Table Of Contents
• Sjögren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands.
• It is most common in women in the middle of the 5th decade (postmenopausal).
• F/M ratio is 9/1.
• Patients mainly present with sicca symptoms (dry eye-dry mouth)
• As can be seen as a primary; It may also be associated (secondary) with some autoimmune diseases. Secondary Sjogren's syndrome is most commonly associated with rheumatoid arthritis. (Others; SLE, scleroderma, mixed connective tissue disease, etc.)
Glandular Findings
• Dry mouth (xerostomia) and dry eyes (keratoconjuntivitis sicca) are the most common findings (> 90%).
• Due to decreased salivation; dry mouth, difficulty in swallowing, dental caries, etc. can be seen.
• Due to the decrease in tears; dry eye, itching, stinging-foreign body sensation etc. may develop.
Extraglandular Findings
• The most common extraglandular finding is arthritis/arthralgia.
• Involvements associated with periepithelial lymphocyte infiltration (autoimmune epithelium)
o Lung involvement: Small airway disease, lymphocytic interstitial pneumonia
o Renal involvement: It may cause tubulointerstitial nephritis, causing Type 1 RTA, nephrocalcinosis and nephrogenic diabetes insipidus.
o Liver involvement: Primary biliary cholangitis (Sjögren's syndrome is the most common disease accompanying primary biliary cholangitis)
• Immune-complex related involvements (vasculitis)
o Small vessel vasculitis: cryoglobulinemic vasculitis (hypocomplementemia), palpable purpura
o Peripheral polyneuropathy (sensory/mixed)
o Glomerulonephritis: Membranoproliferative glomerulonephritis type 1
• lymphoma
o Glandular MALToma (extranodal marginal zone lymphoma) develops most frequently.
Findings that may indicate the development of lymphoma:
• Persistent growth in the parotid
• Splenomegaly
• lymphadenopathy
• Palpable purpura
• Leukopenia
• Cryoglobinemia
• Autoantibody positivity (Anti Ro, Anti La, RF)
• Low C4 complement
Laboratory Findings
• Autoantibodies; RF, ANA, Anti Ro and Anti La.
• Immunocomplexes are common in the circulation.
• Cryoglobulin is seen in some patients. It may be accompanied by hypocomplementemia.
• Autoantibodies (anti-parietal, anti-thyroglobulin, anti TPO, anti 21 hydroxylase, anti-mitochondrial, anti-smooth muscle antibody, anti-centromere) associated with other diseases may be found.
• Other findings; chronic disease anemia, increase in sedimentation rate, etc.
Diagnosis
• Causes of dry eyes and dry mouth should be ruled out before diagnosis; history of radiation to the head or neck, HIV and HCV infection, sarcoidosis, IgG4-related disease, etc.
Sjogren's Syndrome International Classification Criteria
1 . ocular symptoms
2. Oral symptoms
3. Findings of eye involvement
Schirmer test (to be 5 mm in 5 minutes)
4. Histopathology (most valuable)
Focal lymphocytic sialoadenitis
5. Signs of salivary gland involvement
Evaluation of the amount of saliva
6. Autoantibody positivity
Anti-Ro and/or anti-La (+)
It is sufficient to meet 4 criteria.
Treatment
• Dry mouth and eyes
o Local applications (artificial drops, moisturizers, oral hygiene, etc.)
o Muscarinic agonists (pilocarpine and sevimelin)
• Arthritis Steroids in addition to hydroxychloroquine or methotrexate
• Renal tubular acidosis Bicarbonate
• Vasculitis Steroids and/or immunosuppressives
• Lymphoma CHOP + Rituximab
• It is most common in women in the middle of the 5th decade (postmenopausal).
• F/M ratio is 9/1.
• Patients mainly present with sicca symptoms (dry eye-dry mouth)
• As can be seen as a primary; It may also be associated (secondary) with some autoimmune diseases. Secondary Sjogren's syndrome is most commonly associated with rheumatoid arthritis. (Others; SLE, scleroderma, mixed connective tissue disease, etc.)
Glandular Findings
• Dry mouth (xerostomia) and dry eyes (keratoconjuntivitis sicca) are the most common findings (> 90%).
• Due to decreased salivation; dry mouth, difficulty in swallowing, dental caries, etc. can be seen.
• Due to the decrease in tears; dry eye, itching, stinging-foreign body sensation etc. may develop.
Extraglandular Findings
• The most common extraglandular finding is arthritis/arthralgia.
• Involvements associated with periepithelial lymphocyte infiltration (autoimmune epithelium)
o Lung involvement: Small airway disease, lymphocytic interstitial pneumonia
o Renal involvement: It may cause tubulointerstitial nephritis, causing Type 1 RTA, nephrocalcinosis and nephrogenic diabetes insipidus.
o Liver involvement: Primary biliary cholangitis (Sjögren's syndrome is the most common disease accompanying primary biliary cholangitis)
• Immune-complex related involvements (vasculitis)
o Small vessel vasculitis: cryoglobulinemic vasculitis (hypocomplementemia), palpable purpura
o Peripheral polyneuropathy (sensory/mixed)
o Glomerulonephritis: Membranoproliferative glomerulonephritis type 1
• lymphoma
o Glandular MALToma (extranodal marginal zone lymphoma) develops most frequently.
Findings that may indicate the development of lymphoma:
• Persistent growth in the parotid
• Splenomegaly
• lymphadenopathy
• Palpable purpura
• Leukopenia
• Cryoglobinemia
• Autoantibody positivity (Anti Ro, Anti La, RF)
• Low C4 complement
Laboratory Findings
• Autoantibodies; RF, ANA, Anti Ro and Anti La.
• Immunocomplexes are common in the circulation.
• Cryoglobulin is seen in some patients. It may be accompanied by hypocomplementemia.
• Autoantibodies (anti-parietal, anti-thyroglobulin, anti TPO, anti 21 hydroxylase, anti-mitochondrial, anti-smooth muscle antibody, anti-centromere) associated with other diseases may be found.
• Other findings; chronic disease anemia, increase in sedimentation rate, etc.
Diagnosis
• Causes of dry eyes and dry mouth should be ruled out before diagnosis; history of radiation to the head or neck, HIV and HCV infection, sarcoidosis, IgG4-related disease, etc.
Sjogren's Syndrome International Classification Criteria
1 . ocular symptoms
2. Oral symptoms
3. Findings of eye involvement
Schirmer test (to be 5 mm in 5 minutes)
4. Histopathology (most valuable)
Focal lymphocytic sialoadenitis
5. Signs of salivary gland involvement
Evaluation of the amount of saliva
6. Autoantibody positivity
Anti-Ro and/or anti-La (+)
It is sufficient to meet 4 criteria.
Treatment
• Dry mouth and eyes
o Local applications (artificial drops, moisturizers, oral hygiene, etc.)
o Muscarinic agonists (pilocarpine and sevimelin)
• Arthritis Steroids in addition to hydroxychloroquine or methotrexate
• Renal tubular acidosis Bicarbonate
• Vasculitis Steroids and/or immunosuppressives
• Lymphoma CHOP + Rituximab