Distinct phenotypes in mixed connective tissue disease: Subgroups and survival

P. Szodoray, A. Hajas, L. Kardos, B. Dezso, G. Soos, E. Zold, J. Vegh, I. Csipo, B. Nakken, M. Zeher, G. Szegedi, E. Bodolay

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38 Citations (Scopus)

Abstract

The aim of the present study was to assess the autoantibody profile, dominant clinical symptoms and cluster characteristics of different Mixed connective tissue disease (MCTD phenotypes. Two-hundred-and-one patients with MCTD were followed-up longitudinally. Five clinical parameters, Raynaud's phenomenon, pulmonary artery hypertension (PAH), myositis, interstitial lung disease (ILD), erosive arthritis and five auto-antibodies besides anti-U1RNP, antiendothelial cell antibodies (AECA), anti-CCP, anti-cardiolipin (anti-CL), anti-SSA/SSB and IgM rheumatoid factor (RF) were selected for cluster analysis. The mean age of patients was 52.9±12.4 years and the mean follow-up of the disease was 12.5±7.2 years. Patients were classified into three cluster groups. Cluster 1 with 77 patients, cluster 2 with 79 patients and cluster 3 with 45 patients. In cluster 1 the prevalence of PAH (55.8%; p<0.001), Raynaud's phenomenon (92.2%; p<0.001) and livedo reticularis (24.6%, p<0.001) was significantly greater than in cluster 2 and 3. In cluster 2, the incidence of ILD (98.7%; p<0.001), myositis (77.2%; p<0.001), and esophageal dysmotility (89.8%; p<0.001) was significantly greater than that in cluster 1 and 3. In cluster 3, anti-CCP antibodies were present in 31 of 45 patients (68.8%) with erosions. Anti-CCP antibodies were present in 37 of 42 patients (88.0%) with erosions. PAH, angina, venous thrombosis was observed in cluster 1 and pulmonary fibrosis in cluster 2, musculosceletal damage, gastrointestinal symptoms and osteoporotic fractures were most frequent in cluster 3. Cumulative survival assessment indicated cluster 1 patients having the worst prognosis. Cluster analysis is valuable to differentiate among various subsets of MCTD and useful prognostic factor regarding the disease course.

Original languageEnglish
Pages (from-to)1412-1422
Number of pages11
JournalLupus
Volume21
Issue number13
DOIs
Publication statusPublished - Nov 1 2012

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Keywords

  • Mixed connective tissue disease
  • autoantibodies
  • cluster analysis
  • phenotypes

ASJC Scopus subject areas

  • Rheumatology

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