eMedicine Specialties > Pulmonology > Interstitial Lung Diseases

Eosinophilic Granuloma (Histiocytosis X): Follow-up

Author: Eleanor M Summerhill, MD, Assistant Professor of Medicine, Division of Pulmonary and Critical Care Medicine, Warren Alpert Medical School of Brown University; Director of Internal Medicine Residency Program, Memorial Hospital of Rhode Island
Contributor Information and Disclosures

Updated: Mar 3, 2008

Follow-up

Further Inpatient Care

• Inpatient admission is indicated only to manage complications related to the disease as listed below.
• Patients with superimposed respiratory infections, such as pneumonia, may require inpatient treatment.
• Patients with spontaneous pneumothorax may require chest-tube placement and subsequent in-patient care. The recurrence rate of secondary spontaneous pneumothorax in PLCH is high. Therefore, some experts recommend surgical intervention, such as mechanical pleurodesis, parietal pleurectomy, or talc insufflation, to prevent further occurrences after the initial episode.
• Acute respiratory failure necessitating in-patient management may occur as the result of a superimposed respiratory infection or spontaneous pneumothorax. Respiratory failure may also occur as a manifestation of end-stage disease.

Further Outpatient Care

• In the care of patients with 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.

Inpatient & Outpatient Medications

• As previously discussed, corticosteroid therapy may be considered in some patients.
• Patients with an obstructive ventilatory defect on pulmonary function testing may benefit from bronchodilator therapy.
• Supplemental oxygen therapy is indicated for all patients with either resting or exertional hypoxemia. Oxygen therapy may help to prevent or slow progression of pulmonary hypertension and cor pulmonale, and it provides a mortality benefit in chronic obstructive pulmonary disease.

Deterrence/Prevention

• Effective antismoking measures can prevent PLCH. See Medscape's Smoking Resource Center.

Complications

• Spontaneous pneumothorax is a common complication (10-20%).
• PLCH is associated with an increased risk of malignancy, including Hodgkin and non-Hodgkin lymphoma, myeloproliferative disorders, and bronchogenic carcinoma.
• Pathologic fracture may occur at the site of bone lesions.
• Diabetes insipidus occurs in 10-15% of patients and indicates disease in the central nervous system.
• Pulmonary artery hypertension and cor pulmonale may develop as a result of hypoxemia and/or vascular disruption due to PLCH lesions.

Prognosis

• Prognosis varies and is related to smoking cessation. Most patients who continue to smoke experience disease progression, but for those who successfully quit smoking, the disease often stabilizes or regresses.
• Retrospective studies suggest that the following factors are associated with adverse outcomes in PLCH:
  • Extremes of age
  • Extensive cysts and honeycombing radiographically
  • Prolonged corticosteroid therapy
  • Multiorgan involvement
  • Abnormal pulmonary function, including reduced gas exchange, as measured by diffusion capacity for carbon monoxide; obstructive ventilatory defect (reduced ratio of forced expiratory volume in 1 second (FEV₁) to forced vital capacity [FVC], or FEV₁/FVC); and/or evidence of airtrapping (high residual volume/total lung capacity)
• Young men who have diabetes insipidus have the worst prognosis.
• Favorable signs include the radiographic finding of sparing of the costophrenic angles and a cellular, nonfibrotic biopsy specimen.

Patient Education

• The public must be educated about the likely etiologic role of cigarette smoking. PLCH is thought to be largely preventable through smoking cessation.
• Instruct patients to promptly report the development of hemoptysis. This symptom may indicate malignancy or superimposed bacterial/fungal infection, such as Aspergillus species infection.

Miscellaneous

Medicolegal Pitfalls

• As with any uncommon medical condition, misdiagnosis or missed diagnosis can result in litigation.
• Furthermore, every treatment plan must include the clear recommendation for smoking cessation.
• Finally, given the near-universal association with cigarette smoking, tobacco company–related litigation may be an issue.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Robert S. Crausman, MD, MMS, to the development and writing of this article.

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