Abstract
Background Sjögren's syndrome is a chronic autoimmune disorder that predominantly affects exocrine
organs. It is characterized by an organ-specific infiltration of lymphocytes. The
involvement of the major cerebral arteries in Sjögren's syndrome has rarely been reported.
A recent study reported a case of successful extracranial–intracranial (EC–IC) bypass
without complications, even in the active inflammatory state, although the optimal
timing of such a bypass remains unclear.
Case Description We here report the case of a 43-year-old woman presenting with acute ischemic stroke
due to progressive middle cerebral artery (MCA) occlusion and signs of primary Sjögren's
syndrome. During intensive immunosuppressive therapy for active Sjögren's syndrome,
the patient was monitored using contrast-enhanced magnetic resonance vessel wall imaging
(MR-VWI). A couple of intravenous cyclophosphamide injections combined with a methylprednisolone
pulse and antiplatelet therapy resulted in clear resolution of vessel wall enhancement,
which suggested remission of inflammatory vasculitis. Nevertheless, she still experienced
a transient ischemic attack (TIA) due to decreased regional cerebral blood flow by
MCA occlusion, as demonstrated by the conventional time-of-flight MR angiography and
single-photon emission computed tomography. Considering the increased risk of further
stroke, the decision was made to perform an EC–IC bypass as a treatment for medically
uncontrollable hemodynamic impairment. Her postoperative course was uneventful without
further repeated TIAs, and continued immunosuppressive therapy for Sjögren's syndrome
provided effective management.
Conclusions Our findings emphasize the diagnostic value of contrast-enhanced MR-VWI in monitoring
the effect of immunosuppressive therapy for the major cerebral artery vasculitis and
in determining the timing of EC–IC bypass as a “rescue” treatment for moyamoya syndrome
associated with active Sjögren's syndrome.
Keywords large cerebral artery - moyamoya syndrome - Sjögren's syndrome - vascular disorder
- vessel wall imaging
