Macitentan for the Treatment of Refractory Digital Ulcers in Patients With Connective Tissue Diseases

Systemic sclerosis (SSc) is a chronic autoimmune disease with complex pathogenesis, characterized by vascular dysfunction and fibrosis. Digital ulcers (DUs) are a common and severe complication in SSc patients, negatively impacting their quality of life. This retrospective study evaluates the use of...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2023-04, Vol.15 (4), p.e38303-e38303
Main Authors: Soto Abánades, Clara, Noblejas Mozo, Ana, Bonilla Hernán, Gema, Alvarez Troncoso, Jorge, Ríos Blanco, Juan José
Format: Article
Language:English
Subjects:
Age
Antibodies
Aspirin
Cardiac arrhythmia
Cardiac/Thoracic/Vascular Surgery
Clinical trials
Connective tissue diseases
Contraindications
Drugs
Enzymes
Immunology
Internal Medicine
Lung diseases
Medical records
Patients
Pulmonary hypertension
Raynaud disease
Rheumatology
Scleroderma
Skin
Ulcers
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Description
Summary:Systemic sclerosis (SSc) is a chronic autoimmune disease with complex pathogenesis, characterized by vascular dysfunction and fibrosis. Digital ulcers (DUs) are a common and severe complication in SSc patients, negatively impacting their quality of life. This retrospective study evaluates the use of macitentan, an endothelin receptor antagonist, in six female patients with connective tissue disease (CTD) and sclerodermiform features (five SSc and one mixed connective tissue disease) for the treatment of refractory DUs. Macitentan demonstrated a safe and effective alternative to bosentan, reducing DU relapses, hospitalizations, and the use of systemic prostaglandin therapy. The findings suggest that macitentan may be a valuable therapeutic option in specific cases of recurrent or refractory DUs and warrant further investigation in larger, long-term studies.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.38303