SECONDARY SPONTANEOUS PNEUMOTHORAX IN IDIOPATHIC PULMONARY HEMOSIDEROSIS

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: IPH is a rare disease that causes recurrent episodes of diffuse alveolar hemorrhage, most commonly presenting in children[1]. The classic triad includes iron deficiency anemia, hemoptysis, and pulmonary infiltrates; however, the initial symptom often only anemia[1-2]. This case describes an unusual complication IPH. CASE PRESENTATION: A 20-year-old African American female with history presented acute onset left-sided chest pain mild shortness breath. She had no other associated symptoms. was diagnosed at 15-months-old. previously been on azathioprine hydroxychloroquine for many years, however stopped taking them about year prior to presentation. frequent cough, fevers when she young, over past 10 years has very minimal respiratory currently medications. Physical exam significant absent lung sounds left.Chest X-ray showed peripherally predominant fibrosis apical subpleural cysts, large pneumothorax 3.4 centimeters pleural separation. Atelectasis left infrahilar region noted without evidence mediastinal shift.She placed oxygen tube inserted. Repeat significantly decreased size. High resolution CT peripheral extensive upper bilateral perihilar peribronchovascular coarse linear opacities, bibasilar dependent opacities. Due VATS pleurodesis offered due high risk recurrence. patient ultimately declined procedure. removed repeat pneumothorax. followed up pulmonology clinic restarted azathioprine. DISCUSSION: presents unique disease. With intrapulmonary can progress development increasing pneumonthoraces. Previous studies have found common complications include severe chronic progressive fibrosis[1], hemorrhagic flares[2]. To our knowledge, there are two documented patients spontaneous setting underlying IPH[2,3]. Despite apparent remission, continued progression hemorrhage or until later CONCLUSIONS: While this presentation, it demonstrates may appear clinically silent while continue causing future sometimes complications. REFERENCE #1: LaFreniere K, Gupta V. Idiopathic Pulmonary Hemosiderosis. 2021 Mar 3. In: StatPearls [Internet]. Treasure Island (FL): Publishing; Jan–. PMID: 32644388. #2: Gencer M, Ceylan E, Bitiren Koc A. Two sisters idiopathic hemosiderosis. Can Respir J. 2007 Nov-Dec;14(8):490-3. doi: 10.1155/2007/150926. 18060095; PMCID: PMC2677775. #3: NICKOL KH. haemosiderosis Tubercle. 1960 Jun;41:216-8. 10.1016/s0041-3879(60)80082-7. 14426939. DISCLOSURES: No relevant relationships by John Cox, source=Web Response Ashley Reluzco,