Updated: 11/17/2020

Sjogren Syndrome

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Snapshot
  • A 60-year-old man presents to his physician’s office for fatigue, dry mouth, and dry eyes for the past year. He reports having always had a history of dry eyes that is managed with artificial tears. A year ago, he started feeling thirsty and was evaluated for diabetes, which was ruled out. He reports that he had several dental caries at his last dental appointment, despite maintaining overall good oral hygiene habits. Physical exam reveals bilateral swollen parotid glands, conjunctivitis, and dry mucous membranes. He also has dental caries. He is sent for additional workup for suspicion of an autoimmune disease.
Introduction
  • Clinical definition
    • an autoimmune disease of the exocrine glands
  • Epidemiology
    • prevalence
      • relatively common (up to 5% prevalence)
    • demographics
      • female > male
      • 40-60 years of age
      • second most common autoimmune rheumatic disease
    • risk factors
      • family history
      • comorbid autoimmune disease
  • Pathogenesis
    • associated with HLA-DR52 
    • environmental trigger, such as a viral infection, may cause
      • inflammatory destruction of exocrine glands and is characterized by aggregation of lymphocytes, primarily CD4+ T-cells and memory cells
        • the salivary and lacrimal glands are most commonly affected
    • glandular neurodegeneration from apoptosis mediated by cytokines and inflammatory cells
  • Associated conditions
    • other autoimmune diseases
      • primary biliary cholangitis
      • CREST syndrome
      • rheumatoid arthritis
      • systemic lupus erythematosus
    • marginal zone lymphoma
    • viral infections
      • hepatitis C virus
      • EBV
      • HIV
  • Prognosis
    • patients are at risk for non-Hodgkin lymphoma and other autoimmune diseases
Presentation
  • Symptoms
    • sicca symptoms in > 95% of patients
      • ↓ saliva production causing dry mouth (xerostomia)
      • ↓ tear production causing dry eyes (keratoconjunctivitis sicca or xerophthalmia)
        • foreign body sensation
      • dyspareunia in women
    • constitutional symptoms
      • fatigue
      • weight loss
      • myalgias
      • joint pain
  • Physical exam
    • cutaneous findings
      • may have cutaneous vasculitis
        • flat or palpable purpura
        • urticarial vasculitis
      • dry skin
      • cheilitis
    • ocular findings
      • conjunctival injection
    • oral findings
      • dental caries or periodontal disease
      • dry mucous membranes
    • bilateral parotid gland enlargement
Imaging
  • Salivary gland scintigraphy or contrast sialography
    • indication
      • to evaluate the salivary gland function
Studies
  • Labs
    • positive anti-SSA (anti-Ro) (50-70% of cases)
    • positive anti-SSB (anti-La) (50-70% of cases)
      • anti-Ro/La can cross the placenta and cause neonatal heart block in infants of women with Sjogren syndrome 
    • positive antinuclear antibodies (50-90% of cases)
    • possible positive rheumatoid factor (30-90% of cases)
  • Corneal staining to assess ocular damage
  • Labial salivary gland biopsy
    • indication
      • to confirm diagnosis, especially in patients with negative anti-Ro or anti-La antibodies
    • findings
      • dense inflammatory infiltrate in exocrine glands
  • Making the diagnosis
    • based on clinical presentation and laboratory studies
Differential
  • Diabetes
  • Hepatitis C
  • Anticholinergic drug use
  • Mumps
Treatment
  • Medical
    • artificial tears
      • indication
        • dry eyes
    • pilocarpine or cevimeline
      • indication
        • dry eyes or dry mouth
    • vitamin D supplementation         
      • indications
        • for all patients
        • vitamin D deficiency may increase risk of neuropathy and lymphoma
Complications
  • Dental caries
  • Oral candidiasis
  • Corneal abrasion or ulceration
  • Mucosa-associated lymphoid tissue lymphoma
  • Parotid gland infection
 

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Questions (2)

(M2.RH.17.4799) A 29-year-old G2P1001 presents to her obstetrician’s office complaining of dyspareunia. She endorses ongoing vaginal dryness resulting in uncomfortable intercourse over the last month. In addition, she has noticed a gritty sensation in her eyes as well as difficulty tasting food and halitosis. She denies pain with urination and defecation. Her medications include a daily multivitamin, folic acid, and over-the-counter eye drops. The patient’s temperature is 98.6°F (37.0°C), pulse is 70/min, blood pressure is 121/80 mmHg, and respirations are 13/min. Physical exam is notable for a well-appearing female with fullness in the bilateral cheeks and reduced salivary pool. For which of the following is the patient’s fetus at increased risk? Tested Concept

QID: 109270
1

Macrosomia

8%

(6/71)

2

Neonatal hypoglycemia

4%

(3/71)

3

Heart block

48%

(34/71)

4

Pulmonary hypertension

13%

(9/71)

5

Meconium aspiration

24%

(17/71)

M 8 D

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(M2.RH.17.4796) A 38-year-old G1P1 female arrives to your clinic complaining of pain during sexual intercourse. She reports that the discomfort has been happening for months, and despite a normal libido, it has been putting a strain on her relationship with her fiancé. The patient denies urinary symptoms but reports some vaginal itching. Her past medical history is significant for type I diabetes mellitus, generalized anxiety disorder, and Raynaud’s phenomenon. Her medications include insulin and citalopram. Her last menstrual period was two weeks ago. She smokes half a pack of cigarettes a day and marijuana two times a week to help relieve chronic joint pain. Physical exam is notable for dental abnormalities shown in Figure A. A pelvic exam is within normal limits, but the patient’s vaginal mucosa appears dry and mildly erythematous. There is minimal yellow-white discharge. Which of the following auto-antibodies is most likely to confirm the diagnosis? Tested Concept

QID: 109175
FIGURES:
1

Anti-microsomal

0%

(0/0)

2

Anti-nuclear

0%

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3

Anti-DNA topoisomerase I

0%

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Anti-Ro

0%

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5

Rheumatoid factor

0%

(0/0)

M 6 D

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