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sjogren's syndrome

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sjogren's syndrome

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sjogren's syndrome by Dr.Aakanksha singh,Resident SKIN & VD

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  1. 1. SJOGREN’S SYNDROME Dr.Aakanksha Singh
  2. 2.  Sjogren’s syndrome is a late onset chronic systemic autoimmune disease characterized by lymphocytic infiltration and destruction of the salivary and lacrimal glands resulting in dry mouth(xerostomia) and dryness and atrophy of conjunctiva and cornea (keratoconjunctivits sicca).  Swedish Ophthalmologist Henrik Sjögren who first described it (1933) ;
  3. 3.  This disease is caused by an immune-mediated inflammation of salivary,lacrimal and sweat glands as Sicca Syndrome or with internal organ involvement. Clinical Types  PRIMARY SS - Alone.  SECONDARY SS - associated underlying connective tissue diseases (RA / SLE / Scleroderma )  SICCA SYNDROME – Xerophthalmia + Xerostomia – Internal Organ / Bone Inv
  4. 4. ETIOLOGY  Female : Male = 9 : 1  4/5/6th decade  Autoimmune ; HLA-B8 / DR3 ,although DR4 is more closely associated with syndrome occuring with raynaud’s phenomenon  Antibodies to the Ro antigen occur in excess in relatives of pts with sjogren’s syndrome.
  5. 5. Pathogenesis of Sjögren’s syndrome is believed to be multifactorial. Known to be autoimmune, but studies suggest that the disease process has genetic, environmental(EBV/HCV) and hormonal(associated with high prevalence in women,esp estrogen) components
  6. 6.  Lymphocyte and plasma cell infiltration  Auto- antibody production (to ‘Ro’)  Connective tissue proliferation  Glandular cell apoptosis  atrophy of glandular structures in affected tissues (salivary glands, sebaceous glands, sweat glands)  Secondary changes – oedema of conjuctiva
  7. 7. C/F  Glandular manifestation  Dry mouth (Xerostomia) due to decreased production of saliva by salivary glands Cracked, peeling and atrophic appearance of the lips. Dry and fissured tongue
  8. 8.  Teeth – multilpe carries and early loss
  9. 9.  Chronic oral candidiasis is frequent.  Parotid Gland Enlargement
  10. 10.  Lacrimal Gland Involvement – Dryness of eyes causes keratoconjuctivitis sicca.  Burning itchy sensation in the eyes.
  11. 11. SKIN MANIFESTATIONS (50%) Xeroderma, pruritus and scaling Annular erythema, Papular Erythema including Sweet’s-like lesions. Raynaud’s syndrome Hyperglobulinemic Purpura Vitiligo Sweating abnormalities Cutaneous Amyloidosis Alopecia—diffuse and generalized
  12. 12. OTHER MANIFESTATIONS Joint symptoms - Arthralgia and arthritis Myalgia and myositis ENT : Sinusitis / Hearing Loss GI : GERD Resp : Interstitial pneumonitis, pulmonary fibrosis and pulmonary hypertension Nephro : Interstitial nephritis, Renal Tubular Acidosis Neuro : migraine, neuropathies, cerebral vasculitis
  13. 13. Sjogren’s Syndrome – Associations
  14. 14. Diagnosis  SS patients of both primary and secondary Sjögren’s syndrome have marked hypergammaglobulinemia (IgG>IgA>IgM),ANA(>50%) elevated total protein and sedimentation rate.  Anti-Ro and Anti-La Antibodies occur in approximately 60% of patients with Sjögren's syndrome  Histolgy of skin shows an absence of sebaceous glands and decrease in the sweat glands.
  15. 15.  Biopsy of labial salivary glands Ly lymphocytic and plasma cells infiltrate Two excretory ducts and 3 mucous salivary gland acini are seen
  16. 16. SCHIRMER’S TEST  German Ophthalmologist Otto Schirmer  determines whether the eye produces enough tears to keep it moist  This test is used when a person experiences very dry eyes or excessive watering of the eyes
  17. 17. SCHIRMER’S TEST  Schirmer's test places a small strip of filter paper inside the lower eyelids (conjunctival sac). The eyes are closed for 5 minutes. The paper is then removed and the amount of moisture is measured. This technique measures basic tear function.  A young person normally moistens 15 mm of each paper strip. Because hypolacrimation occurs with aging, 33% of normal elderly persons may wet only 10 mm in 5 minutes. Persons with Sjögren's syndrome moisten less than 5 mm in 5 minutes.  INTERPRETATION 1. Normal which is ≥15 mm wetting of the paper after 5 minutes 2. Mild which is 14-9 mm wetting of the paper after 5 minutes 3. Moderate which is 8-4 mm wetting of the paper after 5 minutes 4. Severe which is <4 mm wetting of the paper after 5 minutes.
  18. 18. ROSE BENGAL DYE
  19. 19. Revised classification criteria for Sjogren’s Syndrome  Ocular symptoms : at least one of -  Dry eyes for more than 3 months  Sensation of sand or gravel in the eyes  Need for tear substitutes more than 3 times a day  Oral Symptoms : at least one of –  Dry mouth for more than 3 months  Recurrently or Persistently swollen salivary glands  Need liquids to swallow dry food  Ocular Signs –at least one the following two tests positive  Schirmer’s test  Rose Bengal score
  20. 20. 4. Histopathology: in minor salivary glands, focal lymphocytic sialoadenitis (focus score ≥1). 5. Salivary gland involvement: a positive result for at least one of the following diagnostic tests: 1 Unstimulated whole salivary flow (≤1.5 ml in 15 min) 2 Parotid sialography showing punctate, cavitary, or destructive pattern, without evidence of obstruction in the major ducts 3 Salivary scintigraphy showing delayed uptake, reduced concentration 6. Autoantibodies – Anti Ro and Anti La
  21. 21. Criteria For primary SS  In patients without any potentially associated disease, primary SS may be defined as follows:  a. The presence of any four of the six items is indicative of primary SS, as long as either item 4 (Histopathology) or 6 (Serology) is positive.  b. The presence of any three of the four objective criteria items (that is, items 3, 4, 5, 6) For secondary SS  In patients with a potentially associated disease, the presence of item 1 or item 2 plus any two from among items 3, 4, and 5 may be considered as indicative of secondary SS
  22. 22. Treatment  Symptomatic treatment for dryness of eyes – by lubricating agents such as 0.5% methylcellulose eye drops for 4-5 times daily.  Cyclosporine ocular drops  Bromhexine 16 mg TDS has been found to increase the lacrimal secretion.  Artificial saliva and cyclosporin(2.5-5mg/kg), for xerostomia.  Routine dental care  Steam inhalation may help dryness of the respiratory tract.
  23. 23.  Candidiasis – topical nystatin 3times/day for a week  Systemic – ketoconazole 200-400mg/day or fluconazole 50-100 mg/day or itraconazole -100mg/day for 2 weeks  Systemic steroids are effective in reducing parotid swelling  hydroxychloroquine 200 mg daily is useful

Description

sjogren's syndrome by Dr.Aakanksha singh,Resident SKIN & VD

Transcript

  1. 1. SJOGREN’S SYNDROME Dr.Aakanksha Singh
  2. 2.  Sjogren’s syndrome is a late onset chronic systemic autoimmune disease characterized by lymphocytic infiltration and destruction of the salivary and lacrimal glands resulting in dry mouth(xerostomia) and dryness and atrophy of conjunctiva and cornea (keratoconjunctivits sicca).  Swedish Ophthalmologist Henrik Sjögren who first described it (1933) ;
  3. 3.  This disease is caused by an immune-mediated inflammation of salivary,lacrimal and sweat glands as Sicca Syndrome or with internal organ involvement. Clinical Types  PRIMARY SS - Alone.  SECONDARY SS - associated underlying connective tissue diseases (RA / SLE / Scleroderma )  SICCA SYNDROME – Xerophthalmia + Xerostomia – Internal Organ / Bone Inv
  4. 4. ETIOLOGY  Female : Male = 9 : 1  4/5/6th decade  Autoimmune ; HLA-B8 / DR3 ,although DR4 is more closely associated with syndrome occuring with raynaud’s phenomenon  Antibodies to the Ro antigen occur in excess in relatives of pts with sjogren’s syndrome.
  5. 5. Pathogenesis of Sjögren’s syndrome is believed to be multifactorial. Known to be autoimmune, but studies suggest that the disease process has genetic, environmental(EBV/HCV) and hormonal(associated with high prevalence in women,esp estrogen) components
  6. 6.  Lymphocyte and plasma cell infiltration  Auto- antibody production (to ‘Ro’)  Connective tissue proliferation  Glandular cell apoptosis  atrophy of glandular structures in affected tissues (salivary glands, sebaceous glands, sweat glands)  Secondary changes – oedema of conjuctiva
  7. 7. C/F  Glandular manifestation  Dry mouth (Xerostomia) due to decreased production of saliva by salivary glands Cracked, peeling and atrophic appearance of the lips. Dry and fissured tongue
  8. 8.  Teeth – multilpe carries and early loss
  9. 9.  Chronic oral candidiasis is frequent.  Parotid Gland Enlargement
  10. 10.  Lacrimal Gland Involvement – Dryness of eyes causes keratoconjuctivitis sicca.  Burning itchy sensation in the eyes.
  11. 11. SKIN MANIFESTATIONS (50%) Xeroderma, pruritus and scaling Annular erythema, Papular Erythema including Sweet’s-like lesions. Raynaud’s syndrome Hyperglobulinemic Purpura Vitiligo Sweating abnormalities Cutaneous Amyloidosis Alopecia—diffuse and generalized
  12. 12. OTHER MANIFESTATIONS Joint symptoms - Arthralgia and arthritis Myalgia and myositis ENT : Sinusitis / Hearing Loss GI : GERD Resp : Interstitial pneumonitis, pulmonary fibrosis and pulmonary hypertension Nephro : Interstitial nephritis, Renal Tubular Acidosis Neuro : migraine, neuropathies, cerebral vasculitis
  13. 13. Sjogren’s Syndrome – Associations
  14. 14. Diagnosis  SS patients of both primary and secondary Sjögren’s syndrome have marked hypergammaglobulinemia (IgG>IgA>IgM),ANA(>50%) elevated total protein and sedimentation rate.  Anti-Ro and Anti-La Antibodies occur in approximately 60% of patients with Sjögren's syndrome  Histolgy of skin shows an absence of sebaceous glands and decrease in the sweat glands.
  15. 15.  Biopsy of labial salivary glands Ly lymphocytic and plasma cells infiltrate Two excretory ducts and 3 mucous salivary gland acini are seen
  16. 16. SCHIRMER’S TEST  German Ophthalmologist Otto Schirmer  determines whether the eye produces enough tears to keep it moist  This test is used when a person experiences very dry eyes or excessive watering of the eyes
  17. 17. SCHIRMER’S TEST  Schirmer's test places a small strip of filter paper inside the lower eyelids (conjunctival sac). The eyes are closed for 5 minutes. The paper is then removed and the amount of moisture is measured. This technique measures basic tear function.  A young person normally moistens 15 mm of each paper strip. Because hypolacrimation occurs with aging, 33% of normal elderly persons may wet only 10 mm in 5 minutes. Persons with Sjögren's syndrome moisten less than 5 mm in 5 minutes.  INTERPRETATION 1. Normal which is ≥15 mm wetting of the paper after 5 minutes 2. Mild which is 14-9 mm wetting of the paper after 5 minutes 3. Moderate which is 8-4 mm wetting of the paper after 5 minutes 4. Severe which is <4 mm wetting of the paper after 5 minutes.
  18. 18. ROSE BENGAL DYE
  19. 19. Revised classification criteria for Sjogren’s Syndrome  Ocular symptoms : at least one of -  Dry eyes for more than 3 months  Sensation of sand or gravel in the eyes  Need for tear substitutes more than 3 times a day  Oral Symptoms : at least one of –  Dry mouth for more than 3 months  Recurrently or Persistently swollen salivary glands  Need liquids to swallow dry food  Ocular Signs –at least one the following two tests positive  Schirmer’s test  Rose Bengal score
  20. 20. 4. Histopathology: in minor salivary glands, focal lymphocytic sialoadenitis (focus score ≥1). 5. Salivary gland involvement: a positive result for at least one of the following diagnostic tests: 1 Unstimulated whole salivary flow (≤1.5 ml in 15 min) 2 Parotid sialography showing punctate, cavitary, or destructive pattern, without evidence of obstruction in the major ducts 3 Salivary scintigraphy showing delayed uptake, reduced concentration 6. Autoantibodies – Anti Ro and Anti La
  21. 21. Criteria For primary SS  In patients without any potentially associated disease, primary SS may be defined as follows:  a. The presence of any four of the six items is indicative of primary SS, as long as either item 4 (Histopathology) or 6 (Serology) is positive.  b. The presence of any three of the four objective criteria items (that is, items 3, 4, 5, 6) For secondary SS  In patients with a potentially associated disease, the presence of item 1 or item 2 plus any two from among items 3, 4, and 5 may be considered as indicative of secondary SS
  22. 22. Treatment  Symptomatic treatment for dryness of eyes – by lubricating agents such as 0.5% methylcellulose eye drops for 4-5 times daily.  Cyclosporine ocular drops  Bromhexine 16 mg TDS has been found to increase the lacrimal secretion.  Artificial saliva and cyclosporin(2.5-5mg/kg), for xerostomia.  Routine dental care  Steam inhalation may help dryness of the respiratory tract.
  23. 23.  Candidiasis – topical nystatin 3times/day for a week  Systemic – ketoconazole 200-400mg/day or fluconazole 50-100 mg/day or itraconazole -100mg/day for 2 weeks  Systemic steroids are effective in reducing parotid swelling  hydroxychloroquine 200 mg daily is useful

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