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.

Prognosis

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

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Table 1. Preliminary Classification Criteria for Undifferentiated Connective-Tissue Disease

Inclusion Criteria

Clinical

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^[14]and Doria et al.^[23]

2. Presence of antinuclear antibodies determined on two different occasions

Malar rash

Subacute cutaneous lupus

Discoid lupus

Cutaneous sclerosis

Heliotrope rash

Gottron papules

Erosive arthritis

Anti-dsDNA

Anti-Smith

Anti-U1-RNP

Anti-Scl70

Anticentromere

Anti-La/SSB

Anti-Jo1

Anti-Mi2

Table 2. Predictors of Progression to Definite Connective-Tissue Disease

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