Pediatric Mixed Connective Tissue Disease Follow-up

  • Author: Marisa S Klein-Gitelman, MD, MPH; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Jan 13, 2015
 

Further Outpatient Care

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  • Observe the patient at regular intervals of 1-3 months depending on disease severity and manifestations. Obtain appropriate laboratory tests during these visits depending on disease manifestations and medication adverse effects.
  • Laboratory data may include lupus serology, renal evaluation, muscle enzymes, and hematologic evaluation.
  • Use physical or occupational therapy as needed for musculoskeletal symptoms.
  • Continue to monitor for early evidence of pulmonary hypertension, interstitial lung disease, esophageal dysmotility, and osteoporosis.
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Further Inpatient Care

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  • Admit patients with mixed connective tissue disease (MCTD) to the hospital for diagnostic evaluation or for chemotherapy as warranted. Most often, this is an outpatient evaluation.
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Inpatient & Outpatient Medications

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  • See Medical Care.
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Transfer

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  • Because patients with mixed connective tissue disease often have complicated medical issues, refer to a tertiary medical center for evaluation and treatment.
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Deterrence/Prevention

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  • No intervention to deter disease onset or to alter progression is known other than the medical management of disease manifestations as described and screening for new disease manifestations.
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Complications

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  • Complications of mixed connective tissue disease depend on the organ systems involved and the adverse effects and risks of immunosuppressive therapy.
  • Patients with mixed connective tissue disease are at risk for infections, cardiovascular disease, and complications observed in lupus, progressive systemic sclerosis, and myositis (see Pathophysiology).
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Prognosis

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  • Prognosis is generally considered similar to that of pediatric lupus. Initial descriptions of mixed connective tissue disease did not include renal disease, and the prognosis was believed to be considerably better than for the major connective tissue diseases. However, patients who fit the criteria for mixed connective tissue disease have had renal disease and considerable morbidity and mortality from major organ manifestations. It appears that, in mixed connective tissue disease, children fare better than adults.
  • Individual patients appear to have severe or mild disease courses.
  • Prognosis also depends on which disease manifestations are more prominent (eg, myocarditis, pulmonary disease, renal disease).
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Patient Education

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  • Patient and family must have a thorough understanding of the disease, potential severity, and complications from the disease and therapy. Treatment of the individual with mixed connective tissue disease is difficult, especially for adolescent patients. The physician, parents, and/or caregivers should expect issues including depression and noncompliance. They must be prepared to work together with the patient toward a better outcome.
  • Knight et al aimed to characterize the prevalence of depression and anxiety in pediatric systemic lupus erythematosus and mixed connective tissue disease patients and determine their association with healthcare utilization. Depression and anxiety symptoms were prevalent in patients with SLE/MCTD, and suicidal ideation significantly more common in SLE/MCTD than in healthy subjects. Despite prevalent symptoms, there were poor rates of prior mental health treatment, and less frequent PCP visits among those with depression symptoms. The authors concluded that further investigation of barriers to mental health care and interventional strategies for symptomatic youth with SLE/MCTD is needed.[12]
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Contributor Information and Disclosures
Author

Marisa S Klein-Gitelman, MD, MPH Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Head, Division of Rheumatology, Ann and Robert H Lurie Children's Hospital of Chicago

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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

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

Disclosure: Nothing to disclose.

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, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Barry L Myones, MD Co-Chair, Task Force on Pediatric Antiphospholipid Syndrome

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, Texas Medical Association

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. 1972 Feb. 52(2):148-59. [Medline].

  2. Alarcon-Segovia D, Villareal M. Classification and diagnostic criteria for mixed connective tissue disease. 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. 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. 1993 Dec. 95(6):595-600. [Medline].

  5. Nowicka-Sauer K, Czuszynska Z, Majkowicz M, Smolenska Z, Jarmoszewicz K, Olesinska M, et al. Neuropsychological assessment in mixed connective tissue disease: comparison with systemic lupus erythematosus. Lupus. 2012 Mar 20. [Medline].

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

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

  8. Ortega-Hernandez OD, Shoenfeld Y. Mixed connective tissue disease: An overview of clinical manifestations, diagnosis and treatment. Best Pract Res Clin Rheumatol. 2012 Feb. 26(1):61-72. [Medline].

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

  10. Malleson PN, Mackinnon MJ, Sailer-Hoeck M, Spencer CH. Review for the generalist: The antinuclear antibody test in children - When to use it and what to do with a positive titer. Pediatr Rheumatol Online J. 2010 Oct 20. 8:27. [Medline]. [Full Text].

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

  12. Knight A, Weiss P, Morales K, Gerdes M, Gutstein A, Vickery M, et al. Depression and anxiety and their association with healthcare utilization in pediatric lupus and mixed connective tissue disease patients: a cross-sectional study. Pediatr Rheumatol Online J. 2014. 12:42. [Medline]. [Full Text].

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

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

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

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

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

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

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

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

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