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Chest ; 162(4):A1810, 2022.
Article in English | EMBASE | ID: covidwho-2060868


SESSION TITLE: Diagnosis of Lung Disease through Pathology Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Idiopathic pulmonary hemosiderosis (IPH) is a rare pulmonary disease often resulting in diffuse pulmonary fibrosis. The majority of diagnoses present in infanthood with limited studies demonstrating late onset disease in patients older than 30 years. The mainstay of treatment is immunosuppressive therapy including systemic corticosteroids. Here we present a unique case of IPH in an unvaccinated individual with COVID-19 pneumonia. CASE PRESENTATION: Our patient was a 31 year-old male with a history of IPH diagnosed in early childhood with past hospitalizations for DAH and progressive pulmonary fibrosis for which he was treated with corticosteroids and cyclophosphamide years prior to this admission. He presented with six days of progressive shortness of breath and respiratory distress. He tested positive for COVID-19 four days prior to presentation. He was unvaccinated for COVID-19. Initial oxygen saturation was found to be 56% and non-invasive mechanical ventilation was started. CT angiography of the chest revealed diffuse ground glass opacities, bilateral consolidative changes, and redemonstration of pulmonary fibrosis with extensive honeycombing. Lab results were remarkable for elevated inflammatory enzymes including ferritin 1,335 ng/mL, lactate dehydrogenase 1,369 units/L, and C-reactive protein 6.5 ml/dL. Patient was started on intravenous glucocorticoids, IL-6 inhibitor, remdesivir. Work up for bacterial superinfection was unremarkable. His hospitalization was complicated by acute kidney injury, elevated liver enzymes, and anxiety. Despite the immunosuppressive therapy, the patient continued to have refractory hypoxemia. Due to his persistent hypoxemia, the family was contacted regarding the impending need for endotracheal intubation. They ultimately declined and the patient succumbed to his respiratory failure. DISCUSSION: Idiopathic pulmonary hemosiderosis remains to be a largely unstudied and rare disease with catastrophic respiratory sequela. There remains a scarcity of evidence surrounding the most effective treatment of these patients, although limited studies have shown mortality benefit with immunosuppressive therapy. In patients with IPH an insult such as COVID-19 infection could prove fatal. Preventative measures such as vaccination is vital in the protection of these patients. Further research regarding pathogenesis and treatment mechanisms for IPH is an aim of future study. CONCLUSIONS: Idiopathic Pulmonary Hemosiderosis is a rare but deadly disease often complicated by diffuse alveolar hemorrhage and pulmonary fibrosis. Considering the underlying pulmonary compromise in these patients, secondary insult from infection can have catastrophic outcomes. Reference #1: Saha B. K. (2021). Idiopathic pulmonary hemosiderosis: A state of the art review. Respiratory medicine, 176, 106234. Reference #2: Ioachimescu, O. C., Sieber, S., & Kotch, A. (2004). Idiopathic pulmonary haemosiderosis revisited. The European respiratory journal, 24(1), 162–170. Reference #3: Thornton, G. & Alotaibi, M. (2016). 979: IDIOPATHIC PULMONARY HEMOSIDEROSIS IN ADULT PATIENTS: AN EPIDEMIOLOGIC ANALYSIS. Critical Care Medicine, 44 (12), 321-321. doi: 10.1097/01.ccm.0000509655.03624.6e. DISCLOSURES: No relevant relationships by Allison Kunze No relevant relationships by Mohammed Siddiqui