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Sjogren's SyndromeAka: Sjogren Syndrome, Keratoconjunctivitis Sicca, Dry Eye Syndrome

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  1. Epidemiology
    1. Incidence: 4 per 100,000
    2. Prevalence: 1-2 Million in United States
    3. Gender: Predominately women (93% in one study)
    4. Age of onset: Middle aged (mean age of onset 52 years old in one study)
    5. Garcia-Carrasco (2002) Medicine 81(4):270
  2. Causes and Associated Conditions
    1. Primary: Specific autoimmune disease (Sjogren's)
    2. Secondary associations (Keratoconjunctivitis sicca)
      1. Rheumatoid Arthritis (occurs in 25% of RA cases)
      2. Hypothyroidism (10-15%)
      3. Systemic Lupus Erythematosus
      4. Scleroderma
      5. Polymyositis
      6. Felty's Syndrome
      7. Pulmonary Fibrosis
  3. Etiology
    1. Environmental stimulus triggers autoimmune reaction directed at exocrine glands
    2. Postulated triggers: CMV and EBV
    3. Association with HLA-DR haplotype
    4. B-Lymphocyte mediated exocrine gland destruction
  4. Pathophysiology: Chronic destruction of exocrine glands
    1. Lacrimal glands affected in Keratoconjunctivitis sicca
    2. All exocrine glands affected in Sjogren's Syndrome
      1. Salivary Glands
      2. Lacrimal glands
      3. Sebaceous Glands
      4. Vaginal glands
  5. Symptoms and Signs: Most Common (93-98% of patients at presentation)
    1. Xerostomia (Dry Mouth)
      1. Frequent sips of water
      2. May impact speaking, eating and swallowing
      3. Exam demonstrates dry mucus membranes (secondary redness and ulcers may be present)
      4. Dental Caries are more common
      5. Parotid Glands tender to touch in some cases
    2. Xerophthalmia (Dry Eyes): Keratoconjunctivitis sicca
      1. Symptoms worse toward latter part of day
      2. Foreign Body sensation
      3. Painful or itching eyes
      4. Conjunctival injection
      5. Keratoconjunctivitis
      6. Corneal clouding in severe cases
  6. Symptoms and Signs: Other Associations
    1. Gastrointestinal
      1. Dyspepsia
      2. Altered stool habits
    2. Genitourinary
      1. Renal conditions (e.g. Glomerulonephritis, Interstitial Nephritis, Renal Tubular Acidosis)
    3. Hematologic
      1. Lymphadenopathy
    4. Musculoskeletal
      1. Non-erosive arthritis
    5. Neurologic
      1. Peripheral Neuropathy
    6. Respiratory
      1. Nasal dryness
      2. Sinusitis
      3. Chronic Cough or recurrent Bronchitis
      4. Imaging
        1. XRay may show chronic Interstitial Infiltrates
        2. CT Chest may demonstrate alveolitis or fibrosis
    7. Skin
      1. Dry Skin
      2. Cutaneous Vasculitis
      3. Raynaud's Phenomenon
    8. Constitutional and other effects
      1. Fatigue
      2. Major Depression
      3. Fever
  7. Diagnosis: Revised International Classification Criteria (4 of 6 criteria required)
    1. Lip Salivary Gland biopsy positive (see diagnostics below)
    2. Autoantibodies Anti-Ro (Anti-SSA) and Anti-La (Anti-SSB) present
    3. Ocular symptoms: One or more of the following
      1. Dry Eyes sensation everyday for at least 3 months
      2. Recurrent Eye Foreign Body sensation (sand or gravel sensation)
      3. Artificial tears instilled more than 3 times daily
    4. Ocular signs: One or more of the following
      1. Schirmer Test
      2. Rose Bengal Test (or other ocular dye test)
    5. Oral symptoms: One or more of the following
      1. Dry Mouth sensation everyday for at least 3 months
      2. Salivary Gland swelling recurrent or persistent
      3. Dry food intake requires frequent swallowing of liquids
    6. Oral signs: One or more of the following demonstrating Salivary Gland involvement
      1. Nonstimulated Whole Salivary Flow Collection (<1.5 ml in 15 minutes)
      2. Contrast parotid sialography demonstrates diffuse sialectasia
      3. Salivary scintigraphy with delayed uptake and decreased concentration and excretion
    7. Vitali (2002) Ann Rheum Dis 61(6):554
  8. Diagnostics: Dry eye evaluation
    1. Schirmer's Test
    2. Rose Bengal Test
    3. Slit Lamp Exam of Cornea
      1. Decreased tear meniscus
      2. Punctate erosive keratopathy
  9. Diagnostics: Dry Mouth evaluation
    1. Nonstimulated Whole Salivary Flow Collection
    2. Minor Salivary Gland biopsy from lip (pathognomonic findings)
      1. Acinar gland lymphocytic infiltration with secondary degeneration, necrosis, atrophy
      2. Positive biopsy: one or more foci of dense inflammatory infiltrate >50 lymphs/4mm
  10. Labs: Serology
    1. Rheumatoid Factor positive (32-90% positive)
    2. Antinuclear Antibody positive (55-97% positive)
      1. Autoantibody Anti-Ro (Anti-SSA) present (16-70%)
      2. Autoantibody Anti-La (Anti-SSB) present (7-50%)
    3. Garcia-Carrasco (2002) Medicine 81(4):270
  11. Labs: Other
    1. Erythrocyte Sedimentation Rate elevated
    2. Complete Blood Count
      1. Leukopenia
      2. Anemia
    3. Liver Function Tests abnormal
  12. Management: General
    1. Dry Eyes (Xerophthalmia)
      1. See Dry Eyes for management
    2. Dry Mouth (Xerostomia)
      1. See Dry Mouth for Management
    3. Dry nose
      1. Saline Nasal Spray
      2. Humidifier
    4. Dry Skin
      1. Skin moisturizing creams and ointments after shower
    5. Vaginal dryness
      1. Vaginal lubricants
      2. Estrogen Replacement Therapy
      3. Vaginal Estrogen cream
    6. Infection
      1. Observe for Thrush or yeast Vaginitis
  13. Management: Systemic Medications
    1. Dry Eyes and Dry Mouth
      1. See Muscarinic Agonists (e.g. Pilocarpine)
    2. Arthralgias
      1. NSAIDs
      2. Hydroxychloroquine (Plaquenil)
      3. Corticosteroids
    3. Monoclonal antibodies
      1. Rituximab (Rituxan): anti-CD20 - decreases B-Lymphocyte activity
  14. Complications
    1. Non-Hodgkin Lymphoma (40 fold increased risk over general population)
    2. Dental Caries
    3. Sialadenitis
    4. Corneal Ulceration
    5. Chronic oral infection
  15. References
    1. Fox (2005) Lancet 366(9482):321
    2. Kassan (2004) Arch Intern Med 164(12):1275
    3. Kruszka (2009) Am Fam Physician 79(6):465

Dry Eye Syndromes (C0013238)

Definition (MSH)Corneal and conjunctival dryness due to deficient tear production, predominantly in menopausal and post-menopausal women. Filamentary keratitis or erosion of the conjunctival and corneal epithelium may be caused by these disorders. Sensation of the presence of a foreign body in the eye and burning of the eyes may occur.
ConceptsDisease or Syndrome (T047)
ICD9375.15, 375.15
MSHD015352
EnglishDry eye syndrome, Dry Eye Syndromes, Tear film insufficiency
Spanishinsuficiencia de la pelicula lagrimal, sindrome del ojo seco
Parent ConceptsOther lacrimal gland disorders (C0155226), Lacrimal Apparatus Diseases (C0022904), Syndrome (C0039082), Non-Neoplastic Eye Disorder (C1335009), Ocular surface finding (C0423176), Disorder of anterior segment of eye (C0730121), Duplicate concept (C1274013)
SourcesICD9CM, MSH, MTH, MTHICD9, NCI, NDFRT, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Keratoconjunctivitis Sicca (C0022575)

Definition (MSH)Drying and inflammation of the conjunctiva as a result of insufficient lacrimal secretion. When found in association with XEROSTOMIA and polyarthritis, it is called SJOGREN'S SYNDROME.
Definition (AIR)WHAT: Keratoconjunctivitis sicca. Keratoconjunctivitis Sicca (KCS): an eye condition in which there is decreased tear production and inflammation of the cornea and conjunctiva. WHY: Keratoconjunctivitis sicca is found in a significant number of patients with rheumatoid arthritis. KCS has also been described in systemic lupus erythematosus, polyarteritis nodosa, and scleroderma. KCS plus xerostomia is called the sicca syndrome. The association of the sicca syndrome with rheumatoid arthritis is known as Sjogren's syndrome. HOW: Clinically, patients with KCS may present with itching, burning eyes. They may complain of a dry sensation or of a foreign body sensation of the eyes. The conjunctiva is hyperemic, and thick strands of mucus may cover the eye. With slit lamp examination there is less than 1 mm of tear width at the margins of the upper and lower lids. There is increased debris and mucus strands within the tear film, seen best after blinking. Tiny punctate opacities are seen throughout the corneal surface. The Schirmer tear test (which is most accurately performed without topical anesthesia) shows less than 5 mm of wetting of filter paper at the end of 5 minutes. Finally, rose bengal, which is a water soluble dye specific for devitalized cells and mucin, will stain the dessicated corneal and conjunctival cells a bright red color. REFS: 1) Henkind, P and Gold, DH: Ocular manifestations of rheumatic disorders. Rheumatology 4:13, 1973. 2) Havener, W: Synopsis of ophthamology. St. Louis, C.V. Mosby Company, 1979. 3) Anderson, JR; Whaley, K; Williams, J and Buchanan, WW: A statistical aid to the diagnosis of keratoconjunctivitis sicca. Quart J Med 41:175, 1972. DN19292-9.
Definition (CSP)drying and inflammation of the conjunctiva as a result of insufficient lacrimal secretion.
ConceptsDisease or Syndrome (T047)
ICD9710.2
MSHD007638
EnglishKCS, KCS - Keratoconjunctivitis sicca, Keraconjunctivitis sicca, Kerato conjunctivitis sicca, Keratoconjunctivitis Sicca
Spanishqueratoconjuntivitis sicca
Parent ConceptsEye Manifestations (C0015411), Eyes and vision (C0150933), Keratoconjunctivitis (C0022573), Dry Eye Syndromes (C0013238)
SourcesAIR, COSTAR, CSP, DXP, LCH, MSH, MTH, MTHICD9, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Sjogren's Syndrome (C1527336)

Definition (MSH)Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both KERATOCONJUNCTIVITIS SICCA and XEROSTOMIA. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis.
Definition (CSP)symptom complex of unknown etiology, usually occurring in middle-aged or older women, marked by the triad of keratoconjunctivitis sicca with or without lacrimal gland enlargement, xerostomia with or without salivary gland enlargement, and the presence of a connective tissue disease, usually rheumatoid arthritis but sometimes systemic lupus erythematosus, scleroderma, or polymyositis; an abnormal immune response has been implicated.
ConceptsDisease or Syndrome (T047)
ICD9710.2
MSHD012859
EnglishGOUGEROUT-SJOEGREN SYNDROME, SJOEGREN SYNDROME, Sjogren Syndrome, Sjogren's disease, Sjogren's Syndrome, SJOGRENS SYNDROME, SYNDROME SJOGREN'S
Spanishenfermedad de Sjogren, sindrome de Sjogren
Parent ConceptsAutoimmune Diseases (C0004364), Collagen Diseases (C0009326), Keratoconjunctivitis Sicca (C0022575), Syndrome (C0039082), Rheumatoid Arthritis (C0003873), Dry Eye Syndromes (C0013238), Xerostomia (C0043352), Salivary Gland Diseases (C0036093), Endophthalmitis (C0014236), Dacryoadenitis (C0155223), Disorder of salivary structure (C0560921), Inflammation of specific body organs (C1285331), Inflammatory disorder of digestive tract (C1290888)
SourcesCOSTAR, CSP, CST, DXP, LCH, MEDLINEPLUS, MSH, MTH, MTHICD9, NCI, NDFRT, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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