Rheumatology Book

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Sjogren's SyndromeAka: Keratoconjunctivitis Sicca, Dry Eye Syndrome

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  1. Epidemiology
    1. Prevalence: 2-3 Million in United States
    2. Women predominantly affected
  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. 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
  4. Symptoms and Signs
    1. 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
    2. Other exocrine effects
      1. Xerostomia (Dry Mouth)
      2. Dry Skin
      3. Nasal dryness
      4. Sinusitis
      5. Bronchitis
    3. Constitutional and other effects
      1. Fatigue
      2. Major Depression
  5. Diagnostics
    1. Schirmer's Test
    2. Slit Lamp Exam of Cornea
      1. Punctate erosive keratopathy
  6. Labs
    1. Rheumatoid Factor positive
    2. Antinuclear Antibody positive
      1. Autoantibody Anti-Ro (Anti-SSA) present
      2. Autoantibody Anti-La (Anti-SSB) present
    3. Erythrocyte Sedimentation Rate elevated
    4. Complete Blood Count
      1. Leukopenia
      2. Anemia
    5. Liver Function Tests abnormal
  7. Management: General
    1. Dry eyes (Xerophthalmia)
      1. Sunglasses
      2. Room humidifiers
      3. Artificial tears or ointments
      4. Surgical lacrimal drainage occlusion in severe cases
    2. Dry Mouth (Xerostomia)
      1. See Dentist regularly
      2. Salivary substitutes
      3. Directly apply fluoride to teeth and in Toothpaste
    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
  8. Management: Systemic Medications
    1. Pilocarpine (for Sjogren's eye and mouth symptoms)
      1. Dose: 5 mg PO qid
      2. Causes diaphoresis and decreased night vision
    2. NSAIDs
    3. Hydroxychloroquine
    4. Corticosteroids
    5. Immunosuppressants
      1. Methotrexate
      2. Imuran
      3. Cyclosporin
      4. Cytoxan

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)



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