Pediatric Mixed Connective Tissue Disease Treatment & Management

  • Author: Marisa S Klein-Gitelman, MD, MPH; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Sep 29, 2009
 

Medical Care

  • The most important tools in treatment of mixed connective tissue disease (MCTD) include tailoring the medical regimen, promptly attending to disease flare, and performing careful and frequent clinical and laboratory evaluations to test for new disease manifestations.
  • Some patients have physical findings and laboratory studies that more closely resemble lupus (particularly double-stranded DNA and hypocomplementemia), and treatment should be tailored to treat a lupuslike disease.
  • Strongly consider annual echocardiography, pulmonary function testing, and barium swallow.
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Surgical Care

  • No specific surgical care is required.
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Consultations

  • A rheumatologist should be an integral part of the medical care team supporting the patient with mixed connective tissue disease.
  • Other consultants depend on the organ systems involved and the disease severity.
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Diet

  • Diet restrictions are driven by medical therapy and disease manifestations. Prescribe a low-fat, calcium-sufficient diet with no added salt for patients receiving corticosteroids.
  • Recognize and evaluate nontraditional remedies along with traditional medications for safety and efficacy.
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Activity

  • Encourage the patient with mixed connective tissue disease to maintain a healthy lifestyle.
  • Limitations should occur only secondary to serious organ involvement that prevents performance of activities.
  • Advise patients with mixed connective tissue disease to avoid fatigue.
  • Advise patients with mixed connective tissue disease to avoid significant cold exposures or to dress accordingly to decrease Raynaud symptoms.
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Contributor Information and Disclosures
Author

Marisa S Klein-Gitelman, MD, MPH  Associate Professor of Pediatrics, Northwestern University Feinberg School of Medicine; Head, Division of Rheumatology, Children's Memorial Hospital

Marisa S Klein-Gitelman, MD, MPH is a member of the following medical societies: American Academy of Pediatrics and American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Barry L Myones, MD  Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital

Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Thomas JA Lehman, MD, FAAP, FACR  Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery

Thomas JA Lehman, MD, FAAP, FACR is a member of the following medical societies: PM American Allergy Society

Disclosure: Nothing to disclose.

Daniel Rauch, MD, FAAP  Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine

Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine

Disclosure: Baxter Honoraria Consulting

Chief Editor

Lawrence K Jung, MD  Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

  1. Sharp GC, Irvin WS, Tan EM, et al. Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). Am J Med. Feb 1972;52(2):148-59. [Medline].

  2. Alarcon-Segovia D, Villareal M. Classification and diagnostic criteria for mixed connective tissue disease. In: Kasukawa R, Sharp GC, eds. Mixed Connective Tissue Disease and Anti-nuclear Antibodies. 1987:33-40.

  3. Kasukawa R, Tojo T, Miyawaki S. Preliminary diagnostic criteria for classification of mixed connective tissue disease. In: Kasukawa R, Sharp GC, eds. Mixed Connective Tissue Disease and Anti-nuclear Antibodies. 1987:41-7.

  4. Mairesse N, Kahn MF, Appelboom T. Antibodies to the constitutive 73-kd heat shock protein: a new marker of mixed connective tissue disease?. Am J Med. Dec 1993;95(6):595-600. [Medline].

  5. Michels H. Course of mixed connective tissue disease in children. Ann Med. Oct 1997;29(5):359-64. [Medline].

  6. Tsai YY, Yang YH, Yu HH, Wang LC, Lee JH, Chiang BL. Fifteen-year experience of pediatric-onset mixed connective tissue disease. Clin Rheumatol. Sep 16 2009;[Medline].

  7. Sharp G. The origin of mixed connective tissue disease: a stimulus for autoimmune disease research. Lupus. 2009;18(12):1031-2. [Medline].

  8. Ingegnoli F, Zeni S, Gerloni V, Fantini F. Capillaroscopic observations in childhood rheumatic diseases and healthy controls. Clin Exp Rheumatol. Nov-Dec 2005;23(6):905-11. [Medline].

  9. Biro E, Szekanecz Z, Czirjak L, et al. Association of systemic and thyroid autoimmune diseases. Clin Rheumatol. Mar 2006;25(2):240-5. [Medline].

  10. Bodolay E, Szekanecz Z, Devenyi K, et al. Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD). Rheumatology (Oxford). May 2005;44(5):656-61. [Medline].

  11. Ito S, Nakamura T, Kurosawa R, Miyamae T, Imagawa T, Mori M. Glomerulonephritis in children with mixed connective tissue disease. Clin Nephrol. Sep 2006;66(3):160-5. [Medline].

  12. Mier R, Ansell B, Hall MA, et al. Long term follow-up of children with mixed connective tissue disease. Lupus. Jun 1996;5(3):221-6. [Medline].

  13. Mier RJ, Shishov M, Higgins GC, et al. Pediatric-onset mixed connective tissue disease. Rheum Dis Clin North Am. Aug 2005;31(3):483-96, vii. [Medline].

  14. Mier RJ, Shishov M, Higgins GC, Rennebohm RM, Wortmann DW, Jerath R. Pediatric-onset mixed connective tissue disease. Rheum Dis Clin North Am. Aug 2005;31(3):483-96, vii. [Medline].

  15. Singsen BH, Bernstein BH, Kornreich HK, et al. Mixed connective tissue disease in childhood. A clinical and serologic survey. J Pediatr. Jun 1977;90(6):893-900. [Medline].

  16. Tiddens HA, van der Net JJ, de Graeff-Meeder ER, et al. Juvenile-onset mixed connective tissue disease: longitudinal follow-up. J Pediatr. Feb 1993;122(2):191-7. [Medline].

 
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Raynaud phenomenon showing demarcation of color difference.
 
 
 
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