PT - JOURNAL ARTICLE AU - Mina Gharibzadeh Hizal AU - Sanem Eryilmaz Polat AU - Tugba Ramasli Gursoy AU - Dilber Ademhan Tural AU - Beste Ozesen AU - Nagehan Emiralioglu AU - Sevgi Pekcan AU - Ayse Tana Aslan AU - Ebru Yalcin AU - Deniz Dogru AU - Ugur Ozcelik AU - Nural Kiper TI - Idiopathic Pulmonary Haemosiderosis; Factors That Effect Prognosis AID - 10.1183/13993003.congress-2020.5290 DP - 2020 Sep 07 TA - European Respiratory Journal PG - 5290 VI - 56 IP - suppl 64 4099 - http://erj.ersjournals.com/content/56/suppl_64/5290.short 4100 - http://erj.ersjournals.com/content/56/suppl_64/5290.full SO - Eur Respir J2020 Sep 07; 56 AB - Introduction: Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of alveolar hemorrhage in children with unknown underlying pathophysiology. The prognosis of the disease is extremely different between patients. There is limited study evaluated prognostic factors in IPH.Methods: We performed a multi-center, retrospective study in three tertiary care hospital between 1979-2019.Results: A total of 54 children included to the study. The mean age of onset of symptoms was 62.72 ± 45.8 months. The mean age at diagnosis was 80,2 months. The average time from onset to diagnosis was 18 months. The mean follow-up period was 56.7 months (0.75months to 27years).Patients are categorized into three groups according to treatment response; good, partial, and poor. Thirty-seven patients with long term follow-up or current prognosis information present were included in this grouping. Six of the patients were in a good response group, 18 of the patients were in partial response groups, and 13 of the patients were in the poor response group. There was no significant difference in terms of the age of diagnosis, sex, onset time of symptoms, consequently, initial signs, radiologic findings, and PFT. There was a significant relationship between the presence of hypoxia, transfusion history, ANA positivity, and elevated liver enzyme at the time of initial evaluation and treatment response.Conclusions: We evaluate a large pediatric cohort who were under follow-up for a long time. To the best of our knowledge, this association of hypoxia and ANA positivity and poor prognosis never been reported. This information would be useful for clinicians to decide treatment strategies.FootnotesCite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 5290.This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).