Case Report: A Chinese woman with primary Sjögren's syndrome presented with simultaneous involvement of the cerebral and coronary arteries as initial symptoms.

INTRODUCTION: Primary Sjögren's syndrome (pSS) is a heterogeneous autoimmune disorder that may be associated with systemic manifestations such as pulmonary or articular involvement.Compared to other autoimmune diseases, vascular injury and accelerated atherosclerosis in pSS are less studied.

CASE PRESENTATION: A 51-year-old woman presented with a 2-month history of dizziness, chest tightness, and exertional dyspnea. Vascular imaging revealed multifocal stenoses in the left anterior descending coronary artery (70%), bilateral middle cerebral arteries, right anterior cerebral artery, and left posterior cerebral artery. Her only traditional cardiovascular risk factor was hypertension, and she reported only mild xerostomia. Serology showed anti-SSA positivity. Schirmer's test was abnormal, and a labial gland biopsy confirmed focal lymphocytic sialadenitis (≥1 foci/4 mm²), leading to a pSS diagnosis per 2016 ACR-EULAR criteria. She responded favorably to a combination of immunomodulators (hydroxychloroquine and total glucosides of paeony), antiplatelet therapy, and statins, with symptom resolution at follow-up.

CONCLUSION: This case represents an exceptionally rare documented instance of pSS simultaneously affecting both cerebral and coronary arterial systems. It underscores that severe multifocal arterial stenosis can present as the initial manifestation of this disease, even in the absence of prominent dryness symptoms. This highlights the necessity for vascular assessment in pSS patients and the urgency of conducting autoimmune evaluations in cases of unexplained multivessel arteriopathy.