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

Abstract

IntroductionThe 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 presentationWe 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.ConclusionHyposplenism 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.

Howell-Jolly bodies and liver-spleen scanning for assessment of splenic filtrative function yields discordant results in renal transplant recipients

“…There is also further supporting evidence from the literature. Indeed, based on data from the literature, 55 of 90 (61.1%) patients with a presence of HJBs had an absent or small spleen, [ 1 – 3 , 10 , 11 , 13 , 16 , 18 , 19 , 21 , 22 , 24 – 27 , 29 , 32 , 33 , 35 , 36 , 41 – 45 , 49 , 50 , 52 , 54 , 55 , 57 – 59 , 61 , 62 , 66 , 68 , 69 , 71 ] , whereas only 1 of 13 (7.7%) patients with absence of HJBs had a small spleen. [ 16 , 26 , 28 , 37 , 40 , 53 , 64 , 65 ] Moreover, of 35 cases of HJBs present with normal or enlarged spleen, 9 were sickle cell anemia cases [ 1 ] and in 5, microscopic sections showed extensive splenic parenchymal replacement by tissue infiltration of the underlying disease.…”

Energy Dynamics in the Autoimmune Disorders: The Unity of Immunity

“…Over the next several decades clinical syndromes very much similar to that seen with OPSI were recognized in people with intact spleens and gradually the concept of 'functional hyposplenism' evolved [503][504][505][506][507]. Originally described in children with sickle cell anemia, it occurs more frequently than previously thought and can be seen in celiac disease, alcoholic liver disease, hepatic cirrhosis, lymphomas and a spectrum of AIDs including SLE, rheumatoid arthritis, multiple sclerosis, Sjögren's syndrome, ulcerative colitis and autoimmune thyroiditis [508][509][510][511][512][513][514][515][516].…”