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Erdheim-Chester disease, Histiocytosis, Idiopathic pulmonary fibrosis



  1. Andrysek, Kevin MSN, ACNP-C, CCRN


Diagnosis and treatment of patients who present with respiratory compromise are challenging. What happens when these patients do not respond to your intervention, and their condition declines rapidly? Having a variety of differential diagnoses is key. An addition to your differential list can include a rare disorder of non-Langerhans cells histiocytosis also known as Erdheim-Chester disease. This disease often presents as an interstitial lung disease that fails many different treatment modalities. A full understanding of how this disease process works is still being investigated. Provided are a literature review and case study for better understanding of this disease.