Severe Autoimmune Digital Vasculopathy in Systemic Sclerosis with Lupus- and Antiphospholipid-Spectrum Serologic Positivity and Libman–Sacks Endocarditis: A Case Report

Authors

• Mayada F. Bawayan, Internal Medicine Department Section of Rheumatology, East Jeddah hospital, Ministry of Health - Jeddah -Saudi Arabia
• Thekra A. Alwafi, Department of Medicine, College of Medicine, Umm Al-Qura University, Makkah, Saudi Arabia
• Abdulaziz F. Samandar, Department of Medicine, College of Medicine, Umm Al-Qura University, Makkah, Saudi Arabia
• Abdulaziz M. Gari, Internal Medicine Department Section of Rheumatology, East Jeddah hospital, Ministry of Health - Jeddah -Saudi Arabia

Keywords

“Systemic sclerosis”, “Digital ischemia”, “Libman–Sacks endocarditis”, “Overlap connective tissue disease”, “Case report

Document Type

Research Article

Abstract

Background: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by prominent vasculopathy, which may lead to severe digital ischemia and ulceration. The coexistence of SSc-related vasculopathy with lupus- and antiphospholipid-spectrum manifestations, including Libman–Sacks endocarditis, is uncommon and poses diagnostic and therapeutic challenges. Case Presentation: We report a 37-year-old female with a systemic sclerosis phenotype and overlapping lupus- and antiphospholipid-spectrum features, who presented with severe bilateral digital ischemia and ulceration. Investigations revealed active autoimmune disease with inflammatory, immunologic, and vascular involvement, along with Libman–Sacks endocarditis. Imaging excluded large-vessel occlusive disease. The patient was treated with targeted vasodilator therapy and immunomodulatory agents, resulting in marked clinical improvement with healing of digital ulceration and resolution of ischemic discoloration. Conclusion: This case highlights a rare overlap connective tissue disease phenotype presenting with severe autoimmune-mediated digital vasculopathy and Libman–Sacks endocarditis. Early recognition and a multidisciplinary, individualized treatment approach may lead to favorable outcomes in complex autoimmune overlap syndromes.

Publisher

Saudi Toxicology Society

Recommended Citation

Bawayan, Mayada F.; Alwafi, Thekra A.; Samandar, Abdulaziz F.; and Gari, Abdulaziz M. (2026) "Severe Autoimmune Digital Vasculopathy in Systemic Sclerosis with Lupus- and Antiphospholipid-Spectrum Serologic Positivity and Libman–Sacks Endocarditis: A Case Report," Saudi Toxicology Journal: Vol. 3: Iss. 1, Article 6.
DOI: https://doi.org/10.70957/uqu.edu.sa/s.toxicology.s/stj.2026.1.3.10
Available at: https://stj.researchcommons.org/journal/vol3/iss1/6

DOI

https://doi.org/10.70957/uqu.edu.sa/s.toxicology.s/stj.2026.1.3.10

January 2026