What is included in the long-term monitoring of patients 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

In the care of patients with pulmonary Langerhans cell histiocytosis X (PLCH) , important considerations include the patients' smoking history and current smoking status, the presence or absence of extrapulmonary disease and constitutional symptoms, and close monitoring for progression of pulmonary disease.

Pulmonary artery hypertension is a known complication of infiltrative lung diseases, and in PLCH the magnitude of pulmonary artery hypertension may be greater than expected for given the degree of hypoxemia or level of impairment on pulmonary function testing.

The increased risk of pulmonary malignancies must be considered.

Smoking cessation counseling and adjunctive pharmacologic therapy with bupropion and nicotine replacement are key components of long-term management.

Perform pulmonary function testing and radiographic studies every 3-6 months, as the patient's clinical condition warrants.

Assess arterial oxygen saturation both at rest and with activity.

Echocardiography should be considered in all patients with clinically significant dyspnea in order to screen for pulmonary artery hypertension. If echocardiographic results suggest moderate-to-severe pulmonary artery hypertension, these findings should be further evaluated and confirmed with right-heart catheterization. At the time of catheterization, the response to vasodilators may also be assessed.

Patients should be vaccinated annually for influenza and should also receive the pneumococcal vaccine.


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