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Abstract Number: 276

Long-Term Outcome in Mixed Connective Tissue Disease

Maria Pascual1, Javier Narváez2, Gloria Albert Espi3, Mercè López de Recalde1, Andrea Zacarias4 and Juan José Alegre3, 1Department of Rheumatology, Hospital Universitario de Bellvitge, Barcelona, Spain, 2Rheumatology, Hospital Universitario de Bellvitge, Barcelona, Spain, 3Rheumatology Department, Hospital Universitario Doctor Peset, Valencia, Spain, 4Hospital Universitari de Bellvitge, Barcelona, Spain

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Mixed connective tissue disease (MCTD) and outcomes

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Session Information

Date: Sunday, November 8, 2015

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.


Disclosure: M. Pascual, None; J. Narváez, None; G. Albert Espi, None; M. López de Recalde, None; A. Zacarias, None; J. J. Alegre, None.

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 .
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