Idiopathic pulmonary haemosiderosis with mineralizing pulmonary elastosis: A case report

The case presented here is of an adult man with idiopathic pulmonary haemosiderosis with mineralizing pulmonary elastosis.Pathologists are generally not familiar with this histologic reaction pattern associated with iron encrustation of pulmonary elastic tissue.Diffuse pulmonary haemosiderosis is characterized by repeated episodes of intra-alveolar haemorrhage leading to abnormal accumulation of iron as haemosiderin in alveolar macrophages with subsequent pulmonary fibrosis and severe anaemia [1,2]. Pulmonary haemosiderosis (PH) occurs either primarily as a disease of the lungs or secondary to systemic diseases. Idiopathic PH, first described by Ceelen in 1931, is characterized by a clinical triad of haemoptysis, anaemia and pulmonary infiltrates. Eighty percent of cases of PH occur in children [2]. We report a case of an adult man with idiopathic PH with mineralizing pulmonary elastosis.A 32-year-old, non-smoking, male farmer presented with a history of fever, intermittent episodes of mild haemoptysis and cough with expectoration for the previous six months. There was associated loss of appetite and loss of weight. He started experiencing respiratory distress 8 days prior to hospital admission. Two years earlier he was diagnosed with pulmonary tuberculosis based on X-ray findings whereupon he was commenced on anti-tubercular treatment, however the level compliance is not known. On physical examination his respiratory rate and jugular venous pressure were elevated, and he had clubbing. On chest auscultation there were bilateral coarse crepitations. Pulmonary function tests were consistent with restrictive ventilatory defect. Laboratory investigations revealed iron deficiency anaemia and there were negative results for antinuclear antibodies (ANA) and antineutrophilic cytoplasmic antibodies (ANCA). On ultrasound examination of the abdomen there was evidence of hepatosplenomegaly. The clinical impression was of disseminated tuberculosis. The patient was placed on ambu