Clinical Rheumatology

, Volume 28, Issue 8, pp 915–921 | Cite as

Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients

  • C. C. Vaz
  • M. Couto
  • D. Medeiros
  • L. Miranda
  • J. Costa
  • P. Nero
  • R. Barros
  • M. J. Santos
  • E. Sousa
  • A. Barcelos
  • L. Inês
Original Article


The purpose of this study was to characterize the clinical and serological features of a large cohort of patients with antinuclear antibody (ANA) positive undifferentiated connective tissue disease (UCTD). Consecutive patients with UCTD, followed up at the Rheumatology Clinic of the participating centers, were included. Data from these patients were obtained by clinical evaluation and chart review. All patients were diagnosed as having UCTD on basis of the following criteria: positive ANA plus at least one clinical feature of connective tissue disease, but not fulfilling classification criteria for any differentiated connective tissue disease. One hundred eighty-four patients were studied (female patients—94.5%; mean age at time of evaluation—47 years). The most prevalent manifestations were arthralgia (66%), arthritis (32%), Raynaud’s phenomenon (30%), sicca symptoms (30%), and leukopenia (19%). The prevalence of ANA was 100%, anti-SSA 20%, anti-dsDNA 14%, and anti-SSB 7%. Patients with anti-dsDNA/anti-Sm, anticentromere/anti-Scl70, or anti-SSA/anti-SSB antibodies more frequently presented a set of manifestations close to systemic lupus erythematosus (SLE), systemic sclerosis, or Sjögren syndrome, respectively. We analyze a large cohort of UCTD. Seventy-two percent of these UCTD patients present lupus-, scleroderma-, or Sjögren-like features but do not fulfill classification criteria and mostly present a mild disease.


Classification criteria Connective tissue diseases Systemic lupus erythematosus Undifferentiated connective tissue diseases 





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Copyright information

© Clinical Rheumatology 2009

Authors and Affiliations

  • C. C. Vaz
    • 1
  • M. Couto
    • 1
    • 2
  • D. Medeiros
    • 3
  • L. Miranda
    • 3
  • J. Costa
    • 4
  • P. Nero
    • 5
  • R. Barros
    • 5
  • M. J. Santos
    • 6
  • E. Sousa
    • 7
  • A. Barcelos
    • 8
  • L. Inês
    • 1
    • 9
  1. 1.Department of RheumatologyCoimbra University HospitalCoimbraPortugal
  2. 2.Institute of Physiology, Faculty of MedicineCoimbra UniversityCoimbraPortugal
  3. 3.Portuguese Institute of RheumatologyLisbonPortugal
  4. 4.Department of RheumatologyAlto Minho Hospital CentrePonte de LimaPortugal
  5. 5.Department of RheumatologyEgas Moniz HospitalLisbonPortugal
  6. 6.Department of RheumatologyGarcia de Orta HospitalAlmadaPortugal
  7. 7.Department of RheumatologySanta Maria HospitalLisbonPortugal
  8. 8.Rheumatology UnitInfante D. Pedro HospitalAveiroPortugal
  9. 9.Faculty of Health SciencesUniversity of Beira InteriorCovilhãPortugal

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