Sjogrens Syndrome

Question 1

What is Sjögren’s syndrome?

Answer 1

1. Autoimmune disorder
2. There will be destructions of exocrine glands
   *will be dryness
3. Sicca syndrome (isolated dry eyes and salivary glands)

Question 2

What is the general population of Sjogren syndrome and prevalence?

Answer 2

1. Rare 1% of the population
2. Can occur in 10-15% of RA patients
3. 9 to 1 F>M
   *in the 30’s and 50’s most occur between 40-60

Question 3

What will be at an increased risk if a patient has SS?

Answer 3

Increased risk of lymphomas

Question 4

What causes Sjorgrens ?

Answer 4

1. A combination of genetic and environmental factors
2. Genetics:
   *HLA-DR correlates with +ANA and +Anti-SS-a (Ro) antibody

Question 5

What are the clinical manifestations of SS?

Answer 5

1. Xerophtalmia
2. Keratoconjunctivitis sicca
3. Xerostomia
4. Parotid enlargement

Question 6

What can be used for SS diagnostic tests?

Answer 6

1. Lip biopsy
2. Schirmer test (seeing the amount of tears present)
3. Labs
   *RF (+70%) and ANA (+95%)
   *SS-A/Ro (+) (most helpful)
   *SS-B/La (+) (most helpful)
   *CBC/diff (anemia)

Question 7

What are the neck and head manifestations of SS?

Answer 7

1. 80% complaints of xerostomia
2. Difficulty chewing
3. Dysphasia
4. Taste changes
5. Fissures of the tongue and lips
6. Increased dental caries
7. Oral candidiasis
8. Salivary gland enlargement

Question 8

Non-Salivary gland disease Ddx

Answer 8

1. Drugs
   *BP and cardiac
2. Acute anxiety and depression
3. Mouth breathing
4. Central lesions
   *MS
   *Alzheimers

Question 9

Salivary gland Ddx

Answer 9

1. Hepatitis C
2. Sarcoidosis
3. Fatty infiltrate of gland (change to the gland itself)
4. HIV disease
5. Lymphoma

Question 10

What are the eye complaints of SS?

Answer 10

1. Dryness
2. Burning
3. Itching
4. Foreign body sensation
5. Keratoconjunctivitis sicca
   *corneal abrasions

Question 11

What other manifestations can occur due to SS?

Answer 11

1. Skin vasculitis
2. Fatigue
3. Cognitive loss
4. Skin-dry
5. Lung- interstitial pneumonitis
6. Cardiac pulmonary hypertension

Question 12

What is the titer of ANA in SS?

Answer 12

1:80
*80 dilutions took place and you can still see the ANA

Question 13

What is the gold standard of testing for SS?

Answer 13

Lip biopsy
*still can be misread by pathologists

Question 14

What are the labs that you should order when suspecting SS?

Answer 14

1. CBC
2. ESR, CRP
3. Chemistry panel
4. UA
5. Serologies
   *ANA, Anti-SSA/Ro, anti-SSB/La, Rf as initial screen
   *test for vitamin A deficiency
6. Minor salivary gland biopsy
   *to confirm dx of SS in patients lacking or low titer anti-SSA/Ro antibodies

Question 15

What is the treatment of SS?

Answer 15

1. Oral fluid intake
2. Salvia substitutes
3. Artificial tears

Question 16

What should a patient with SS avoid?

Answer 16

1. Decongestants
2. Antihistamines
3. Diuretics
4. Anticholinergic

Question 17

What are common pharmacological treatments of SS?

Answer 17

1. Pilocarpine (cholinergic) for xerostomia
   *will increase lacrimation and salivation
2. Clotrimazole/nystatin (for thrush)
3. Cervimeline (Exovac)
   *stimulates muscarinic cholinergic receptors
   *increases the production of saliva

Question 18

What are the side effects of Pilocarpine?

Answer 18

1. Diaphoresis
2. Flushing
3. Sweating
4. Bradycardia
5. Diarrhea
   6.N/V
6. Incontinence
7. Blurred vision

Question 19

If progresses to systemic problems what treatment can you use?

Answer 19

1. Steroids
   *They work but once you start them, you’re on them for life
2. Hydroxychloroquine or methotrexate
   *For rash or arthralgia
3. Immunosuppressive tx
   *make sure that immunizations are up-to-date

Question 20

When should you refer and admit a patient with SS?

Answer 20

1. Refer
   *when there is a systemic s/Sx
   *ocular dryness is not responding to artificial tears
2. Admit
   *when severe systemic signs not responsive to outpatient tx