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

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Abstract

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.

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References

  1. Mosca M, Neri R, Bombardieri S (1999) Undifferentiated connective tissue diseases: a review of the literature and a proposal for preliminary classification criteria. Clin Exp Rheumatol 17(5):615–620

    PubMed  CAS  Google Scholar 

  2. Zold E, Szodoray P, Gaal J, Kappelmayer J, Csathy L, Gyimesi E, Zeher M, Szegedi G, Bodolay E (2008) Vitamin D deficiency in undifferentiated connective tissue disease. Arthritis Res Ther 10(5):R123

    Article  PubMed  Google Scholar 

  3. James JA, Kim-Howard XR, Bruner BF, Jonsson MK, McClain MT, Arbuckle MR, Walker C, Dennis GJ, Merrill JT, Harley JB (2007) Hydroxychloroquine sulfate treatment is associated with later onset of systemic lupus erythematosus. Lupus 16(6):401–409

    Article  PubMed  CAS  Google Scholar 

  4. Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725

    Article  PubMed  CAS  Google Scholar 

  5. Petri M (2005) Review of classification criteria for systemic lupus erythematosus. Rheum Dis Clin North Am 31(2):245–254 vi

    Article  PubMed  Google Scholar 

  6. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criterion Committee (1980) Preliminary criteria for the classification of systemic scleroris (scleroderma). Arthritis Rheum 23:581–590

    Article  Google Scholar 

  7. LeRoy EC, Medsger TA (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28:1573–1576

    PubMed  CAS  Google Scholar 

  8. Johnson SR, Feldman BM, Hawker GA (2007) Classification criteria for systemic sclerosis subsets. J Rheumatol 34(9):1855–1863

    PubMed  Google Scholar 

  9. Vitali C, Bombardieri S, Moutsopoulos HM, Balestrieri G, Bencivelli W, Bernstein RM, Bjerrum KB, Braga S, Coll J, de Vita S et al (1993) Preliminary criteria for the classification of Sjögren’s syndrome. Results of a prospective concerted action supported by the European community. Arthritis Rheum 36:340–347

    Article  PubMed  CAS  Google Scholar 

  10. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH (2002) European Study Group on Classification Criteria for Sjögren’s Syndrome. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American–European Consensus Group. Ann Rheum Dis 61(6):554–558

    Article  PubMed  CAS  Google Scholar 

  11. Mosca M, Tani C, Bombardieri S (2007) Undifferentiated connective tissue diseases: a new frontier in rheumatology. Best Pract Res Clin Rheumatol 21(6):1011–1023

    Article  PubMed  CAS  Google Scholar 

  12. Alarcon GS, Williams GV, Singer JZ, Steen VD, Clegg DO, Paulus HE, Billingsley LM, Luggen ME, Polisson RP, Willkens RF et al (1991) Early undifferentiated connective tissue disease. I Early clinical manifestations in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of well established connective tissue disease. J Rheumatol 18:1332–1339

    PubMed  CAS  Google Scholar 

  13. Bodolay E, Csiki Z, Szekanecz Z, Ben T, Kiss E, Zeher M, Szücs G, Dankó K, Szegedi G (2003) Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease. Clin Exp Rheumatol 21:313–320

    PubMed  CAS  Google Scholar 

  14. Mosca M, Neri R, Bencivelli W, Tavoni A, Bombardieri S (2002) Undifferentiated connective tissue disease: analysis of 83 patients with a minimum follow up of 5 years. J Rheumatol 29:2345–2348

    PubMed  Google Scholar 

  15. Mosca M, Tavoni A, Neri R, Bencivelli W, Bombardieri S (1998) Undifferentiated connective tissue diseases: the clinical and serological profiles in 91 patients followed for at least 1 year. Lupus 7:95–100

    Article  PubMed  CAS  Google Scholar 

  16. Danieli MG, Fraticelli P, Salvi A, Gabrielli A, Danieli G (1998) Undifferentiated connective tissue diseases: natural history and evolution into definite CTD assessed in 84 patients initially diagnosed as early UCTD. Clin Rheumatol 17:195–201

    Article  PubMed  CAS  Google Scholar 

  17. Danieli MG, Fraticelli P, Franceschini F, Cattaneo R, Farsi A, Passaleva A, Pietrogrande M, Invernizzi F, Vanoli M, Scorza R, Sabbadini MG, Gerli R, Corvetta A, Farina G, Salsano F, Priori R, Valesini G, Danieli G (1999) Five-year follow-up of 165 Italian patients with undifferentiated connective tissue diseases. Clin Exp Rheumatol 17:585–591

    PubMed  CAS  Google Scholar 

  18. Dijkstra S, Nieuwenhuys EJ, Swaak AJ (1999) The prognosis and outcome of patients referred to an outpatient clinic for rheumatic diseases characterized by the presence of antinuclear antibodies (ANA). Scand J Rheumatol 28:33–37

    Article  PubMed  CAS  Google Scholar 

  19. Mosca M, Tani C, Bombardieri S (2008) A case of undifferentiated connective tissue disease: is it a distinct clinical entity? Nat Clin Pract Rheumatol 4(6):328–332

    Article  PubMed  Google Scholar 

  20. Swaak AJ, van de Brink H, Smeenk RJ, Manger K, Kalden JR, Tosi S, Marchesoni A, Domljan Z, Rozman B, Logar D, Pokorny G, Kovacs L, Kovacs A, Vlachoyiannopoulos PG, Moutsopoulos HM, Chwalinska-Sadowska H, Dratwianka B, Kiss E, Cikes N, Anic B, Schneider M, Fischer R, Bombardieri S, Mosca M, Graninger W, Smolen JS (2001) Study group on incomplete SLE and SLE with disease duration longer than 10 years. Incomplete lupus erythematosus: results of a multicenter study under the supervision of the EULAR Standing Commitee on International Clinical Studies Including Therapeutic Trials (ESCISIT). Rheumatol 40:89–94

    Article  CAS  Google Scholar 

  21. Calvo-Alen J, Alarcon GS, Burgard SL, Burst N, Bartolucci AA, Williams HJ (1996) Systemic lupus erythematosus: predictors of its occurrence among a cohort of patients with early undifferentiated connective tissue disease: multivariate analyzes and identification of risk factors. J Rheumatol 23:469–475

    PubMed  CAS  Google Scholar 

  22. Vila LM, Mayor AM, Valentín AH, García-Soberal M, Vilá S (2000) Clinical outcome and predictors of disease evolution in patients with incomplete lupus erythematosus. Lupus 9:110–115

    Article  PubMed  CAS  Google Scholar 

  23. Cavazzana I, Franceschini F, Belfiore N, Quinzanini M, Caporali R, Calzavara-Pinton P, Bettoni L, Brucato A, Cattaneo R, Montecucco C (2001) Undifferentiated connective tissue disease with antibodies to Ro/SSA: clinical features and follow up of 148 patients. Clin Exp Rheumatol 19:403–409

    PubMed  CAS  Google Scholar 

  24. Arbuckle MR, McClain MT, Rubertone MV, Scofield RH, Dennis GJ, James JA, Harley JB (2003) Development of autoantibodies before the clinical onset on systemic lupus erythematosus. N Eng J Med 349(16):1526–1533

    Article  CAS  Google Scholar 

  25. Doria A, Mosca M, Gambari PF, Bombardieri S (2005) Defining unclassifiable connective tissue diseases: incomplete, undifferentiated, or both? J Rheumatol 32:213–215

    PubMed  Google Scholar 

  26. Clegg DO, Williams HJ, Singer JZ, Steen VD, Schlegel S, Ziminski C, Alarcón GS, Luggen ME, Polisson RP, Willkens RF et al (1991) Early undifferentiated connective tissue disease. II. The frequency of circulating antinuclear antobodies in patients with early rheumatic diseases. J Rheumatol 18:1340–1343

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Rheumatology, Coimbra University Hospital, Praceta Prof. Mota Pinto, 3000-075, Coimbra, Portugal

    C. C. Vaz, M. Couto & L. Inês

  2. Institute of Physiology, Faculty of Medicine, Coimbra University, Coimbra, Portugal

    M. Couto

  3. Portuguese Institute of Rheumatology, Lisbon, Portugal

    D. Medeiros & L. Miranda

  4. Department of Rheumatology, Alto Minho Hospital Centre, Ponte de Lima, Portugal

    J. Costa

  5. Department of Rheumatology, Egas Moniz Hospital, Lisbon, Portugal

    P. Nero & R. Barros

  6. Department of Rheumatology, Garcia de Orta Hospital, Almada, Portugal

    M. J. Santos

  7. Department of Rheumatology, Santa Maria Hospital, Lisbon, Portugal

    E. Sousa

  8. Rheumatology Unit, Infante D. Pedro Hospital, Aveiro, Portugal

    A. Barcelos

  9. Faculty of Health Sciences, University of Beira Interior, Covilhã, Portugal

    L. Inês

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  1. C. C. Vaz
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Correspondence to C. C. Vaz.

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Vaz, C.C., Couto, M., Medeiros, D. et al. Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients. Clin Rheumatol 28, 915–921 (2009). https://doi.org/10.1007/s10067-009-1175-2

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  • DOI: https://doi.org/10.1007/s10067-009-1175-2

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