Session Information
Date: Tuesday, October 28, 2025
Title: (2470–2503) Systemic Sclerosis & Related Disorders – Clinical Poster III
Session Type: Poster Session C
Session Time: 10:30AM-12:30PM
Background/Purpose: Mixed connective tissue disease (MCTD) is a rare systemic disorder that belongs to connective tissue diseases (CTD). Approximately 25% of MCTD patients will meet the classification criteria for at least one other CTD after a median follow-up of 8 years. MCTD has long been debated as an early nonspecific phase/symptom of differentiated connective tissue diseases (dCTD), similarly as others “pre-CTD” states described in literature such as interstitial pneumonia with autoimmune features (IPAF) or very early diagnosis of systemic sclerosis (SSc) (VEDOSS).
Methods: We aimed to evaluate the predictive value of dCTD classification, IPAF, and VEDOSS criteria in MCTD patients. We conducted an observational study within the French MCTD cohort. This cohort includes patients diagnosed with MCTD in 15 rare disease reference and competence centers, including two pediatric centers, across France, according to at least one of the four diagnostic criteria for MCTD and who, at disease onset, did not fulfill classification criteria for SSc, systemic lupus erythematosus, Sjögren’s disease (SjD), idiopathic inflammatory myopathies, nor rheumatoid arthritis. IPAF, VEDOSS and current dCTD classification criteria were used to classify patients.
Results: Three hundred and twenty-four patients were included and followed for a median [IQR] duration of 101 [48-176] months. Two hundred and eighty-four (87.7%) patients were females, with a median age at diagnosis of 34 [24-45] years. Among them, 111 (34.3%) progressed into a dCTD, i.e. 50 (15.4%) SSc, 40 (12.3%) systemic lupus erythematosus (SLE), and 11 (3.4%) SjD. At MCTD diagnosis, 38 (11.7%) patients had ILD, and all of them fulfilled the IPAF criteria; among which 15 (39.5%) progressed into a dCTD (vs. 75 (26.2%) in patients who did not fulfill IPAF criteria; p=0.09) (Table 1). At diagnosis, 293 (90.4%) patients fulfilled VEDOSS criteria (MCTD-VEDOSS+) (Table 2). All of them had Raynaud’s phenomenon and antinuclear antibodies. After a median follow-up of 101 [48-176] months, 46 (15.7%) of the 293 MCTD-VEDOSS+ patients progressed to SSc, compared to 4 (12.9%) of patients not fulfilling VEDOSS criteria (p=0.8). Among MCTD-VEDOSS+ patients, an abnormal nailfold capillaroscopy at MCTD diagnosis was significantly associated with progression toward SSc (p=0.01). At baseline, SSc classification criteria did not predict evolution toward SSc, whereas antiphospholipid antibodies and low C3 and/or low C4 were predictive of an evolution toward SLE (p=0.01 and p=0.04, respectively) (Table 3).
Conclusion: At MCTD diagnosis, fulfillment of IPAF and/or VEDOSS classification criteria was not predictive of evolution toward SSc, whereas antiphospholipid antibodies and low C3 and/or low C4 were predictive of an evolution toward SLE. This suggests that MCTD patients should be excluded from IPAF and VEDOSS.1. Chevalier K, et al. J Intern Med. 2023
.jpg)
.jpg)
To cite this abstract in AMA style:
Chevalier K, Torreau B, Michel M, Godeau B, AGARD C, Papo T, Sacré K, Bader-Meunier B, Seror R, Mariette X, Patrice C, Benhamou Y, Levesque H, goujard C, Lambotte O, Bonnotte B, Samson M, Ackermann F, Schmidt J, Duhaut P, Kone-Paut I, Kahn J, Hanslik T, Costedoat-Chalumeau N, Terrier B, REGENT A, Dunogue b, Cohen P, Le Guern V, HACHULLA E, Chaigne B, Mouthon L. Unraveling IPAF, VEDOSS and connective tissue diseases classifications through the mixed connective tissue disease spectrum [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/unraveling-ipaf-vedoss-and-connective-tissue-diseases-classifications-through-the-mixed-connective-tissue-disease-spectrum/. Accessed .« Back to ACR Convergence 2025
ACR Meeting Abstracts - https://acrabstracts.org/abstract/unraveling-ipaf-vedoss-and-connective-tissue-diseases-classifications-through-the-mixed-connective-tissue-disease-spectrum/