Sjögren’s Syndrome with Polyserositis, Gastrointestinal Findings and Ascending Aortic Aneurysm

Sjögren’s syndrome (SS) is an autoimmune disease with glandular and extraglandular manifestations. Pleuraland pericardial effusions in association with SS are rare. Similarly, ascites is rare and it can occur in SS whencombined with primary biliary cirrhosis (PBC). Inflammatory Abdominal Aortic Aneurysm together with SS has beendescribed only in one case. We report herein the case of a 70-year-old man with SS presenting with polyserositis(pleural and pericardial effusion and ascites) and gastrointestinal manifestations (atrophic gastritis and candidaesophagitis) and ascending aorta aneurysm. SS was diagnosed based on xerophthalmia, xerostomia,extraglandular manifestations, positive results for the Schirmer test, ocular surface staining score, histopathologicexamination of labial buccal mucosa revealing focal lymphocytic sialadenitis and unstimulated salivary flow rate. Theonly positive autoantibody was against smooth muscle cells (ASMA). We thought that pleural, pericardial effusions,ascites, gastrointestinal findings and ascending aortic aneurysm may be related with autoimmunologicalinflammation of SS. To evaluate the extent of aortic vasculitis, we performed a whole body 18-Fluorodeoxyglucosepositronemission tomography (FDG-PET) and showed increased uptake of FDG in aneurysmal section of theascending aorta. Treatment with high dose corticosteroid was proved to be successful in both clinically andlaboratory.