Pediatric Mixed Connective Tissue Disease Follow-up
- Author: Marisa S Klein-Gitelman, MD, MPH; Chief Editor: Lawrence K Jung, MD more...
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
- See Medical Care.
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.
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