What is the mortality and morbidity associated with eosinophilic granuloma (histiocytosis X)?

Updated: Mar 29, 2018
  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

Pulmonary Langerhans cell histiocytosis X (PLCH) has a highly variable course. Some patients have spontaneous remissions, especially when they stop cigarette smoking, whereas others progress to end-stage fibrotic lung disease.

In  one retrospective study, median survival was 12.5 years after diagnosis. [7] A European study showed similar findings, with a median survival of 13 years. [8]

Factors associated with a poorer prognosis include multisystem involvement other than bone (including diabetes insipidus related to pituitary involvement), recurrent pneumothorax, severe pulmonary artery hypertension, older age at diagnosis, severe pulmonary function test abnormalities, and more widespread cystic changes on imaging studies.

Cigarette smoking is important to disease activity. Smoking worsens morbidity and mortality. Smoking cessation frequently stabilizes the disease and sometimes leads to its regression.


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