Sjögren's syndrome and acquired splenic atrophy with septic shock: a case report.

INTRODUCTION: The most frequent causes of adult-onset recurrent infections are human immunodeficiency virus infection, malignancy, and autoimmune diseases, while acquired non-surgical hyposplenism is rare. Although acquired asplenia/hyposplenism have been described in association with celiac disease and, less frequently, with autoimmune diseases such as Sjögren's syndrome, the manifestations in this context are usually only detectable in the laboratory setting, with Howell-Jolly bodies or thrombocytosis. To the best of our knowledge, no previous case of pneumococcal septic shock in a patient with acquired hyposplenism and co-morbid Sjögren's syndrome has been reported. CASE PRESENTATION: We report a case of a 45-year-old Caucasian woman who developed pneumococcal pneumonia at age 42 years, pneumococcal meningitis at age 44 years and septic shock with Streptococcus agalactiae bacteremia at age 45 years and was subsequently diagnosed with radiological splenic atrophy and functional asplenia, as well as primary Sjögren's syndrome. After appropriate immunizations, the patient has been free from clinically important infections. CONCLUSION: Hyposplenism should be suspected in patients with adult-onset infections caused by encapsulated bacteria, especially if autoantibodies are present. Early diagnosis can help prevent potentially life-threatening infections. Possible associations between splenic atrophy and Sjögren's syndrome are discussed.