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Open access Oct 2026

Case Report: Upfront sotatercept-based triple therapy in high-risk systemic sclerosis-associated pulmonary arterial hypertension

Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) carries a poor prognosis, and treatment decisions are often complicated by coexisting left-heart disease, lung disease, chronic thromboembolic disease, or pulmonary veno-occlusive disease-like vasculopathy. The role of sotatercept as part of initia...

Yuichi Tamura, Sarasa Isobe, Hiroyuki Fujii et al. · 0 citations
Open access Oct 2026

Clinical characteristics and severity classification in Mixed Connective Tissue Disease: a nationwide retrospective study.

OBJECTIVES To characterize clinical manifestations, major organ involvement, real-world treatment patterns, and factors associated with severity classification in Japanese patients with mixed connective tissue disease (MCTD), aiming to clarify its heterogeneous clinical spectrum. METHODS This nationwide retrospective...

S. Kubo, Yurie Kanda, Hideto Kameda et al. · 0 citations
Review Open access Sep 2026

Conceptual framework for the definition of disease modification in systemic sclerosis and potential implications for drug development and clinical study design.

Systemic sclerosis (SSc) is a multifaceted systemic autoimmune disease, characterised by the interplay of vascular alterations, immune dysregulation, and fibrosis. The molecular changes and the clinical presentation of SSc are highly heterogeneous and vary considerably between individual patients. This heterogeneity ha...

Jörg H. W. Distler, S. Assassi, A. Gabrielli et al. · 0 citations
Open access Sep 2026

Demographic and clinical features of systemic sclerosis patients with anti-U1RNP antibodies: an European Scleroderma Trials and Research (EUSTAR) analysis

To investigate the demographic and clinical features of systemic sclerosis (SSc) patients with anti-U1RNP antibodies using the European Scleroderma Trials and Research (EUSTAR) database, a phenotype less well-defined in SSc than other more commonly associated antibodies. SSc patients fulfilling classificat...

Wan-Long Wu, M. Venables, E. Hachulla et al. · 0 citations

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