Moyamoya syndrome related to systemic lupus erythematosus developing during pregnancy: a case-based review


Moyamoya syndrome (MMS) is a chronic cerebrovascular disorder characterized by occlusion or stenosis of the internal carotid arteries with the formation of abnormal collateral vascular networks. Moreover, the development of MMS, which is a distinct category from “moyamoya disease,” is attributed to the underlying disease, while some cases of MMS related to systemic lupus erythematosus (SLE) have been previously reported. Herein, we present the case of a 29-year-old Japanese woman with SLE in whom intracranial hemorrhage ascribable to MMS developed during pregnancy. Craniotomy was performed to remove hematoma, and prednisolone, tacrolimus, and hydroxychloroquine were consecutively administered. She ultimately achieved remission and childbearing without the relapse of cerebrovascular event. To our knowledge, this is the first report of MMS associated with SLE in pregnancy. Through reviewing published English articles and our case, it was suggested that the pathogenesis of SLE is implicated in the development of moyamoya vasculopathy leading to cerebrovascular events. Moreover, pregnancy may affect the bleeding from the fragile collateral vessel wall.

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We wish to thank the members of the Department of Obstetrics and Gynecology and Department of Neurosurgery, Shinshu University School of Medicine, for their efforts in providing medical care.

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Correspondence to Yasuhiro Shimojima.

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Tanaka, R., Shimojima, Y., Ueno, K. et al. Moyamoya syndrome related to systemic lupus erythematosus developing during pregnancy: a case-based review. Clin Rheumatol (2020).

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  • Intracranial hemorrhage
  • Moyamoya syndrome
  • Pregnancy
  • Systemic lupus erythematosus