A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis.
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.


CONCLUSIONS
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.
Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. chana.chantaw@gmail.com.
Chana I C
CI
Chin
Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. sloloyan@chla.usc.edu.
Shirleen Loloyan
SL
Kohn
Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California.|Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, California.
Thomas G
TG
Keens
Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California.|Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, California.
Monique F
MF
Margetis
Division of Pulmonology, Children's Hospital Los Angeles, Los Angeles, California. rkato@chla.usc.edu.|Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, California. rkato@chla.usc.edu.
Roberta M
RM
Kato
D006486:Hemosiderosis / Q000628:therapy*; D006801:Humans; D008171:Lung Diseases / Q000628:therapy*; D010820:Physicians; D010818:Practice Patterns, Physicians'; D011795:Surveys and Questionnaires; D000097545:Hemosiderosis, Pulmonary