Pediatric Sarcoidosis Treatment & Management

  • Author: Girish D Sharma, MD; Chief Editor: Michael R Bye, MD   more...
 
Updated: Dec 1, 2011
 

Approach Considerations

The goal of therapy in sarcoidosis is to prevent or minimize inflammation and granuloma formation (ie, disease activity) leading to organ system dysfunction, which may ultimately cause end-stage organ destruction by the development of hyaline fibrosis. Treatment is symptomatic and supportive.

Glucocorticoids remain the therapy of choice for children with multisystem involvement. However, because the disease clears spontaneously in approximately 50% of patients and because permanent organ dysfunction often does not improve with glucocorticoids, no clear consensus has been established among clinicians as to the criteria for treatment.

Some reports suggest that methotrexate (MTX) administered orally in low doses in childhood sarcoidosis is effective, safe, and exhibits steroid-sparing properties. Data regarding efficacy of other immunosuppressive agents, such as azathioprine, cyclophosphamide, chlorambucil, and cyclosporine in adult sarcoidosis, have been limited to anecdotal, uncontrolled reports.

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Consultations

Pulmonologist

Determining the extent and activity of the inflammatory process in the organs at greatest risk, such as the lung, is critical to make decisions regarding therapy in sarcoidosis. A complete medical history and physical examination in conjunction with chest radiography and pulmonary function tests are helpful in evaluating the pulmonary disease.

Ophthalmologist

Referral to a pediatric ophthalmologist is invaluable in suspected cases of childhood sarcoidosis, because eye lesions frequently occur. Decisions concerning therapy are based on slitlamp examination and tests for visual acuity.

Rheumatologist

All suspected cases of childhood sarcoidosis necessitate prompt referral to a pediatric rheumatologist for the initial diagnostic evaluation and subsequent management, including long-term follow-up.

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Contributor Information and Disclosures
Author

Girish D Sharma, MD  Associate Professor of Pediatrics, Rush Medical College; Director, Section of Pediatric Pulmonology and Rush Cystic Fibrosis Center, Rush University Medical Center

Girish D Sharma, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, and Royal College of Physicians of Ireland

Disclosure: Nothing to disclose.

Chief Editor

Michael R Bye, MD  Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

Charles Callahan, DO Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center

Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, and Christian Medical & Dental Society

Disclosure: Nothing to disclose.

Abraham Gedalia, MD Head, Division of Pediatric Rheumatology, Professor of Pediatrics, Louisiana State University Health Sciences Center, Children's Hospital of New Orleans

Abraham Gedalia, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Rheumatology

Disclosure: Nothing to disclose.

Susanna A McColley, MD Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Director of Cystic Fibrosis Center, Head, Division of Pulmonary Medicine, Children's Memorial Medical Center of Chicago

Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, and American Thoracic Society

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Honoraria Consulting; Boston Scientific Consulting fee Consulting; Gilead Honoraria Speaking and teaching; Caremark Consulting fee Consulting; Vertex Pharmaceuticals Honoraria Speaking and teaching

Avinash Shetty, MD Department of Pediatrics, Division of Pediatric Infectious Diseases, Assistant Professor of Pediatrics, Wake Forest University School of Medicine

Avinash Shetty, MD is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, Infectious Diseases Society of America, and Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

References

  1. Iannuzzi, MC, Rybicki BA, Teirstein AS. Sarcoidosis. NEJM. 2007;357:2153-65.

  2. Valetonyte R, Hampe J, Huse K et al. Sarcoidosis is associated with a truncating splice site mutation in BTNL2. Nat Genet. 2005;37:357-64.

  3. Rybicki BA, Walewski JL, Maliarik MJ, Kian H, Iannuzzi MC,. The BTNL2 gene and sarcoidosis susceptibility in African Americans and Whites. Am J Hum Genet. Sep 2005;77(3):491-9. [Medline].

  4. Braun T, Siedek V, Assmann G, et al. [Sarcoidosis of lymph nodes in the submandibular compartment.]. HNO. Jul 1 2009;[Medline].

  5. Ozdogan A, Acioglu E, Karaman E, Oz B, Musellim B. A difficult case: sarcoidosis of the middle ear. Am J Otolaryngol. Jul-Aug 2009;30(4):281-4. [Medline].

  6. de Boer JH, Sijssens KM, Smeekens AE, Rothova A. Keratitis and arthritis in children with sarcoidosis. Br J Ophthalmol. Jun 2009;93(6):835, 844. [Medline].

  7. Nilsson K, Pahlson C, Lukinius A, et al. Presence of Rickettsia helvetica in granulomatous tissue from patients with sarcoidosis. J Infect Dis. Apr 15 2002;185(8):1128-38. [Medline].

  8. Svendsen CB, Milman N, Nielsen HW et al. A prospective study evaluating the presence of Rickettsia in Danish patients with sarcoidosis. Scand J Infect Dis. 2009;14:1-8.

  9. Rossman MD, Thompson B, Frederick M, et al. HLA-DRB1*1101: a significant risk factor for sarcoidosis in blacks and whites. Am J Hum Genet. Oct 2003;73(4):720-35. [Medline]. [Full Text].

  10. Darlington P, Tallstedt L, Padyukov L, et al. HLA-DRB1* alleles and symptoms associated with Heerfordt's syndrome in sarcoidosis. Eur Respir J. Nov 2011;38(5):1151-7. [Medline].

  11. Milman N, Svendsen CB, Hoffmann AL. Health-related quality of life in adult survivors of childhood sarcoidosis. Respir Med. Jun 2009;103(6):913-8. [Medline].

  12. Marcille R, McCarthy M, Barton JW, et al. Long-term outcome of pediatric sarcoidosis with emphasis on pulmonary status. Chest. Nov 1992;102(5):1444-9. [Medline].

  13. Sharma, OP. Sarcoidosis. Dis Mon. 1990;36:469-535.

  14. Hetherington S. Sarcoidosis in young children. Am J Dis Child. Jan 1982;136(1):13-5. [Medline].

  15. Ukae S, Tsutsumi H, Adachi N, Takahashi H, Kato F, Chiba S. Preschool sarcoidosis manifesting as juvenile rheumatoid arthritis: a case report and a review of the literature of Japanese cases. Acta Paediatr Jpn. Oct 1994;36(5):515-8. [Medline].

  16. [Guideline] Society of Nuclear Medicine. Procedure guideline for gallium scintigraphy in inflammation. Society of Nuclear Medicine. Jun 2004.

  17. Harada T, Nabeshima K, Matsumoto T, Akagi T, Fujita M, Watanabe K. Histological findings of the computed tomography halo in pulmonary sarcoidosis. Eur Respir J. Jul 2009;34(1):281-3. [Medline].

  18. Heyer CM, Lemburg SP, Kagel T, Mueller KM, Nuesslein TG, Rieger CH, et al. Evaluation of chronic infectious interstitial pulmonary disease in children by low-dose CT-guided transthoracic lung biopsy. Eur Radiol. Jul 2005;15(7):1289-95. [Medline].

  19. Lower EE, Baughman RP. The use of low dose methotrexate in refractory sarcoidosis. Am J Med Sci. Mar 1990;299(3):153-7. [Medline].

  20. Gedalia A, Molina JF, Ellis GS Jr, et al. Low-dose methotrexate therapy for childhood sarcoidosis. J Pediatr. Jan 1997;130(1):25-9. [Medline].

  21. Alhamad EH. The six-minute walk test in patients with pulmonary sarcoidosis. Ann Thorac Med. Apr 2009;4(2):60-4. [Medline].

 
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Chest radiograph showing bilateral hilar lymphadenopathy in a 10-year-old girl with sarcoidosis.
Chest radiograph showing patchy diffuse pulmonary infiltrates involving both lung fields in a 12-year-old girl at onset of her sarcoidosis (left). A repeated study 6 months later showing almost complete resolution of the infiltrates (right).
 
 
 
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