Idiopathic Pulmonary Hemosiderosis Presenting as Anemia and Respiratory Failure in an Adult

Abstract

SESSION TITLE: Miscellaneous SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Idiopathic Pulmonary Hemosiderosis (IPH) is an extremely rare disease, which primarily occurs in children1. Its usual presentation is associated with diffuse alveolar hemorrhage (DAH)2. We describe a case of a 35-year-old patient who presented with recurrent acute hypoxemic respiratory failure requiring intubation and anemia requiring blood transfusions. Extensive workup confirmed diagnosis of DAH secondary to IPH. CASE PRESENTATION: A 35-year-old Caucasian female and active smoker presented with acute hypoxemic respiratory failure/adult respiratory distress syndrome. The patient had similar presentations requiring intubation over the course of previous two years. Her etiology was thought to be secondary to an infectious process or smoking related interstitial lung disease and was responsive to steroid therapy. Autoimmune serology was unremarkable including a vasculitis panel. The patient was also anemic requiring blood transfusions but without hemoptysis. She underwent a bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsy which was consistent with DAH and hemosiderin laden macrophages. There was no evidence of vasculitis, capillaritis or infectious etiology. These findings were consistent with a diagnosis of IPH. She was started on IV Solumedrol and Imuran with excellent response to treatment. She remains stable without further respiratory compromise for the past 1 year. She is on maintenance prednisone and Imuran therapy. DISCUSSION: The etiology of IPH is unknown. It primarily presents in children and is rarely seen in adults1. When in adults, presentation is usually with hemoptysis and bilateral alveolar infiltrates. Anemia and dyspnea without hemoptysis is the common presentation in children. The etiology may be immunological causing a defect in the basement membrane of the capillary, which may explain the responsiveness to corticosteroids and immunosuppressive therapies2. Bronchoscopy with BAL showing hemosiderin-laden macrophages without any evidence of pulmonary vasculitis or capillaritis is diagnostic of the condition2. Patients respond to steroids and immunosuppressive therapies. CONCLUSIONS: IPH should be considered in any patient that presents with recurrent respiratory failure, anemia and negative vasculitis workup. Reference #1: Morgan PG, Turner-Warwick M. Pulmonary haemosiderosis and pulmonary haemorrhage. Br J Dis Chest. 1981;75(3):225. Reference #2: Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J. 2004;24(1):162. DISCLOSURE: The following authors have nothing to disclose: Waheeda Nazneen, Sanjay Dogra, Abdulgadir Adam, Asadulla Mohammed No Product/Research Disclosure Information