Sjögren's disease & overlap diseases

Unravelling interstitial pneumonia with autoimmune features (IPAF), VEDOSS and connective tissue diseases classifications through the mixed connective tissue disease spectrum

RMD OpenPublished Observational cohort study

Authors' 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.

Study context

Population
Mixed connective tissue disease (MCTD) patients in the French MCTD cohort
Exposure
IPAF or VEDOSS criteria fulfilment vs non-fulfilment at MCTD diagnosis
  • IPAFn = 38
  • VEDOSSn = 293
Outcome
Predictive value of IPAF, VEDOSS and dCTD classification criteria variables for progression to a dCTD
Methodological detailsFrench MCTD cohort
  • French MCTD cohort

Main finding

As reported in the abstract

Progression to a dCTD

Progressed into a differentiated connective tissue diseases (dCTD) (all MCTD)

111

34.3%

Progressed into systemic sclerosis (SSc)

50

15.4%

Progressed into systemic lupus erythematosus (SLE)

40

12.3%

Progressed into Sjögren's disease

11

3.4%

Observational data: associations, not effects.

Further findings

OutcomeIPAFNo IPAFVEDOSSNo VEDOSSP value
Progression to a dCTD by IPAF criteria at diagnosisIPAF vs No IPAF39.5%26.2%— not stated in the abstract— not stated in the abstractP = 0.09
Progression to SSc by VEDOSS criteria at diagnosisVEDOSS vs No VEDOSS— not stated in the abstract— not stated in the abstract15.7%12.9%P = 0.8

— not stated in the abstract

Baseline predictors of evolution

aPL predictive of evolution toward SLE
P = 0.01
Low C3 and/or C4 predictive of evolution toward SLE
P = 0.04
SSc classification criteria did not predict evolution toward SSc

Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.

Original publication

Unravelling IPAF, VEDOSS and connective tissue diseases classifications through the mixed connective tissue disease spectrum. RMD Open. Published 23 October 2025. doi:10.1136/rmdopen-2025-006145

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