ArticleJournal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases2017
Mixed Connective Tissue Disease and Epitope Spreading: An Historical Cohort Study.
Article in Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 18 citations in OpenAlex.
- Early detection and risk stratification in autoimmune-related interstitial lung disease: a state-of-the-art review.Respiratory research · 2026Review
- Association of Combined Autoreactivity to Sm/RNP Common Motif and U1 RNP With Mixed Connective Tissue Disease and Systemic Lupus Erythematosus.ACR open rheumatology · 2024Article
- A Practical Multidisciplinary Approach to Identifying Interstitial Lung Disease in Systemic Autoimmune Rheumatic Diseases: A Clinician's Narrative Review.Diagnostics (Basel, Switzerland) · 2024Review
- Review
- T cell help in the autoreactive germinal center.Scandinavian journal of immunology · 2022Review
- Role of autoantibodies in the diagnosis and prognosis of interstitial lung disease in autoimmune rheumatic disorders.Therapeutic advances in musculoskeletal disease · 2021Review
- Diagnostic Imaging of Inflammatory Myopathies: New Concepts and a Radiological Approach.Current rheumatology reports · 2019Review
- Classification and management of adult inflammatory myopathies.The Lancet. Neurology · 2018Review
- The impact of anti-U1-RNP positivity: systemic lupus erythematosus versus mixed connective tissue disease.Rheumatology international · 2018Review
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
objectivesMixed connective tissue disease (MCTD) is characterized by the presence of anti-U1-snRNP autoantibodies and a variable set of associated clinical features. Some MCTD patients test positive over time to autoantibodies against Sm, proteins spatially related with U1-snRNP. This situation has been attributed to expanding of the autoimmune response by a phenomenon known as epitope spreading. Our aim was to study the frequency of this phenomenon in MCTD patients and the specific clinical features of those with epitope spreading.
methodsAll anti-U1-RNP-positive patients (2010-2015) were retrospectively reviewed, and those meeting the MCTD criteria were included in the study. Patients showing epitope spreading were compared with the remainder of the MCTD cohort. In addition, the clinical features of patients with epitope spreading were compared before and after the phenomenon occurred.
resultsAmong 72 anti-U1-RNP-positive patients, 40 (37 women) were diagnosed with MCTD. Thirteen MCTD patients (43%) presented epitope spreading, mainly during the first 2 years after the diagnosis of the disease (median, 1.4 years). Patients with epitope spreading had a significantly lower prevalence of skin sclerosis (0% vs. 44%, P = 0.004) and a greater prevalence of interstitial lung disease (46% vs. 15%, P = 0.05) than those without. Arthritis (92% vs. 25%, P = 0.02) and muscle involvement (67% vs. 17%, P = 0.02) were less frequent after epitope spreading had occurred.
conclusionEpitope spreading is common in MCTD, occurring early after the diagnosis. The clinical manifestations in patients with this phenomenon differ from those without, and their clinical features change after the immunological phenomenon has occurred.
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