Updated: 10/10/2017

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)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M2.RH.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? Review Topic

QID: 109270
1

Macrosomia

9%

(6/66)

2

Neonatal hypoglycemia

5%

(3/66)

3

Heart block

44%

(29/66)

4

Pulmonary hypertension

14%

(9/66)

5

Meconium aspiration

26%

(17/66)

M2

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SUBMIT RESPONSE 3

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(M2.RH.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? Review Topic

QID: 109175
FIGURES:
1

Anti-microsomal

0%

(0/0)

2

Anti-nuclear

0%

(0/0)

3

Anti-DNA topoisomerase I

0%

(0/0)

4

Anti-Ro

0%

(0/0)

5

Rheumatoid factor

0%

(0/0)

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