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

Sjögren’s syndrome (SS) is an autoimmune disease with glandular and extraglandular manifestations. Pleural and pericardial effusions in association with SS are rare. Similarly, ascites is rare and it can occur in SS when combined with primary biliary cirrhosis (PBC). Inflammatory Abdominal Aortic Aneurysm together with SS has been described 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 candida esophagitis) and ascending aorta aneurysm. SS was diagnosed based on xerophthalmia, xerostomia, extraglandular manifestations, positive results for the Schirmer test, ocular surface staining score, histopathologic examination of labial buccal mucosa revealing focal lymphocytic sialadenitis and unstimulated salivary flow rate. The only 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 autoimmunological inflammation of SS. To evaluate the extent of aortic vasculitis, we performed a whole body 18-Fluorodeoxyglucosepositron emission tomography (FDG-PET) and showed increased uptake of FDG in aneurysmal section of the ascending aorta. Treatment with high dose corticosteroid was proved to be successful in both clinically and laboratory.