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Sjögren Syndrome
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Basics
- Chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs
- Typically presents with diminished salivary and lacrimal gland function, resulting in 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: complication of other rheumatologic conditions, most commonly rheumatoid arthritis; associated with HLA-DR4
- First described by Swedish ophthalmologist Henrik Sjögren
Epidemiology
Incidence
Annual incidence: ~4/100,000. Primary Sjögren syndrome (SS) is one of the most common autoimmune diseases, affecting 1–4% of population.
- All races are affected.
- Predominant sex: female > male (9:1)
- Predominant age: can affect patients of any age but is most common in the elderly; onset typically in the 4th to 5th decades of life
Prevalence
SS affects 1 to 4 million people in the United States.
Etiology and Pathophysiology
- Multifactorial systemic autoimmune process characterized by infiltration of glandular tissue by CD4 T lymphocytes
- Theorized that glandular epithelial cells present antigen to the T cells inducing cytokine production. There is also evidence for B-cell activation, resulting in autoantibody production and an increased incidence of B-cell malignancies.
- Etiology is unknown. Estrogen may play a role because SS is more common in women. Exogenous factors such as viral proteins (EBV, HCV, HTLV-1) have also been implicated.
Genetics
- A familial tendency suggests a genetic predisposition.
- Associations in the HLA regions HLA-DQA1*0501, HLA-DQB1*0201, and HLA-DRB*0301 are the strongest genetic risk factors for SS.
Risk Factors
There are no known modifiable risk factors.
General Prevention
Commonly Associated Conditions
Secondary SS associated with rheumatoid arthritis, scleroderma, systemic lupus erythematosus (SLE), polymyositis, HIV, hepatitis C, MCTD, PBC, hypergammaglobulinemic purpura, necrotizing vasculitis, autoimmune thyroiditis, chronic active hepatitis, mixed cryoglobulinemia
Pregnancy Considerations
Pregnant SS patients with anti-SSA Abs have increased risk of delivering fetus with skin rash and 3rd-degree heart block.
-- To view the remaining sections of this topic, please log in or purchase a subscription --
Basics
- Chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs
- Typically presents with diminished salivary and lacrimal gland function, resulting in 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: complication of other rheumatologic conditions, most commonly rheumatoid arthritis; associated with HLA-DR4
- First described by Swedish ophthalmologist Henrik Sjögren
Epidemiology
Incidence
Annual incidence: ~4/100,000. Primary Sjögren syndrome (SS) is one of the most common autoimmune diseases, affecting 1–4% of population.
- All races are affected.
- Predominant sex: female > male (9:1)
- Predominant age: can affect patients of any age but is most common in the elderly; onset typically in the 4th to 5th decades of life
Prevalence
SS affects 1 to 4 million people in the United States.
Etiology and Pathophysiology
- Multifactorial systemic autoimmune process characterized by infiltration of glandular tissue by CD4 T lymphocytes
- Theorized that glandular epithelial cells present antigen to the T cells inducing cytokine production. There is also evidence for B-cell activation, resulting in autoantibody production and an increased incidence of B-cell malignancies.
- Etiology is unknown. Estrogen may play a role because SS is more common in women. Exogenous factors such as viral proteins (EBV, HCV, HTLV-1) have also been implicated.
Genetics
- A familial tendency suggests a genetic predisposition.
- Associations in the HLA regions HLA-DQA1*0501, HLA-DQB1*0201, and HLA-DRB*0301 are the strongest genetic risk factors for SS.
Risk Factors
There are no known modifiable risk factors.
General Prevention
Commonly Associated Conditions
Secondary SS associated with rheumatoid arthritis, scleroderma, systemic lupus erythematosus (SLE), polymyositis, HIV, hepatitis C, MCTD, PBC, hypergammaglobulinemic purpura, necrotizing vasculitis, autoimmune thyroiditis, chronic active hepatitis, mixed cryoglobulinemia
Pregnancy Considerations
Pregnant SS patients with anti-SSA Abs have increased risk of delivering fetus with skin rash and 3rd-degree heart block.
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