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Disease evolution and organ damage accrual in patients with stable UCTD: a long-term monocentric inception cohort
- Source :
- RMD Open, Vol 10, Iss 2 (2024)
- Publication Year :
- 2024
- Publisher :
- BMJ Publishing Group, 2024.
-
Abstract
- Objectives Undifferentiated connective tissue diseases (UCTDs) are systemic autoimmune conditions that cannot be diagnosed nor classified as defined CTD; the majority maintains an undifferentiated profile (stable UCTD, sUCTD) over time. Data on long-term outcomes of sUCTD are lacking.Methods Retrospective longitudinal analysis of an inception cohort of 141 patients with sUCTD.Disease evolution and damage accrual were evaluated at 1, 5 and 10 years. Partial least square (PLS) regression was used to identify the basal variables contributing to damage accrual at 1, 5 and 10 years of follow-up. Trend of damage over time was compared with a cohort of age-matched and sex-matched patients with systemic lupus erythematosus (SLE) by means of Nelson-Aalen analysis.Results 11.3% of patients evolved to a definite CTD after a median 11 years (IQR 6–25) from the first symptom. At last visit, 10% were on glucocorticoids and 6% on immunosuppressive therapy. In 27.3%, at least one item of organ damage was recorded according to the SLICC/DI score (mean score 1.19±0.46). At PLS analysis, age at diagnosis and age at first symptoms were related to damage at 1 year, not taking antimalarials and taking immunosuppressants were associated with damage at 5 years.The mean survival without damage was 9.3 years in sUCTD and 8.4 years in SLE. The 10-year probability without damage was 62% and 23% in SLE and sUCTD, respectively (p=0.015).Conclusions Although less significantly impacted than in patients with SLE, in the long-term UCTDs can accumulate organ damage and evolve into defined connective tissue diseases.
- Subjects :
- Medicine
Subjects
Details
- Language :
- English
- ISSN :
- 20565933
- Volume :
- 10
- Issue :
- 2
- Database :
- Directory of Open Access Journals
- Journal :
- RMD Open
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.226b2535f87142e4b7a3994c9dea4035
- Document Type :
- article
- Full Text :
- https://doi.org/10.1136/rmdopen-2023-003967