SESSION TITLE: Miscellaneous Cases II
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 01:15 PM - 02:45 PM
INTRODUCTION: Idiopathic Pulmonary Hemosiderosis (IPH) is a very rare cause of diffuse alveolar hemorrhage (DAH) usually seen in children. (1,2,3.) The clinical triad of hemoptysis, pulmonary infiltrates and iron deficiency anemia (IDA) without another identified cause and lung biopsy excluding other causes of pulmonary hemorrhage is considered diagnostic. (1,2,3.) IDA from IPH is unusual with a handful of reports occurring primarily in children. We report an adult case of IPH as the sole identified cause of IDA.
CASE PRESENTATION: A 58 year old African American man with IDA was referred for seven months of chronic cough and hemoptysis described as daily blood streaked sputum with occasional small blood clots. EGD and colonscopy were negative. He remained symptomatic despite several courses of antibiotics and had persistent symptomatic IDA, twice requiring blood transfusions and intravenous iron. A chest radiograph showed bilateral lower lobe infiltrates and a chest CT revealed multi-focal, patchy, ground glass opacities predominately at the bases. Endoluminal blood streaked secretions were noted on fiberoptic bronchoscopy with numerous alveolar and interstitial hemosiderophages on transbronchial biopsies without evidence of vasculitis or immune complexes. This was consistent with IPH and subsequently confirmed on open lung biopsy. Serologies including ANCA and Anti-GBM were negative and he was started on Prednisone.
DISCUSSION: IPH is a rare condition that can be difficult to diagnose. First described by Virchow in 1864, most cases occur in children with up to 20% diagnosed in adults before the age of 30. (1,2,3.) The disease course is severe and rapid in children. However, prolonged survival has been observed in adults. (1,2.) IDA in IPH is typically mild and rarely requires blood transfusions as in our case. The pathogenesis of IPH is unknown, but several reports document a response to corticosteroids and an association with celiac disease with resolution of symptoms after starting a gluten free diet. (1,2.) This infers an immunologic cause, however it is not evident on histologic examinations.
CONCLUSIONS: Patients with hemoptysis, diffuse alveolar infiltrates and unexplained IDA should raise suspicion for IPH. Diagnosis requires exclusion of other causes of DAH. Based on small case series of IPH, corticosteroids are the foundation of treatment. (1,2,3.) Most respond favorably to Prednisone at a dose of 1 mg/kg/day, tapering over a few months. (1)
Reference #1: Ioachimescu OC, Sieber S, Kotch. Idiopathic pulmonary hemosiderosis revisited. European Respiratory Journal 2004; 24:162-170.
Reference #2: Milman N, Pedersen FM. Idiopathic pulmonary haemosiderosis: Epidemioloyg, pathogenic aspects and diagnosis. Respiratory Medicine 1998; 92: 902-7
Reference #3: Miwa S, Imokawa S, Kato M, Ide K, Uchiyama H, Yokomura K, Suda T, Shirai M,Hayakawa H, Chida K. Prognosis in adult patients with idiopathic pulmonary hemosiderosis. Internal Medicine 2011;50(17):1803-8
DISCLOSURE: The following authors have nothing to disclose: Jeffrey Chen, Antonio Bonet, Scott Oh, G. Pezeshkpour, Guy Soo Hoo
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