bilateral lungs with few small mediastinal lymph nodes.

Figure 4. High-resolution CT (HRCT) of the chest (axial section) of the child with idiopathic pulmonary haemosiderosis. HRCT scan reveals multiple areas of consolidation with areas of ground-glass opacities with superimposed interstitial septal thickening diffusely in bilateral lungs with few small mediastinal lymph nodes.

Figure 5. high-resolution CT (HRCT) of the chest (sagittal section) of the child with idiopathic pulmonary haemosiderosis. HRCT scan reveals multiple areas of consolidation with areas of ground-glass opacities with superimposed interstitial septal thickening.

Figure 6. Bronchoalveolar lavage cytology slide with Leishman stain at ×40 magnification. Arrows point to the haemosiderin-laden macrophages.