eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Mixed Connective Tissue Disease: Follow-up

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
Contributor Information and Disclosures

Updated: Sep 29, 2009

Follow-up

Further Inpatient Care

  • 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.

Further Outpatient Care

  • 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.

Inpatient & Outpatient Medications

Transfer

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

Deterrence/Prevention

  • 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.

Complications

  • 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).

Prognosis

  • 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).

Patient Education

  • 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.
  • For excellent patient education resources, visit eMedicine's Muscle Disorders Center.

Miscellaneous

Medicolegal Pitfalls

  • Pitfalls exist because of the complicated nature of this illness. Primary health care providers are urged to seek the advice of a subspecialist in the diagnosis and treatment of mixed connective tissue disease (MCTD).

Special Concerns

  • Patients are often on potentially teratogenic medications; these women should avoid pregnancy. Children with mixed connective tissue disease benefit most from evaluation and treatment by a pediatric rheumatologist.
 


More on Mixed Connective Tissue Disease

Overview: Mixed Connective Tissue Disease
Differential Diagnoses & Workup: Mixed Connective Tissue Disease
Treatment & Medication: Mixed Connective Tissue Disease
Follow-up: Mixed Connective Tissue Disease
Multimedia: Mixed Connective Tissue Disease
References
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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].

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  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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Further Reading

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Keywords

mixed connective tissue disease, MCTD, autoimmune disorder, Raynaud phenomenon, puffy fingers, mild myositis, arthritis, anti-U1-68kD antibody, undifferentiated connective tissue disease, UCTD, lupus, rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, myositis, alopecia, leukopenia, anemia, pleuritis, pericarditis, trigeminal neuralgia, malar rash, thrombocytopenia, gastroesophageal reflux, GERD, treatment, diagnosis

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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 College of Rheumatology
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

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.

 
 
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