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Idiopathic Pulmonary Hemosiderosis as a Mimic of Pulmonary Vasculitis: A Case Report and Review of the Literature

  • Autoimmunity (TK Tarrant, Section Editor)
  • Published:
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Abstract

Purpose of Review

Idiopathic pulmonary hemosiderosis (IPH) is one of the rarest and least understood causes of pulmonary hemorrhage in children. Illustrated by a complex case presentation, we discuss the clinical manifestations, diagnosis, pathology, proposed etiologies, and treatment of this rare disease. We also compare IPH with anti-glomerular basement membrane antibody syndrome (anti-GBM disease), another rare causes of pediatric pulmonary hemorrhage.

Recent Findings

Recent retrospective studies regarding IPH along with advanced immunotherapy have led to an improved understanding of how to best treat this condition, potential associations, and improved prognosis. Pathogenesis remains unknown, but several reports have suggested involvement of the alveolar capillary basement membrane.

Summary

IPH is a poorly understood disease of unknown etiology that is a diagnosis of exclusion. Our patient was diagnosed with IPH after an exhaustive workup, including lung biopsy, into other immune-mediated causes of disease. While the pathogenesis of this rare disease remains elusive, our patient’s immunofluorescent staining along the alveolar basement membrane without evidence of circulating antibody to type IV collagen raises the question of an immune-mediated pathogenesis of the disease with involvement of the alveolar basement membrane.

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Acknowledgments

We would like to acknowledge Carolyn Glass, MD, PhD and David Howell, MD, PhD from the Duke University Department of Pathology for the provision of pathology images and assistance in interpretation of the images.

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  1. Duke Pediatric Rheumatology, Box 3212 2301 Erwin Rd., Durham, NC, 27705, USA

    Mary Buckley & Heather Van Mater

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  1. Mary Buckley
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Contributions

The literature search, data analysis, and draft of this article were completed by Mary Buckley. Heather Van Mater critically revised the article and contributed to the concept of the article.

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Correspondence to Mary Buckley.

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Buckley, M., Van Mater, H. Idiopathic Pulmonary Hemosiderosis as a Mimic of Pulmonary Vasculitis: A Case Report and Review of the Literature. Curr Allergy Asthma Rep 20, 13 (2020). https://doi.org/10.1007/s11882-020-00907-7

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