Sjogren syndrome

Question 1

Mean age of onset and M:F predominance of Sjogren syndrome:

Answer 1

30-50 y/o

strong female predominance 9:1 F:M

Question 2

What is the pathogenesis of Sjogren syndrome?

Answer 2

• lymphocytic infiltration of exocrine glands (lacrimal and salivary glands)

Question 3

What are the antibodies a/w Sjogren syndrome?

Answer 3

Anti Ro/SSA and Anti La/SSB

Question 4

Patients with Sjogren syndrome who have germline mutation in ____ have an increased risk of antigen-driven B-cell lymphoma

Answer 4

TNFAIP3

Question 5

What are the diagnostic criteria for sjogren syndrome?

Answer 5

must have clinical signs/symptoms suggestive of Sjogren syndrome, plus at least two of the three objective features:

1. positive SSA/Ro and/or SSB/La Or positive RF and ANA titier > 1:320
2. postive labial salivary gland biopsy
3. keratoconjunctivitis sicca (xerophthalma) w/ ocular staining score >3

Question 6

What are the signs and symptoms of Sjogren syndrome?

Answer 6

• Mucosal xerosis occurs later in disease course after 50% of glands distroyed (early may just have fatigue, arthralgias and myalgias)
• Xerostomia (dry mouth) - due to involvement of major (parotid and submandibular) and minor salivary glands—> sore/burning mouth/lips, dysphagia, transient swelling of parotids and submandibular glands
• dry eyes (xerophthalmia)- due to involvement of lacrimal gland
• vaginal xerosis–> dyspareunia, dryness, and burning (can lead to bacterial and candida overgrowth)

Question 7

What are complications that can occur as a result of dry mouth in Sjogren syndrome?

Answer 7

• perleche, thrush, dental caries and severe GERD

Question 8

What test can be done to check the function of the lacrimal glands in Sjogren syndrome?

Answer 8

Schirmer test (Whatman paper wick fold over lower eye)

• this is uncommonly done

Question 9

Skin findings in Sjogren syndrome?

Answer 9

xerosis (most common finding)

• Vasculitis (most important finding)
• annular erythema of Sjogren syndrome
• Raynauds
• Purpura with capillaritis
• Waldenstroms hypergammaglobulinemic purpura
• erythema nodosum
• Livedo reticularis

Question 10

If you have a patient with Sjogren syndrome and vasculitis, you must be thinking of the associations which include:

Answer 10

• systemic involvement (arthritis, renal involvement, peripheral neuropathy)
• Lymphoma

- increased mortality

Question 11

What is the most severe complication or association with Sjogren syndrome?

Answer 11

Lymphoma (19 fold increased risk of non-hodgkin lymphoma)

Question 12

What lymphoma is most commonly a/w Sjogren syndrome? Where does this classically occur?

Answer 12

- non-hodgkin (mostly extranodal MALT)

- classically in the tissues most affected by Sjogren syndrome (major salivary glands)

Question 13

What are neurologic findings that can be seen in Sjogren syndrome?

Answer 13

• painful sensory or sensorimotor polyneuropathy

- can also have memory loss, hearing loss

Question 14

What do you have to think of in pregnant patient who has Sjogren syndrome and positive Ro/La antibodies?

Answer 14

increased risk of ​neonatal lupus

Question 15

Histopath of Sjogren syndrome salivary gland?

Answer 15

• focal lymphocytic sialoadenitis with two or more aggregates of 50 or more lymphocytes per 4mm glandular tissue

Question 16

What is the most sensitive and specific test for Sjogren syndrome?

Answer 16

Anti-fodrin antibody (sounds like sjogren almost)

• other antibodies to check are Ro/La

Question 17

How can you treat the dry eyes of sjogren syndrome?

Answer 17

• preservative-free artificial tears during day
• lubricating ointments at night
• Punctae occlusion (plugs placed into lacrimal puncta to increase accumulation of tear film)
• cyclosporine eye drops BID for moderate to severe dry eyes

Question 18

Treatment for dry mouth in Sjogren syndrome?

Answer 18

• frequent water ingestion
• meticulous dental hygiene
• avoid alcohol, smoking, and low pH drinks (soda)
• if residual salivary gland function:
• chewing gum with xylitol and sorbitol

- pilocarpine and cevimeline

Question 19

Are immunosuppressive drugs used for Sjogren syndrome?

Answer 19

reserved for severe extraglandular systemic involvement

Question 20

What is the prognosis of Sjogren syndrome?

Answer 20

• on average mortality is equal to general population
• adverse prognostic factors a/w increased mortality are: lymphoproliferative disorders, vasculitis, cryoglobulinemia, hypocomplementemia

Question 21

What are the prognostic factors a/w increased mortality in Sjogren syndrome?

Answer 21

• adverse prognostic factors a/w increased mortality are: lymphoproliferative disorders, vasculitis, cryoglobulinemia, hypocomplementemia