Unravelling IPAF, VEDOSS and connective tissue diseases classifications through the mixed connective tissue disease spectrum Article Swipe
Kévin Chevalier
,
Benjamin Thoreau
,
Marc Michel
,
Bertrand Godeau
,
C. Agard
,
T. Papo
,
Karim Sacré
,
Brigitte Bader-Meunier
,
Raphaèle Séror
,
Xavier Mariette
,
P. Cacoub
,
Y. Benhamou
,
H. Lévesque
,
Cécile Goujard
,
O. Lambotte
,
Bernard Bonnotte
,
Maxime Samson
,
Félix Ackermann
,
Jean M. Schmidt
,
P. Duhaut
,
Isabelle Koné‐Paut
,
Jean‐Emmanuel Kahn
,
Thomas Hanslik
,
N. Costedoat‐Chalumeau
,
Benjamin Terrier
,
Alexis Régent
,
Bertrand Dunogué
,
Pascal Cohen
,
Véronique Le Guern
,
Eric Hachulla
,
Benjamin Chaigne
,
Luc Mouthon
·
YOU?
·
· 2025
· Open Access
·
· DOI: https://doi.org/10.1136/rmdopen-2025-006145
YOU?
·
· 2025
· Open Access
·
· DOI: https://doi.org/10.1136/rmdopen-2025-006145
Background Mixed connective tissue disease (MCTD) has long been debated as an early nonspecific phase/symptom of differentiated connective tissue diseases (dCTD), similarly to interstitial pneumonia with autoimmune features (IPAF) and very early diagnosis of systemic sclerosis (SSc) (VEDOSS). Objective We aimed to evaluate the predictive value of IPAF, VEDOSS and dCTD classification criteria variables in MCTD patients. Methods We conducted an observational study within the French MCTD cohort. IPAF, VEDOSS and current dCTD classification criteria were used to classify patients. Results Three hundred and twenty-four MCTD patients were included and followed for 8 (3.3–13) years. Among them, 111 (34.3%) progressed into a dCTD, that is, 50 (15.4%) SSc, 40 (12.3%) systemic lupus erythematosus (SLE) and 11 (3.4%) Sjögren’s disease. At diagnosis, 38 (11.7%) patients fulfilled IPAF criteria, among which 15 (39.5%) progressed into a dCTD (vs 75 (26.2%) in patients who did not fulfil IPAF criteria; p=0.09). At diagnosis, 293 (90.4%) patients fulfilled VEDOSS criteria but did not progress significantly more frequently to SSc than MCTD patients without VEDOSS criteria (46 (15.7%) vs 4 (12.9%); p=0.8). At baseline, SSc classification criteria did not predict evolution toward SSc, whereas antiphospholipid antibodies and low C3 and/or C4 were predictive of an evolution toward SLE (p=0.01 and p=0.04, respectively). Conclusion At MCTD diagnosis, fulfilment of IPAF and/or VEDOSS criteria was not predictive of evolution toward SSc, whereas antiphospholipid antibodies and low C3 and/or C4 were predictive of an evolution toward SLE. This suggests that MCTD patients should be excluded from IPAF and VEDOSS.
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Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1136/rmdopen-2025-006145
- https://rmdopen.bmj.com/content/11/4/e006145.full.pdf
- OA Status
- gold
- References
- 48
- OpenAlex ID
- https://openalex.org/W4415467089
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W4415467089Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.1136/rmdopen-2025-006145Digital Object Identifier
- Title
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Unravelling IPAF, VEDOSS and connective tissue diseases classifications through the mixed connective tissue disease spectrumWork title
- Type
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articleOpenAlex work type
- Language
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enPrimary language
- Publication year
-
2025Year of publication
- Publication date
-
2025-10-01Full publication date if available
- Authors
-
Kévin Chevalier, Benjamin Thoreau, Marc Michel, Bertrand Godeau, C. Agard, T. Papo, Karim Sacré, Brigitte Bader-Meunier, Raphaèle Séror, Xavier Mariette, P. Cacoub, Y. Benhamou, H. Lévesque, Cécile Goujard, O. Lambotte, Bernard Bonnotte, Maxime Samson, Félix Ackermann, Jean M. Schmidt, P. Duhaut, Isabelle Koné‐Paut, Jean‐Emmanuel Kahn, Thomas Hanslik, N. Costedoat‐Chalumeau, Benjamin Terrier, Alexis Régent, Bertrand Dunogué, Pascal Cohen, Véronique Le Guern, Eric Hachulla, Benjamin Chaigne, Luc MouthonList of authors in order
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https://doi.org/10.1136/rmdopen-2025-006145Publisher landing page
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https://rmdopen.bmj.com/content/11/4/e006145.full.pdfDirect link to full text PDF
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YesWhether a free full text is available
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goldOpen access status per OpenAlex
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https://rmdopen.bmj.com/content/11/4/e006145.full.pdfDirect OA link when available
- Cited by
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0Total citation count in OpenAlex
- References (count)
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48Number of works referenced by this work
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