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Sjögren’s syndrome
ALI NAJAT JABBAR
KIRKUK MEDICAL COLLAGE
Introduction
 a long-term autoimmune disease that affect lacrimal and salivary
glands.
 Characterised by lymphatic infiltration of salivary and lacrimal
glands leading to fibrosis and exocrine failure.
 The typical age of onset is between 40-50.
 9:1 female to male ratio.
Clinical features
1- Keratoconjunctivitis sicca
2- Xerostomia
3- Salivary gland enlargement
4- Rashes and skin irritation
5- Non erosive arthralgia
6- Generalised osteoarthritis
7- Raynaud’s phenomenon
8- Fatigue
9- Low grade fever
10- Interstitial lung disease
11- Anemia, leucopenia and thrombocytopenia
12- Cryoglobulinemia
13- Vasculitis
14- peripheral neuropathy
15- Lymphadenopathy
16- Lymphoreticular lymphoma
17- Glomerulonephritis
18- Interstitial nephritis
19- Renal tubular acidsis
Investigations
1. Schirmer tear test: which measures tear flow over 5 minutes using
absorbent paper strips placed on the lower eyelid, normal result is
more than 6mm of wetting.
2. Salivary gland biopsy: demonstrating focal lymphocytic infiltrate.
3. Most patients have an elevated ESR and hypergammaglobulinemia.
4. Autoantibodies frequently detected including: rheumatoid factor,
antinuclear antibody, anti-Ro and anti-Lo.
5. Chest x-ray and lung function test should be performed.
Note: antinuclear antibody negative disease exists.
Management
 Lacrimal substitutes such as hypromellose should used during the
day in combination with more viscous lubricating application at
night.
 Soft contact lenses for corneal protection in patients with
filamentary keratitis.
 Artificial saliva sprays, saliva-stimulating tablets, and pastilles and
oral gels can be tried for xerostomia but often chewing gum is most
effective.
 Postprandial oral hygiene and prompt treatment of oral candidiasis.
Management
 A trial of systematic pilocarpine(5-30mg in divided doses) is worthwhile in
early disease amplify glandular function.
 Hydroxychloroquine(200mg twice daily) is often used to address skin and
musculoskeletal features and may help fatigue.
 Immunosuppression dose not improve sicca symptoms but is essential for
progressive interstitial lung disease( glucocorticoid and cyclophosphamide)
and for interstitial nephritis (if hydroxychloroquine is ineffective alone)
 If non resolving lymphadenopathy or salivary gland enlargement develops,
biopsy should be undertaken to exclude malignancy.
Associated autoimmune disorder
1. Systemic lupus erythematous
2. Systemic sclerosis
3. Celiac disease
4. Primary biliary cholangitis
5. Chronic active hepatitis
6. Myasthenia gravis

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Sjogren's syndrome

  • 1. Sjögren’s syndrome ALI NAJAT JABBAR KIRKUK MEDICAL COLLAGE
  • 2. Introduction  a long-term autoimmune disease that affect lacrimal and salivary glands.  Characterised by lymphatic infiltration of salivary and lacrimal glands leading to fibrosis and exocrine failure.  The typical age of onset is between 40-50.  9:1 female to male ratio.
  • 3. Clinical features 1- Keratoconjunctivitis sicca 2- Xerostomia 3- Salivary gland enlargement 4- Rashes and skin irritation 5- Non erosive arthralgia 6- Generalised osteoarthritis 7- Raynaud’s phenomenon 8- Fatigue 9- Low grade fever 10- Interstitial lung disease 11- Anemia, leucopenia and thrombocytopenia 12- Cryoglobulinemia 13- Vasculitis 14- peripheral neuropathy 15- Lymphadenopathy 16- Lymphoreticular lymphoma 17- Glomerulonephritis 18- Interstitial nephritis 19- Renal tubular acidsis
  • 4. Investigations 1. Schirmer tear test: which measures tear flow over 5 minutes using absorbent paper strips placed on the lower eyelid, normal result is more than 6mm of wetting. 2. Salivary gland biopsy: demonstrating focal lymphocytic infiltrate. 3. Most patients have an elevated ESR and hypergammaglobulinemia. 4. Autoantibodies frequently detected including: rheumatoid factor, antinuclear antibody, anti-Ro and anti-Lo. 5. Chest x-ray and lung function test should be performed. Note: antinuclear antibody negative disease exists.
  • 5. Management  Lacrimal substitutes such as hypromellose should used during the day in combination with more viscous lubricating application at night.  Soft contact lenses for corneal protection in patients with filamentary keratitis.  Artificial saliva sprays, saliva-stimulating tablets, and pastilles and oral gels can be tried for xerostomia but often chewing gum is most effective.  Postprandial oral hygiene and prompt treatment of oral candidiasis.
  • 6. Management  A trial of systematic pilocarpine(5-30mg in divided doses) is worthwhile in early disease amplify glandular function.  Hydroxychloroquine(200mg twice daily) is often used to address skin and musculoskeletal features and may help fatigue.  Immunosuppression dose not improve sicca symptoms but is essential for progressive interstitial lung disease( glucocorticoid and cyclophosphamide) and for interstitial nephritis (if hydroxychloroquine is ineffective alone)  If non resolving lymphadenopathy or salivary gland enlargement develops, biopsy should be undertaken to exclude malignancy.
  • 7. Associated autoimmune disorder 1. Systemic lupus erythematous 2. Systemic sclerosis 3. Celiac disease 4. Primary biliary cholangitis 5. Chronic active hepatitis 6. Myasthenia gravis