A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis
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RESEARCH
Open Access
A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis Chana I.C. Chin1†, Shirleen Loloyan Kohn1*†, Thomas G. Keens1,2, Monique F. Margetis1,2 and Roberta M. Kato1,2
Abstract Background: Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unknown etiology characterized by chronic pulmonary hemorrhage and presents with a triad of anemia, hemoptysis and pulmonary infiltrates. IPH is a diagnosis of exclusion with a variable and disparate clinical course. Despite existing therapies, few children achieve full remission while others have recurrent hemorrhage, progressive lung damage, and premature death. Methods: We surveyed physicians who care for patients with IPH via a web-based survey to assess the most common practices. 88 providers responded, caring for 274 IPH patients from five continents. Results: 63.3 % of respondents had patients that were initially misdiagnosed with anemia (60.0 %) or gastrointestinal bleed (18.2 %). Respondents varied in diagnostic tools used for evaluation. The key difference was in the use of lung biopsy (51.9 %) for diagnosis. Common medications respondents used for treatment at initial presentation and chronic maintenance therapy were corticosteroids (98.7 and 84.0 %, initial and chronic therapy respectively), hydroxychloroquine (33.3 and 64.0 %), azathioprine (8.0 and 37.3 %), and cyclophosphamide (4.0 and 16.0 %). There was agreement on the use of corticosteroids for exacerbation amongst all respondents. Reported deaths before adulthood occurred in 7.3 % of patients. We conclude that there were common features and specific variations in physician management of IPH. Respondents were divided on whether to perform lung biopsy for diagnosis. Conclusion: Despite the availability of various immunomodulators, corticosteroids remained the primary therapy. We speculate that the standardization of care for diffuse alveolar hemorrhage will improve patient outcomes. Keywords: Interstitial lung disease, Lung pathology, Pulmonary vascular disorders, Vasculitis, Idiopathic pulmonary hemosiderosis, Diffuse alveolar hemorrhage, Capilliritis, Lung biopsy
Background Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder characterized by diffuse alveolar hemorrhage. Its incidence is about 0.5–1 case per million [1, 2]. IPH classically presents with a triad of anemia, hemoptysis and diffuse pulmonary infiltrates. Some patients achieve full remission while others have recurrent hemorrhage, progressive lung damage and premature death [3]. IPH is more common in children than adults with patients often presenting prior to 10 years of age. However, the diagnosis can be delayed in young children who swallow * Correspondence: [email protected] † Equal contributors 1 Division of Pulmonology, Children’s Hospital Los Angeles, Los Angeles, California Full list of author information is available at the end of the article
their sputum and do not present with hemoptysis [3–7]. IPH is a diagnosis of exclusion and other etiologies such as au
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