Updated: 11/13/2019

Sjogren Syndrome

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Snapshot
  • A 50-year-old woman presents to her primary care physician's office for persistently dry eyes and mouth. She reports that this started several months ago and has not improved. She has been using artificial tears for the past few weeks without much relief. She also reports feeling very tired and anxious. She has a past medical history of systemic lupus erythematosus and a family history of rheumatoid arthritis. Physical exam reveals dry mucous membranes, swollen parotid glands, and conjunctivitis on exam. She is prescribed pilocarpine and sent for an autoimmune workup.
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
  • Physical exam
    • cutaneous findings
      • dry skin
      • cheilitis
    • ocular findings
      • conjunctival injection
    • oral findings
      • dental caries or periodontal disease
      • dry mucous membranes
    • bilateral parotid gland enlargement
Studies
  • Labs 
    • positive anti-SSA (anti-Ro) (50-70% of cases)  
    • positive anti-SSB (anti-La) (50-70% of cases)
    • 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 (MALT) lymphoma
  • Parotid gland infection

 

 

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Questions (3)
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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(M1.MK.202) A 48-year-old female presents to your office with a 1-year history of dry eyes and difficulty swallowing. She complains of blinking frequently and of eye strain while using her computer at work. She also reports stiffness in her knees and lower back. Past medical history is unremarkable and she does not take medications. She denies cigarette or alcohol use. Family history is notable for Hashimoto's thyroiditis in her mother. Physical exam shows dry oral mucosa and enlargement of the parotid glands. Which of the following serologies is likely to be positive in this patient? Review Topic

QID: 106236
1

Anti-SS-A

74%

(106/143)

2

Anti-CCP

3%

(4/143)

3

Anti-dsDNA

3%

(5/143)

4

Anti-Smith

6%

(8/143)

5

Anti-Jo-1

13%

(18/143)

M1

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