Undifferentiated Connective-Tissue Disease Follow-up

Updated: Jun 15, 2021
  • Author: Bernard Hildebrand, MD, MA; Chief Editor: Herbert S Diamond, MD  more...
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Further Outpatient Care

Patients with undifferentiated connective-tissue disease (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 greater, given the propensity for UCTD to evolve early in the disease course.



Prognostic considerations include the following:

  • 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. [22]
  • Leading factors associated with evolution to definite CTD are high antinuclear antibody (ANA) titers, presence of cytopenias at baseline, and progression of nailfold-capillaroscopy patter during follow-up [34]
  • Approximately 10%-20% of patients with UCTD have symptoms that subside or remit and never evolve into a defined CTD. [29]
  • The incidence of evolution to a defined CTD is highest during the first 3-5 years of the disease course, [35] and the rate of evolution continues to decrease over time. [36]
  • 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. [19, 36]
  • Survival rates in UCTD are similar to those associated with rheumatoid arthritis and systemic lupus erythematosus (SLE). [44]

Patient Education

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, systemic sclerosis, mixed CTD). Furthermore, education should focus on the following:

  • Symptomatic treatment
  • Appropriate understanding of treatment medications
  • The indications for contacting the primary care physician or rheumatologist