IDIOPATHIC PULMONARY HEMOSIDEROSIS PROGRESSING TO INTERSTITIAL LUNG DISEASE

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder first described in 1931, the incidence of which is around 0.24 to 1.23 per million. Pulmonary hemosiderosis can occur as a primary disease of the lung or can be secondary to systemic disease. The classical triad of IPH includes anemia, hemoptysis and pulmonary infiltrates, of which anyone could be the initial presentation. In some IPH may progress to interstitial lung disease. Interstitial lung disease (ILD) is a broad group of lung diseases comprising of both idiopathic and secondary causes such as IPH. Interstitial lung disease is characterized by inflammation and or fibrosis of the lungs. A systematic approach including a high index of suspicion, a thorough clinical evaluation, HRCT and BAL may suffice to make an early diagnosis of ILD. In a child with secondary ILD early diagnosis and appropriate treatment with immunosuppressive drugs will result in long term survival.