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Sjogren's Syndrome
Aka: Sjogren's Syndrome, Sjogrens Syndrome, Sjogren Syndrome, Keratoconjunctivitis Sicca, Dry Eye Syndrome- Epidemiology
- Incidence: 4 per 100,000
- Prevalence: 1-2 Million in United States
- Gender: Predominately women (93% in one study)
- Age of onset: Middle aged (mean age of onset 52 years old in one study)
- Garcia-Carrasco (2002) Medicine 81(4): 270-80 [PubMed]
- Causes and Associated Conditions
- Primary: Specific Autoimmune Disease (Sjogren's)
- Secondary associations (Keratoconjunctivitis Sicca)
- Rheumatoid Arthritis (occurs in 25% of RA cases)
- Hypothyroidism (10-15%)
- Systemic Lupus Erythematosus
- Scleroderma
- Polymyositis
- Felty's Syndrome
- Pulmonary Fibrosis
- Pathophysiology
- Environmental stimulus triggers autoimmune reaction directed at exocrine glands
- Postulated triggers: CMV and EBV
- Association with HLA-DR haplotype
- B-Lymphocyte mediated chronic exocrine gland destruction
- Lacrimal glands affected in Keratoconjunctivitis Sicca
- All exocrine glands affected in Sjogren's Syndrome
- Salivary Glands
- Lacrimal glands
- Sebaceous Glands
- Vaginal glands
- Symptoms: Most Common (93-98% of patients at presentation)
- Xerostomia (Dry Mouth)
- Frequent sips of water
- May impact speaking, eating and swallowing
- Exam demonstrates dry mucus membranes (secondary redness and ulcers may be present)
- Dental Caries are more common
- Parotid Glands tender to touch in some cases
- Xerophthalmia (Dry Eyes): Keratoconjunctivitis Sicca
- Symptoms worse toward latter part of day
- Foreign Body sensation
- Painful or itching eyes
- Conjunctival injection
- Keratoconjunctivitis
- Corneal clouding in severe cases
- Xerostomia (Dry Mouth)
- Symptoms: Other Associations
- Gastrointestinal
- Dyspepsia
- Altered stool habits
- Genitourinary
- Renal conditions (e.g. Glomerulonephritis, Interstitial Nephritis, Renal Tubular Acidosis)
- Hematologic
- Musculoskeletal
- Non-erosive Arthritis
- Neurologic
- Respiratory
- Nasal dryness
- Sinusitis
- Chronic Cough or recurrent Bronchitis
- Imaging
- XRay may show chronic Interstitial Infiltrates
- CT Chest may demonstrate alveolitis or fibrosis
- Skin
- Dry Skin
- Cutaneous Vasculitis
- Raynaud's Phenomenon
- Constitutional and other effects
- Gastrointestinal
- Diagnosis: Revised International Classification Criteria (4 of 6 criteria required)
- Lip Salivary Gland biopsy positive (see diagnostics below)
- Autoantibodies Anti-Ro (Anti-SSA) and Anti-La (Anti-SSB) present
- Ocular symptoms: One or more of the following
- Dry Eyes sensation everyday for at least 3 months
- Recurrent Eye Foreign Body sensation (sand or gravel sensation)
- Artificial tears instilled more than 3 times daily
- Ocular signs: One or more of the following
- Schirmer Test
- Rose Bengal Test (or other ocular dye test)
- Oral symptoms: One or more of the following
- Dry Mouth sensation everyday for at least 3 months
- Salivary Gland swelling recurrent or persistent
- Dry food intake requires frequent swallowing of liquids
- Oral signs: One or more of the following demonstrating Salivary Gland involvement
- Nonstimulated Whole Salivary Flow Collection (<1.5 ml in 15 minutes)
- Contrast parotid sialography demonstrates diffuse sialectasia
- Salivary scintigraphy with delayed uptake and decreased concentration and excretion
- References
- Diagnostics: Dry Eye evaluation
- Schirmer's Test
- Rose Bengal Test
- Slit Lamp Exam of Cornea
- Decreased tear meniscus
- Punctate erosive keratopathy
- Diagnostics: Dry Mouth evaluation
- Nonstimulated Whole Salivary Flow Collection
- Minor Salivary Gland biopsy from lip (pathognomonic findings)
- Acinar gland lymphocytic infiltration with secondary degeneration, necrosis, atrophy
- Positive biopsy: one or more foci of dense inflammatory infiltrate >50 lymphs/4mm
- Labs: Serology
- Rheumatoid Factor positive (32-90% positive)
- Antinuclear Antibody positive (55-97% positive)
- ANA Test Sensitivity: 48%
- ANA Test Specificity: 52%
- Positive and Negative Likelihood Ratios approach 1.0
- Sjogren Antibodies
- Autoantibody Anti-Ro (Anti-SSA) present (16-70%)
- Autoantibody Anti-La (Anti-SSB) present (7-50%)
- Increased risk of Sjogren Syndrome when antibodies present in Rheumatoid Arthritis
- Increased risk of cytopenias, Discoid Lupus, Lupus Nephritis when antibodies present in SLE
- References
- Labs: Other
- Management: General
- Dry Eyes (Xerophthalmia)
- See Dry Eyes for management
- Dry Mouth (Xerostomia)
- See Dry Mouth for Management
- Dry nose
- Saline Nasal Spray
- Humidifier
- Dry Skin
- Skin moisturizing creams and ointments after shower
- Vaginal Dryness
- Infection
- Observe for Thrush or Yeast Vaginitis
- Dry Eyes (Xerophthalmia)
- Management: Systemic Medications
- Dry Eyes and Dry Mouth
- See Muscarinic Agonists (e.g. Pilocarpine)
- Arthralgias
- Monoclonal antibodies
- Rituximab (Rituxan): anti-CD20 - decreases B-Lymphocyte activity
- Dry Eyes and Dry Mouth
- Complications
- Non-Hodgkin Lymphoma (40 fold increased risk over general population)
- Dental Caries
- Sialadenitis
- Corneal Ulceration
- Chronic Oral Infection
- References