Chevalier Kevin, Thoreau Benjamin, Michel Marc, Godeau Bertrand, Agard Christian, Papo Thomas, Sacre Karim, Bader-Meunier Brigitte, Seror Raphaele, Mariette Xavier, Cacoub Patrice, Benhamou Ygal, Levesque Hervé, Goujard Cécile, Lambotte Olivier, Bonnotte Bernard, Samson Maxime, Ackermann Félix, Schmidt Jean, Duhaut Pierre, Koné-Paut Isabelle, Kahn Jean-Emmanuel, Hanslik Thomas, Costedoat-Chalumeau Nathalie, Terrier Benjamin, Regent Alexis, Dunogue Bertrand, Cohen Pascal, Le Guern Véronique, Hachulla Eric, Chaigne Benjamin, Mouthon Luc
Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d'Ile de France, AP-HP.Centre, Université Paris Cité, Paris, France.
Department of Internal Medicine, CHRU de Tours, Tours, France.
RMD Open. 2025 Oct 23;11(4):e006145. doi: 10.1136/rmdopen-2025-006145.
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).
We aimed to evaluate the predictive value of IPAF, VEDOSS and dCTD classification criteria variables in MCTD patients.
We conducted an observational study within the French MCTD cohort. IPAF, VEDOSS and current dCTD classification criteria were used to classify patients.
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).
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.
混合性结缔组织病（MCTD）长期以来一直被争论是否是分化型结缔组织病（dCTD）的早期非特异性阶段/症状，类似于具有自身免疫特征的间质性肺炎（IPAF）和系统性硬化症的极早期诊断（VEDOSS）。
我们旨在评估IPAF、VEDOSS和dCTD分类标准变量对MCTD患者的预测价值。
我们在法国MCTD队列中进行了一项观察性研究。使用IPAF、VEDOSS和当前的dCTD分类标准对患者进行分类。
纳入324例MCTD患者并随访8（3.3 - 13）年。其中，111例（34.3%）进展为dCTD，即50例（15.4%）系统性硬化症（SSc）、40例（12.3%）系统性红斑狼疮（SLE）和11例（3.4%）干燥综合征。诊断时，38例（11.7%）患者符合IPAF标准，其中15例（39.5%）进展为dCTD（未符合IPAF标准的患者中75例（26.2%）进展为dCTD；p = 0.09）。诊断时，293例（90.4%）患者符合VEDOSS标准，但进展为SSc的频率并不比不符合VEDOSS标准的MCTD患者更高（46例（15.7%）对4例（12.9%）；p = 0.8）。基线时，SSc分类标准不能预测向SSc的进展，而抗磷脂抗体以及低C3和/或C4可预测向SLE的进展（分别为p = 0.01和p = 0.04）。
在MCTD诊断时，符合IPAF和/或VEDOSS标准不能预测向SSc的进展，而抗磷脂抗体以及低C3和/或C4可预测向SLE的进展。这表明MCTD患者应被排除在IPAF和VEDOSS之外。
