In a 67-year-old man with speech impairment and right-hand clumsiness, a brain computed tomography revealed signs of acute ischemia in the left frontal lobe while an echo-color Doppler sonography of the cervical vessels showed a tight stenosis of left internal carotid artery with a large pseudoaneurysm. Histological findings performed on the surgical specimen disclosed IgG4-related disease.
To the best of our knowledge, this is the first manifestation of IgG4-related disease with carotid artery dissection complicated by pseudoaneurysm. Even though unsuccessful since the patients died within 48 h, this case highlights the diverse facets of the IgG4-related disease representing a new complication with important clinical implications of such a diagnosis targeting immunosuppressive therapy particularly B-cell depletion.