Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD)

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Abstract

Objective. To determine the clinical symptoms and the panel of autoantibodies of patients with early undifferentiated connective tissue disease (UCTD) followed for at least 1 year. Methods. 716 UCTD patients with manifestations suggestive but not diagnostic of specific connective tissue disease (CTD) were recruited and followed up between 1994-1999. The patients with early UCTD were subdivided into those with isolated Raynaud's phenomenon (RP) (50 patients), unexplained polyarthritis (31 patients) and "true " UCTD (665 patients). UCTD was diagnosed on the basis of clinical manifestations suggestive of a connective tissue disease and the presence of at least one non-organ specific autoantibody. The patients' sera were tested for anti-nuclear (ANA), as well as for nine different specific autoantibodies (anti-dsDNA, -Sm, -RNP, -SSA, -SSB, -Scl-70, -centromere, -Jo1 and -PM-Scl). Results. The most common clinical manifestations of UCTD included RP, arthritis/arthralgias, pleuritis/pericarditis, sicca symptoms, cutaneous involvement (photosensitivity, rash), central nervous symptoms, peripheral neuropathy, fever, vasculitis, less pulmonary involvement and myositis. 230 of the 665 true UCTD patients (34.5%) developed a defined CTD (28 systemic hipus erythematosus [SLE], 26 mixed connective tissue disease [MCTD], 19 progressive systemic sclerosis [PSS], 45 Sjögren's syndrome, 3 polymyositis/dermatomyositis [PM/DM], 87 rheumatoid arthritis [RA], and 22 systemic vasculitis. 435 of 665 patients (65.4%) remained in the UCTD state, and 82 of 665 patients (12.3%) achieved complete remission with symptoms not reappearing within the 5-year period. The highest probability of evolution to a defined CTD was during the first 2 years after onset: of 230 UCTD patients 183 (79.5%) developed major organ symptoms and signs. In particular skin and cardiac complications seemed to spread during the follow-up period in those patients who progressed to SLE. The condition of 18/50 patients with isolated RP evolved to UCTD and 3 of 31 patients with unexplained polyarthritis progressed to definite CTD (2 patients RA and one MCTD). Conclusion. In our study most of the UCTD patients did not develop a definite CTD, but during the follow-up period we found new clinical and serological manifestations. One-third of the UCTD patients showed progress into different types of specific CTD.

Original languageEnglish
Pages (from-to)313-320
Number of pages8
JournalClinical and Experimental Rheumatology
Volume21
Issue number3
Publication statusPublished - May 2003

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Connective Tissue Diseases
Raynaud Disease
Autoantibodies
Mixed Connective Tissue Disease
Arthritis
Rheumatoid Arthritis
Diffuse Scleroderma
Systemic Vasculitis
Pleurisy
Skin
Myositis
Dermatomyositis
Pericarditis
Centromere

Keywords

  • Connective tissue disease (CTD)
  • Prognosis
  • Treatment
  • Undifferentiated connective tissue disease (UCTD)

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

@article{6f1dab59ea994db5b0467e177ea8b93d,
title = "Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD)",
abstract = "Objective. To determine the clinical symptoms and the panel of autoantibodies of patients with early undifferentiated connective tissue disease (UCTD) followed for at least 1 year. Methods. 716 UCTD patients with manifestations suggestive but not diagnostic of specific connective tissue disease (CTD) were recruited and followed up between 1994-1999. The patients with early UCTD were subdivided into those with isolated Raynaud's phenomenon (RP) (50 patients), unexplained polyarthritis (31 patients) and {"}true {"} UCTD (665 patients). UCTD was diagnosed on the basis of clinical manifestations suggestive of a connective tissue disease and the presence of at least one non-organ specific autoantibody. The patients' sera were tested for anti-nuclear (ANA), as well as for nine different specific autoantibodies (anti-dsDNA, -Sm, -RNP, -SSA, -SSB, -Scl-70, -centromere, -Jo1 and -PM-Scl). Results. The most common clinical manifestations of UCTD included RP, arthritis/arthralgias, pleuritis/pericarditis, sicca symptoms, cutaneous involvement (photosensitivity, rash), central nervous symptoms, peripheral neuropathy, fever, vasculitis, less pulmonary involvement and myositis. 230 of the 665 true UCTD patients (34.5{\%}) developed a defined CTD (28 systemic hipus erythematosus [SLE], 26 mixed connective tissue disease [MCTD], 19 progressive systemic sclerosis [PSS], 45 Sj{\"o}gren's syndrome, 3 polymyositis/dermatomyositis [PM/DM], 87 rheumatoid arthritis [RA], and 22 systemic vasculitis. 435 of 665 patients (65.4{\%}) remained in the UCTD state, and 82 of 665 patients (12.3{\%}) achieved complete remission with symptoms not reappearing within the 5-year period. The highest probability of evolution to a defined CTD was during the first 2 years after onset: of 230 UCTD patients 183 (79.5{\%}) developed major organ symptoms and signs. In particular skin and cardiac complications seemed to spread during the follow-up period in those patients who progressed to SLE. The condition of 18/50 patients with isolated RP evolved to UCTD and 3 of 31 patients with unexplained polyarthritis progressed to definite CTD (2 patients RA and one MCTD). Conclusion. In our study most of the UCTD patients did not develop a definite CTD, but during the follow-up period we found new clinical and serological manifestations. One-third of the UCTD patients showed progress into different types of specific CTD.",
keywords = "Connective tissue disease (CTD), Prognosis, Treatment, Undifferentiated connective tissue disease (UCTD)",
author = "E. Bodolay and Zoltan Csiki and Z. Szekanecz and Thomas Ben and E. Kiss and M. Zeher and G. Szűcs and K. Dank{\'o} and G. Szegedi",
year = "2003",
month = "5",
language = "English",
volume = "21",
pages = "313--320",
journal = "Clinical and Experimental Rheumatology",
issn = "0392-856X",
publisher = "Clinical and Experimental Rheumatology S.A.S.",
number = "3",

}

TY - JOUR

T1 - Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD)

AU - Bodolay, E.

AU - Csiki, Zoltan

AU - Szekanecz, Z.

AU - Ben, Thomas

AU - Kiss, E.

AU - Zeher, M.

AU - Szűcs, G.

AU - Dankó, K.

AU - Szegedi, G.

PY - 2003/5

Y1 - 2003/5

N2 - Objective. To determine the clinical symptoms and the panel of autoantibodies of patients with early undifferentiated connective tissue disease (UCTD) followed for at least 1 year. Methods. 716 UCTD patients with manifestations suggestive but not diagnostic of specific connective tissue disease (CTD) were recruited and followed up between 1994-1999. The patients with early UCTD were subdivided into those with isolated Raynaud's phenomenon (RP) (50 patients), unexplained polyarthritis (31 patients) and "true " UCTD (665 patients). UCTD was diagnosed on the basis of clinical manifestations suggestive of a connective tissue disease and the presence of at least one non-organ specific autoantibody. The patients' sera were tested for anti-nuclear (ANA), as well as for nine different specific autoantibodies (anti-dsDNA, -Sm, -RNP, -SSA, -SSB, -Scl-70, -centromere, -Jo1 and -PM-Scl). Results. The most common clinical manifestations of UCTD included RP, arthritis/arthralgias, pleuritis/pericarditis, sicca symptoms, cutaneous involvement (photosensitivity, rash), central nervous symptoms, peripheral neuropathy, fever, vasculitis, less pulmonary involvement and myositis. 230 of the 665 true UCTD patients (34.5%) developed a defined CTD (28 systemic hipus erythematosus [SLE], 26 mixed connective tissue disease [MCTD], 19 progressive systemic sclerosis [PSS], 45 Sjögren's syndrome, 3 polymyositis/dermatomyositis [PM/DM], 87 rheumatoid arthritis [RA], and 22 systemic vasculitis. 435 of 665 patients (65.4%) remained in the UCTD state, and 82 of 665 patients (12.3%) achieved complete remission with symptoms not reappearing within the 5-year period. The highest probability of evolution to a defined CTD was during the first 2 years after onset: of 230 UCTD patients 183 (79.5%) developed major organ symptoms and signs. In particular skin and cardiac complications seemed to spread during the follow-up period in those patients who progressed to SLE. The condition of 18/50 patients with isolated RP evolved to UCTD and 3 of 31 patients with unexplained polyarthritis progressed to definite CTD (2 patients RA and one MCTD). Conclusion. In our study most of the UCTD patients did not develop a definite CTD, but during the follow-up period we found new clinical and serological manifestations. One-third of the UCTD patients showed progress into different types of specific CTD.

AB - Objective. To determine the clinical symptoms and the panel of autoantibodies of patients with early undifferentiated connective tissue disease (UCTD) followed for at least 1 year. Methods. 716 UCTD patients with manifestations suggestive but not diagnostic of specific connective tissue disease (CTD) were recruited and followed up between 1994-1999. The patients with early UCTD were subdivided into those with isolated Raynaud's phenomenon (RP) (50 patients), unexplained polyarthritis (31 patients) and "true " UCTD (665 patients). UCTD was diagnosed on the basis of clinical manifestations suggestive of a connective tissue disease and the presence of at least one non-organ specific autoantibody. The patients' sera were tested for anti-nuclear (ANA), as well as for nine different specific autoantibodies (anti-dsDNA, -Sm, -RNP, -SSA, -SSB, -Scl-70, -centromere, -Jo1 and -PM-Scl). Results. The most common clinical manifestations of UCTD included RP, arthritis/arthralgias, pleuritis/pericarditis, sicca symptoms, cutaneous involvement (photosensitivity, rash), central nervous symptoms, peripheral neuropathy, fever, vasculitis, less pulmonary involvement and myositis. 230 of the 665 true UCTD patients (34.5%) developed a defined CTD (28 systemic hipus erythematosus [SLE], 26 mixed connective tissue disease [MCTD], 19 progressive systemic sclerosis [PSS], 45 Sjögren's syndrome, 3 polymyositis/dermatomyositis [PM/DM], 87 rheumatoid arthritis [RA], and 22 systemic vasculitis. 435 of 665 patients (65.4%) remained in the UCTD state, and 82 of 665 patients (12.3%) achieved complete remission with symptoms not reappearing within the 5-year period. The highest probability of evolution to a defined CTD was during the first 2 years after onset: of 230 UCTD patients 183 (79.5%) developed major organ symptoms and signs. In particular skin and cardiac complications seemed to spread during the follow-up period in those patients who progressed to SLE. The condition of 18/50 patients with isolated RP evolved to UCTD and 3 of 31 patients with unexplained polyarthritis progressed to definite CTD (2 patients RA and one MCTD). Conclusion. In our study most of the UCTD patients did not develop a definite CTD, but during the follow-up period we found new clinical and serological manifestations. One-third of the UCTD patients showed progress into different types of specific CTD.

KW - Connective tissue disease (CTD)

KW - Prognosis

KW - Treatment

KW - Undifferentiated connective tissue disease (UCTD)

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C2 - 12846049

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