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Sjögren's syndrome

Sjögren's syndrome is a chronic autoimmune disease characterized by progressive lymphocytic infiltration of exocrine glands. The pathology belongs to the group of rheumatic diseases that can present with the development of secondary vasculitis and leads to severe damage to the mucous membranes.

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Core PathologyLymphocytic infiltration and destruction of exocrine glands
Key SymptomsXerostomia, xerophthalmia, and mucosal dryness
GeneticsAssociation with major histocompatibility antigens HLA-DR3, -DQ1, -DQ2
HistologyFoci of lymphoid infiltration in glandular tissue
3 min readFor medical studentsUpdated 10.10.2026
Material prepared by WhiteCoat Pro editorial team based on platform study guidesPending instructor review

Etiology and Predisposing Factors

The development of Sjögren's syndrome is multifactorial in nature. The disease does not occur spontaneously; its onset requires a combination of internal predisposition and external triggers.

  • Genetic Background: A close association of the disease with certain major histocompatibility complex antigens has been proven, primarily with HLA-DR3, HLA-DQ1, and HLA-DQ2 alleles.
  • Hormonal Influence: Estrogens play a significant role in the development of the pathology, which largely determines the profile of patients susceptible to this disease.
  • External Triggers: Chronic viral infection often acts as the initiating mechanism for immunological disorders. It triggers a cascade of reactions in genetically compromised individuals.

Immunopathogenesis

The disease is based on a complex immunopathological process targeting exocrine glands (predominantly salivary and lacrimal glands).

  1. Cellular Infiltration: CD4+ T lymphocytes dominate in the affected areas of glandular tissue, initiating local inflammation.
  2. Cytokine Cascade: Activated cells actively synthesize pro-inflammatory cytokines, among which interleukins (IL-1, IL-6), interferon-gamma (IFN-γ), and tumor necrosis factor-alpha (TNF-α) play a key role.
  3. Vicious Cycle of Inflammation: Under the influence of high concentrations of cytokines, epithelial cells of exocrine glands begin to aberrantly express class II HLA molecules. This makes them visible to the immune system, leading to further activation of both T and B lymphocytes.
  4. Autoantibody Production: Hyperactivation of the B-lymphocyte link enhances the production of antibodies, including specific autoantibodies, ultimately causing irreversible destruction of the salivary and lacrimal glands.
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Clinical and morphological picture

The consequence of exocrine gland destruction is a sharp decrease in their secretory function. Clinically, this manifests as progressive dryness syndrome.

  • Ocular Involvement: Xerophthalmia (dry eyes) develops, which morphologically manifests as keratoconjunctivitis sicca.
  • Oral Involvement: Xerostomia (dry mouth) occurs due to the death of salivary gland cells.
  • Generalized Mucosal Dryness: The pathological process is not limited to the eyes and mouth. Patients suffer from dryness of the mucous membranes of the nose, larynx, trachea, and bronchi. In women, genital dryness is also observed.
  • Gastrointestinal Involvement: A characteristic manifestation of the syndrome is the development of atrophic gastritis.

Diagnostic criteria

The diagnosis of Sjögren's syndrome is based on confirming the autoimmune nature of the inflammation and visualizing structural changes in the glands.

  • Laboratory Diagnostics: Autoantibodies to nuclear antigens are detected in patients' blood serum. The presence of rheumatoid factor is also characteristic.
  • Histological examination: Biopsy of the affected glands (most commonly minor salivary glands) reveals a pathognomonic sign — foci of pronounced lymphoid infiltration in the tissue.
How to memorize

To memorize the clinical picture, use the 'Three Xeros' rule: Xerophthalmia (eyes), Xerostomia (mouth), and Xero-mucosa (respiratory tract and genitalia).

Class and exam questions

Which cells predominate in the glandular infiltrate in Sjögren's syndrome?

CD4+ T lymphocytes predominate in the foci of autoimmune destruction of exocrine glands.

How do cytokines affect the glandular epithelium?

Pro-inflammatory cytokines (IL-1, IL-6, IFN-γ, TNF-α) induce aberrant expression of class II HLA molecules on epithelial cells, which triggers further activation of T and B lymphocytes.

What laboratory markers are characteristic of this disease?

Key laboratory findings are autoantibodies to nuclear antigens and positive rheumatoid factor.

Are dry eyes and dry mouth the only symptoms?

No, the process is systemic. The mucous membranes of the nose, larynx, trachea, bronchi, and female genitals are also affected, and atrophic gastritis develops.

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What else to study on this topic

  • Behçet's disease: systemic vasculitis with ulcerative lesions
  • Systemic lupus erythematosus (SLE) and anti-DNA antibodies
  • Secondary vasculitis in rheumatic diseases
  • Immune-complex microcirculatory vessel damage

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