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Undifferentiated Connective-Tissue Disease Follow-up

  • Author: Bernard Hildebrand, MD, MA; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Apr 16, 2015
 

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.
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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.
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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

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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/Prevention

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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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Complications

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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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Prognosis

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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.[18]
  • Approximately 10%-20% of patients with UCTD have symptoms that subside or remit and never evolve into a defined CTD.[25] The incidence of evolution to a defined CTD is highest during the first 3-5 years of the disease course,[28] and the rate of evolution continues to decrease over time.[29]
  • 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.[37]
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Patient Education

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  • 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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Contributor Information and Disclosures
Author

Bernard Hildebrand, MD, MA Associate Program Director, San Antonio Military Medical Center Rheumatology Fellowship

Bernard Hildebrand, MD, MA is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Society for Bioethics and Humanities

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel F Battafarano, DO MACP, FACR, Chief, Rheumatology Service, San Antonio Military Medical Center; Professor of Medicine, Uniformed Services University of the Health Sciences, F Edward Hebert School of Medicine; Adjunct Professor of Medicine, University of Texas Health Science Center at San Antonio

Daniel F Battafarano, DO is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

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



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[10] and Doria et al.[19]



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 AssociationSigns or SymptomsLaboratory Data
Systemic lupus erythematosusAge, fever, photosensitivity, serositis, alopeciaANA, Anti-dsDNA, Anti-Smith, Anti-cardiolipin antibodies, Coombs positivity, leukopenia
Systemic sclerosisSclerodactyly, Raynaud phenomenon, sicca symptoms, esophageal dysfunctionANA with nucleolar pattern
Sjögren syndromeXerostomia, xerophthalmia, Raynaud phenomenonAnti-SSA, Anti-SSB
Rheumatoid arthritisSymmetric polyarthritisRF, elevated ESR (>70 mm/h)
Mixed connective-tissue diseaseEsophageal reflux, polyarthritis, Raynaud phenomenonANA, Anti-U1-RNP
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