Sjögren Syndrome

Descriptive text is not available for this image BASICS

  • First described by Swedish ophthalmologist Henrik Sjögren; chronic autoimmune disorder characterized by inflammation from lymphocytic infiltration of exocrine organs (especially lacrimal and salivary glands) resulting in decreased gland function; typically, presents with sicca symptoms such as dry eyes (xerophthalmia), dry mouth (xerostomia), and parotid enlargement
  • Extraglandular manifestations: arthralgia, myalgia, Raynaud phenomenon, pulmonary disease, GI disease, leukopenia, anemia, lymphadenopathy, vasculitis, renal tubular acidosis, lymphoma, CNS involvement with longitudinal transverse myelitis (>4 vertebral segments), and optic neuritis associated with anti–aquaporin-4 antibodies, PNS involvement with small fiber neuropathy
  • Primary Sjögren: not associated with other diseases; HLA-DRB1*0301 and HLA-DRB1*1501 are the most common
  • Secondary Sjögren: occurs in conjunction with other autoimmune rheumatic disorders, such as rheumatoid arthritis (most common); associated with HLA-DR4, systemic lupus erythematous (SLE), or systemic sclerosis

EPIDEMIOLOGY

Incidence

Annual incidence: ~4/100,000

  • No racial or geographic predilection
  • Predominant sex: female > male (9:1)
  • Predominant age at onset: 4th to 5th decades of life

Prevalence

Sjögren syndrome (SS) affects 1 to 4 million people in the United States.

ETIOLOGY AND PATHOPHYSIOLOGY

  • Etiology is unknown; theorized to be a viral trigger (EBV, HCV, HTLV-1) in genetically predisposed people
  • Pathophysiology involves multifactorial systemic autoimmune process characterized by infiltration of glandular tissue predominately by CD4 T lymphocytes.
    • Theorized that glandular epithelial cells present antigen to the T cells inducing cytokine production, ultimately leading to B-cell dysregulation, autoantibody production, chronic inflammation, and increased incidence of B-cell malignancies.

Genetics

A familial tendency suggests a genetic predisposition.

GENERAL PREVENTION

No known mode of prevention; complications can be prevented by early diagnosis and treatment. Oral health providers play a key role in early detection and management of salivary dysfunction.

COMMONLY ASSOCIATED CONDITIONS

Secondary SS is associated with rheumatoid arthritis, scleroderma, SLE, polymyositis, HIV, hepatitis C, MCTD, PBC, hypergammaglobulinemic purpura, necrotizing vasculitis, autoimmune thyroiditis, chronic active hepatitis, and mixed cryoglobulinemiaPregnancy Considerations
Pregnant SS patients with high anti-SSA Abs have increased risk of delivering a fetus with congenital heart block. Currently, there are no established evidence-based protocols for screening, prophylaxis, or treatment.

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