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The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
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Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma
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Sjögren's syndrome.

Clio P Mavragani1, Haralampos M Moutsopoulos

  • 1Department of Physiology, University of Athens, Athens, 11527 Greece;

Annual Review of Pathology
|September 21, 2013
PubMed
Summary

Sjögren's syndrome (SS) is an autoimmune disorder causing dry eyes and mouth due to exocrine gland damage. Research highlights B cell hyperactivity and immune system activation as key features, potentially linked to genetic factors and nucleic acid overexpression.

Area of Science:

  • Immunology
  • Rheumatology
  • Pathology

Background:

  • Sjögren's syndrome (SS) is a chronic autoimmune disorder primarily affecting exocrine glands, leading to dry mouth and eyes.
  • Histopathological hallmarks include mononuclear cell infiltrates in exocrine and other organs, prompting the term 'autoimmune epithelitis'.
  • B cell hyperactivity, characterized by autoantibodies and hypergammaglobulinemia, is a key feature, with immune complex-mediated phenotypes indicating lymphoma risk.

Purpose of the Study:

  • To review the key immunological features of Sjögren's syndrome.
  • To discuss the role of B cell hyperactivity and immune activation in SS pathogenesis.
  • To explore potential triggers for immune dysregulation in SS.

Main Methods:

  • Literature review of Sjögren's syndrome pathogenesis.

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  • Analysis of histopathological and serological findings in SS patients.
  • Discussion of recent research on immune system activation pathways in SS.

    • Main Results:

    • Mononuclear cell infiltrates are the hallmark of SS, affecting various organs.
    • B cell hyperactivity contributes to autoantibody production and immune complex formation.
    • Activation of the type I interferon/B cell-activating factor axis is a significant finding in SS.

    Conclusions:

    • Sjögren's syndrome involves complex autoimmune processes centered on exocrine glands and B cell activity.
    • Immune complex-mediated complications and lymphoma risk are associated with SS.
    • Genetic susceptibility and overexpression of endogenous nucleic acids may trigger immune activation in SS.