Rare combo: moyamoya and lupus in men

Clin Rheumatol. 2024 Jun;43(6):2139-2143. doi: 10.1007/s10067-024-06960-1. Epub 2024 Apr 16.

Abstract

Moyamoya syndrome (MMS) is a rare, chronic, progressive cerebrovascular disorder characterized by stenosis at the apices of the intracranial internal carotid arteries, including the proximal anterior cerebral arteries and middle cerebral arteries. Cerebral angiography images are used for detection through measurement. Systemic lupus erythematosus (SLE) is an autoimmune disease that can cause multisystemic involvement. The coexistence of SLE and MMS has been rarely reported in the literature. A 46-year-old male patient with malar rash, Raynaud phenomenon presented to the hospital with a complaint of weakness in the left lower extremity, which began 3 days before the date of the visit. In the diffusion magnetic resonance imaging, multiple diffusion restrictions were observed in the right frontal region. The patient underwent MR angiography, revealing stenosis in the terminal and supraclinoid segments of the right internal carotid artery, which made us consider moyamoya disease. This patient, with a malar rash and Raynaud's, a positive antibody profile, was diagnosed as a male with SLE accompanied by MMS.

Keywords: Lupus; Male lupus; Moyamoya; SLE.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Carotid Artery, Internal / diagnostic imaging
  • Cerebral Angiography
  • Diffusion Magnetic Resonance Imaging
  • Humans
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnosis
  • Lupus Erythematosus, Systemic* / diagnostic imaging
  • Magnetic Resonance Angiography*
  • Male
  • Middle Aged
  • Moyamoya Disease* / complications
  • Moyamoya Disease* / diagnostic imaging
  • Raynaud Disease / complications
  • Raynaud Disease / diagnosis