Keywords

Erdheim-Chester disease, Histiocytosis, Idiopathic pulmonary fibrosis

 

Authors

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

Abstract

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.