eMedicine Specialties > Pulmonology > Interstitial Lung Diseases

Eosinophilic Granuloma (Histiocytosis X): Treatment & Medication

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

Treatment

Medical Care

Smoking cessation is the most important medical intervention. Smoking cessation often stabilizes the disease and sometimes leads to regression. It is also helpful in preventing bronchogenic carcinoma. Largely because of the rarity of PLCH, well-designed, prospective, randomized data regarding therapy are lacking. Treatment considerations are as follows:

  • The use of corticosteroids is controversial. Corticosteroids may be considered in patients with a persistence of clinically significant pulmonary or constitutional symptoms or those with documented progression of disease. Corticosteroid therapy is not indicated in patients with normal lung function. Recommendations for the use of corticosteroids are based largely on retrospective data and expert opinion.
  • Investigational therapies include interleukin-2 (IL-2) and anti–tumor necrosis factor-alpha (anti–TNF-alpha). Both agents have been reported to improve outcomes in pediatric disseminated histiocytosis. This finding may lead to the investigation of their use in adult PLCH.
  • Useful adjunctive therapies include the following:
    • Supplemental oxygen therapy for those with clinically significant hypoxemia (SaO2 <89% or PaO2 <55 mmHg) at rest or with exertion
    • Aggressive treatment for pulmonary infections with prompt initiation of antibiotic therapy
    • Bronchodilator therapy in the presence of an obstructive ventilatory defect

Surgical Care

Lung transplantation is an option for select patients with advanced disease. Recurrence of PLCH has been reported in the transplanted lung.7

Consultations

Refer patients with suspected PLCH to a pulmonary disease specialist.

Activity

Exercise and pulmonary rehabilitation are encouraged. These activities may improve the patient's functional status, even if they have no effect on disease progression.

Medication

The mainstays of treatment for PLCH are smoking cessation and supportive therapy. The use of corticosteroids in the treatment of PLCH is controversial. Their efficacy has not been proven in well-designed, prospective, randomized, controlled trials. Some experts recommend a trial of corticosteroids for those patients with persistent symptomatic disease or evidence of progression.

Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.


Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Used as immunosuppressant to treat autoimmune disorders. By reversing increased capillary permeability and suppressing activity of polymorphonuclear cells, may decrease inflammation. Oral corticosteroid with relatively limited mineralocorticoid activity. Best prescribed in consultation with pulmonary disease specialist.

Adult

0.5-1.0 mg/kg/d PO initially, followed by 6- to 12-mo taper

Pediatric

Not established

Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective-tissue infections, and fungal or tubercular skin infections; GI bleeding or ulceration

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

More on Eosinophilic Granuloma (Histiocytosis X)

Overview: Eosinophilic Granuloma (Histiocytosis X)
Differential Diagnoses & Workup: Eosinophilic Granuloma (Histiocytosis X)
Treatment & Medication: Eosinophilic Granuloma (Histiocytosis X)
Follow-up: Eosinophilic Granuloma (Histiocytosis X)
Multimedia: Eosinophilic Granuloma (Histiocytosis X)
References
Further Reading
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References

  1. Gaensler EA, Carrington CB. Open biopsy for chronic diffuse infiltrative lung disease: clinical, roentgenographic, and physiological correlations in 502 patients. Ann Thorac Surg. Nov 1980;30(5):411-26. [Medline].

  2. Colby TV, Lombard C. Histiocytosis X in the lung. Hum Pathol. Oct 1983;14(10):847-56. [Medline].

  3. Thomeer M, Demedts M, Vandeurzen K; VRGT Working Group on Interstitial Lung Diseases. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. Acta Clin Belg. May-Jun 2001;56(3):163-72. [Medline].

  4. Watanabe R, Tatsumi K, Hashimoto S, Tamakoshi A, Kuriyama T,. Clinico-epidemiological features of pulmonary histiocytosis X. Intern Med. Oct 2001;40(10):998-1003. [Medline].

  5. Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med. Feb 14 2002;346(7):484-90. [Medline].

  6. Delobbe A, Durieu J, Duhamel A, Wallaert B. Determinants of survival in pulmonary Langerhans' cell granulomatosis (histiocytosis X). Groupe d'Etude en Pathologie Interstitielle de la Société de Pathologie Thoracique du Nord. Eur Respir J. Oct 1996;9(10):2002-6. [Medline].

  7. Alalawi R, Whelan T, Bajwa RS, Hodges TN. Lung transplantation and interstitial lung disease. Curr Opin Pulm Med. Sep 2005;11(5):461-6. [Medline].

  8. Aricò M. Langerhans cell histiocytosis in adults: more questions than answers?. Eur J Cancer. Jul 2004;40(10):1467-73. [Medline].

  9. Chaowalit N, Pellikka PA, Decker PA, Aubry MC, Krowka MJ, Ryu JH, et al. Echocardiographic and clinical characteristics of pulmonary hypertension complicating pulmonary Langerhans cell histiocytosis. Mayo Clin Proc. Oct 2004;79(10):1269-75. [Medline].

  10. Crausman RS, Jennings CA, Tuder RM, Ackerson LM, Irvin CG, King TE Jr. Pulmonary histiocytosis X: pulmonary function and exercise pathophysiology. Am J Respir Crit Care Med. Jan 1996;153(1):426-35. [Medline].

  11. Dacic S, Trusky C, Bakker A, Finkelstein SD, Yousem SA. Genotypic analysis of pulmonary Langerhans cell histiocytosis. Hum Pathol. Dec 2003;34(12):1345-9. [Medline].

  12. King TE, Crausman RS. Pulmonary histiocytosis X. In: Fishman AP, ed. Fishman's Pulmonary Diseases and Disorders. Vol 1. 3rd ed. New York, NY: Mc-Graw Hill; 1998:1163-70.

  13. Sundar KM, Gosselin MV, Chung HL, Cahill BC. Pulmonary Langerhans cell histiocytosis: emerging concepts in pathobiology, radiology, and clinical evolution of disease. Chest. May 2003;123(5):1673-83. [Medline].

  14. Tazi A. Adult pulmonary Langerhans' cell histiocytosis. Eur Respir J. Jun 2006;27(6):1272-85. [Medline].

  15. Vassallo R, Ryu JH. Pulmonary Langerhans' cell histiocytosis. Clin Chest Med. Sep 2004;25(3):561-71, vii. [Medline].

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Further Reading

See References section.

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Keywords

eosinophilic granulomatosis, EG, pulmonary histiocytosis X, PHX, pulmonary Langerhans cell histiocytosis, PLCH, pulmonary histiocytosis, histiocytosis, cigarette smoking, end-stage fibrotic lung disease, fibrotic lung disease

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Contributor Information and Disclosures

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
Eleanor M Summerhill, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Thoracic Society, Association of Program Directors in Internal Medicine, and Rhode Island Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Michael Peterson, MD, Chief of Medicine, Vice-Chair of Medicine, University of California at San Francisco; Endowed Professor of Medicine, University of California at San Francisco-Fresno
Michael Peterson, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Daniel R Ouellette, MD, FCCP, Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System
Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society
Disclosure: Boehringer Ingleheim Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

 
 
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