Cerebral vasculopathy in systemic sclerosis A case report and review of literature

• Published in: Medicine, case reports and study protocols Vol. 1; no. 2; p. e0027
• Main Authors: Iwata, Mai, Nakai, Noriyoshi, Kunitake, Katsuhiko, Inagakai, Ryosuke, Furukawa, Soma, Suzuki, Junichiro, Nishida, Suguru, Itoh, Takayuki, Ito, Yasuhiro
• Format: Journal Article
• Language: English
• Published: 01.12.2020
• ISSN: 2691-3895; 2691-3895
• DOI: 10.1097/MD9.0000000000000027

Abstract Introduction: Systemic sclerosis (SS) is 1 of the representative connective tissue diseases commonly affecting vascular endothelial cells and has an unknown pathogenesis. In comparison with other connective tissue diseases, vascular involvement in the brain is relatively rare in SS. Patient concerns: A 40-year-old woman developed neurological symptoms at 1 year after the diagnosis of SS. Head magnetic resonance imaging demonstrated diverse neuroradiologic abnormalities indicating brain edema, ischemic lesions and vasodilation or microbleeds from small vessels. 99 mTc-ethyl cysteinate dimer-single photon emission computed tomography revealed decreased blood flow in both frontal lobes. Diagnosis: The patient was diagnosed with cerebral vasculopathy in SS. Interventions: Oral administration of prednisolone 50 mg/d was started and gradually tapered. Outcomes: Her neurologic symptoms improved, and most of the neuroradiologic abnormalities were also resolved. Conclusions: Our case sheds light on the rare cerebral vasculopathy associated with SS and the importance of head magnetic resonance images and single photon emission computed tomography in the diagnosis and assessment of therapeutic efficacy.