Date: Sunday, November 8, 2015
Session Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster I
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: The question of whether mixed connective tissue disease (MCTD) is a distinct entity still remains controversial. For this reason, we have investigated the long-term outcome in a well characterized cohort of patients with MCTD in order to determine the frequency of evolution toward other connective tissue diseases (CTDs).
Methods: Ambispective study of thirty-four MTCD patients, all of them fulfilling the diagnostic criteria proposed by Alarcon-Segovia (J Rheumatol 1989;16:328-34), with a minimal follow-up after the first clinical presentation of at least 2 yr. The endpoint of patient follow-up was the date of the last clinic visit.
Results: At the end of the follow-up period (median ± SD: 90.5 ± 62.1 months; minimum, maximum: 25-228 months), 41.1% of patients (14/34) still satisfied MCTD classification criteria of Alarcon-Segovia; 14.7% (5/34) evolved into systemic sclerosis [SSc] (fulfilling the 2013 ACR/EULAR classification criteria for SSc), 26.5% (9/34) into systemic lupus erythematosus (fulfilling the 2012 SLICC criteria), 8.8% (3/34) into seronegative rheumatoid arthritis (fulfilling 2010 ACR-EULAR classification criteria), and 8.8% (3/34) developed an overlap syndrome (2 cases of SSc+SLE and 1 case of SSc+RA).
The mean score in patients that meet the 2013 EULAR/ACR criteria for SSc was 11 (minimum, maximum: 9, 16). The mean score in patients satisfying the 2012 SLICC SLE criteria was 6 (minimum, maximum: 5, 9); only 2 of these patients developed major organ involvement.
Using multiple variable regression analysis, the presence of sclerodactyly (OR 1.1; 95% CI 1.1 – 1.7, P =0.04) was associated with evolution into SSc. No clinical predictors of potential evolution toward SLE were identified.
Conclusion: MCTD is a distinct clinical entity but it is evident that the majority of these patients (59% of the cases) will evolve into another CTD during disease progression.
To cite this abstract in AMA style:Pascual M, Narváez J, Albert Espi G, López de Recalde M, Zacarias A, Alegre JJ. Long-Term Outcome in Mixed Connective Tissue Disease [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/long-term-outcome-in-mixed-connective-tissue-disease/. Accessed September 28, 2021.
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