European practices for screening and follow-up in mixed connective tissue disease: a survey of clinicians from the European Federation of Internal Medicine and ERN ReCONNET.

Authors

Kevin Chevalier, Marie-Elise Truchetet, Cloé Comarmond, Jeska de Vries-Bouwstra, Voon Ong, Paola Triggianese, Diana Marinello, Marta Mosca, Silvia Bellando Randone, Sergio Pietro-Gonzalez, Zana Brkic, Vera Guimarães, Magdalena Ciupera, Marzena Olesinska, Luc Mouthon, Benjamin Chaigne, European Federation of Internal Medicine Diseases Systemic Autoimmune Diseases working group and the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Mixed connective tissue group

Published in

European journal of internal medicine. Pages 107199. Sep 22, 2026. Epub Sep 22, 2026.

Abstract

Mixed connective tissue disease (MCTD) is a rare systemic autoimmune disorder in which interstitial lung disease (ILD) and pulmonary hypertension (PH) constitute the leading causes of death. No MCTD-specific recommendations currently exist regarding the timing, frequency, or indications for key paraclinical investigations. This study aimed to assess current European clinical practices regarding the use of high-resolution computed tomography (HRCT), pulmonary function tests (PFTs), transthoracic echocardiography (TTE), and nailfold capillaroscopy (NFC) in MCTD patients.
We conducted a cross-sectional survey distributed through the European Federation of Internal Medicine (EFIM) and the European Reference Network for Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET).
One hundred and thirty-five clinicians from 102 centers in 21 European countries were included. Respondents were predominantly rheumatologists (61.5%) and internists (32.6%). Systematic baseline screening was performed by 91.1% of respondents for PFTs, 85.9% for TTE, 74.1% for HRCT, and 71.9% for NFC. However, substantial heterogeneity was observed in follow-up strategies: 61.5% relied on clinical judgement alone to guide HRCT re-screening in ILD-negative patients, and 35.6% did not repeat NFC in patients with a scleroderma-like pattern at baseline. Referral for RHC after TTE suggestive of PH was also heterogeneous, with 34.8% requiring prior DETECT algorithm validation, despite this algorithm not being validated in MCTD.
This first European survey on paraclinical monitoring practices in MCTD reveals a broadly proactive approach to baseline screening, but highlights critical gaps in follow-up standardization. These data provide an empirical foundation for the development of evidence-based, MCTD-specific monitoring recommendations.

PMID:
42791152
Bibliographic data and abstract were imported from PubMed on 26 Sep 2026.

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