Sjögren's syndrome

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Sjögren's syndrome is an autoimmune disease that is chronic and slowly progressing. It manifests itself as a diffuse, chronic inflammation with the destruction of exocrine glands (salivary, lacrimal, or exocrine glands of the pancreas). We divide it into primary - separate disease and secondary - in another disease of an autoimmune nature (RA, SLE, MCTD - mixed connective tissue disease, systemic vasculitis).

Lymphocyte (plasmatic cell) infiltration in the gland
Biopsy - salivary gland (lymphocyte infiltration in the gland)

Etiology[edit | edit source]

The etiology is idiopathic, there is a possible relation of viruses (EBV, CMV), association in the HLA system.

Clinical characteristics[edit | edit source]

Salivary glands[edit | edit source]

Swelling of the salivary glands, xerostomia (dry mouth) and dysphagia.

Eye affection[edit | edit source]

Due to insufficient tear production, a picture of dry keratoconjunctivitis (burning, foreign body sensation in the eye, photophobia), xerophthalmia is formed.

Other exocrine glands affection[edit | edit source]

Diagnosis[edit | edit source]

Elevated levels of FW, CRP, γ-globulins, ENA autoantibodies type SS / A and SS / B, rheumatoid factor (RF), antinuclear antibodies. Diagnosis is determined if 4 criteria are present (if 3, dg. Is also possible):

  1. dry keratoconjunctivitis (demonstrated by Schirmer's test);
  2. xerostomia;
  3. biopsy of lymphocyte infiltrates in salivary glands; It is necessary to monitor - possible transition to lymphoproliferative disease (lymphoma). Monitoring of beta2-microglobulin levels serologically.
  4. detection of serum autoantibodies (rheumatoid factor, antinuclear antibodies, ENA antibodies).

Therapy[edit | edit source]

Mostly symptomatic:

  • Substitution therapy: eye drops, lubricating gels, moisturizing lotions, chewing gum. The secondary requires proper treatment of the underlying disease:
  • Antimalarials (hydroxychloroquine)
  • Glucocorticoids + combinations with other immunosuppressive drugs (methotrexate, prednisone + cyclophosphamide).
  • Careful dental care is important.

Prognosis[edit | edit source]

Patients with Sjögren's syndrome are limited in several respects. In the secondary form, the risk of malignancies increases. The most common malignancies are lymphoid malignancies - in about 5% [1] the most common are MALTomas and B-cell lymphomas of the salivary gland. [2] Despite this, the average age of patients with sicca syndrome is the same as in the normal population.

References[edit | edit source]

Related Articles[edit | edit source]

Sources[edit | edit source]

  • KLENER, Pavel. Vnitřní lékařství. 3. edition. Praha : Galén : Karolinum, 2006. pp. 1158. ISBN 80-7262-430-X.
  • FUČÍKOVÁ, Terezie. Klinická imunologie v praxi. 2. edition. Praha : Galén, 1997. pp. 339. ISBN 8085824574.

Reference[edit | edit source]

  1. TZIOUFAS, Athanasios G – VOULGARELIS, Michael. Update on Sjögren's syndrome autoimmune epithelitis: from classification to increased neoplasias. Best Practice and Research Clinical Rheumatology [online]2007, vol. 21, no. 6, p. 989-1010, Available from <>. ISSN 1521-6942. PMID: 18068857.DOI: 10.1016/j.berh.2007.09.001.
  2. VOULGARELIS, Michael – SKOPOULI, Fotini N. Clinical, immunologic, and molecular factors predicting lymphoma development in Sjogren's syndrome patients. Clinical Reviews in Allergy and Immunology [online]2007, vol. 32, no. 3, p. 265-74, Available from <>. ISSN 1080-0549. PMID: 17992593.DOI: 10.1007/s12016-007-8001-x.
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