Undifferentiated Connective-Tissue Disease Follow-up
- Author: Bernard Hildebrand, MD, MA; Chief Editor: Herbert S Diamond, MD more...
Further Outpatient Care
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- Patients with UCTD are typically monitored for progression of specific manifestations, evolution to a defined CTD, and safety and efficacy of treatment. The frequency of outpatient visits necessary depends on the severity of disease. During the first 5 years postdiagnosis, the frequency of follow-up visits may be more frequent given the propensity for UCTD to evolve early in the disease course.
Further Inpatient Care
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- Inpatient care in patients with undifferentiated connective-tissue disease (UCTD) may be necessary only for complex/urgent diagnostic evaluations or therapeutic reasons.
Inpatient & Outpatient Medications
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- Medications are prescribed, adjusted, and monitored as indicated for symptoms and safe medical therapy.
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- Transfer of care ultimately depends on any complications of UCTD that might require subspecialty medical or surgical care.
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- Deterrence and prevention of UCTD is directly related to the organ or organs involved. However, appropriate preventive medicine screening and immunizations should be considered annually.
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- The complications of UCTD are related directly to specific organ involvement, susceptibility to infections, drug intolerance/reactions, and drug interactions.
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- The prognosis of UCTD depends primarily on the extent of organ involvement.
- UCTD may evolve into a defined CTD in 20%-40% of patients with UCTD, while 50%-60% of cases remain undifferentiated.
- Patients who develop a defined CTD late have been noted to have milder disease with a lower incidence of serious adverse events and a better prognosis.[15, 29]
- Survival rates in UCTD are similar to those associated with RA and SLE.
See the list below:
- Patients with UCTD, as well as their immediate family members, should be educated about the prognosis of UCTD and the potential for the disease to evolve into a defined CTD (eg, SLE, SSc, MCTD).
- Furthermore, education should focus on symptomatic treatment, an appropriate understanding of treatment medications, and the indications for contacting the primary care physician or rheumatologist.
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Exclusion Criteria (Applicable to patients at disease onset)
|Laboratory Exclusion Criteria (Applicable to patients at disease onset)|
|1. Signs and symptoms suggestive of a CTD but not fulfilling the diagnostic or classification criteria for any of the defined CTDs (using previously established classification criteria for SLE, MCTD, SSc, PM/DM, RA and SS) for at least 3 years. If the disease duration is less than 3 years, patients may be defined as having an early UCTD.|
Adapted from Mosca et al and Doria et al.
2. Presence of antinuclear antibodies determined on two different occasions
Subacute cutaneous lupus
|Connective-Tissue Disease Association||Signs or Symptoms||Laboratory Data|
|Systemic lupus erythematosus||Age, fever, photosensitivity, serositis, alopecia||ANA, Anti-dsDNA, Anti-Smith, Anti-cardiolipin antibodies, Coombs positivity, leukopenia|
|Systemic sclerosis||Sclerodactyly, Raynaud phenomenon, sicca symptoms, esophageal dysfunction||ANA with nucleolar pattern|
|Sjögren syndrome||Xerostomia, xerophthalmia, Raynaud phenomenon||Anti-SSA, Anti-SSB|
|Rheumatoid arthritis||Symmetric polyarthritis||RF, elevated ESR (>70 mm/h)|
|Mixed connective-tissue disease||Esophageal reflux, polyarthritis, Raynaud phenomenon||ANA, Anti-U1-RNP|