Sjogren's Syndrome

Question 1

What are the clinical hallmarks of Sjögren’s syndrome?

Answer 1

keratoconjunctivitis sicca (dry eyes), xerostomia (dry mouth), and parotid gland swelling.

Question 2

What are some of the extraglandular features of primary Sjögren’s syndrome?

Answer 2

include fatigue, Primary Raynaud’s phenomenon, polyarthralgia/arthritis, interstitial lung disease, neuropathy, and purpura.

Question 3

What is the characteristic histopathologic finding of Sjogrens?

Answer 3

autoimmune disorder causing chronic mononuclear cell infiltration of the lacrimal and salivary glands

Question 4

What is the patient population of Sjogrens?

Answer 4

1. Affects all races and all ages, but onset is greatest in MIDDLE age (40-60)- lupus is younger
2. 90% of patients are female
3. Increased incidence of autoimmune disease in family members, especially SLE

Question 5

Renal disease is infrequently of clinical significance in primary SS. How?

Answer 5

Can cause:

tubular interstitial nephritis,

type I renal tubular acidosis (RIA),

glomerulonephritis OR

nephrogenic diabetic insipidus.

Not that severe

Question 6

What are some potential GI symptoms associated with SS?

Answer 6

1/3 have varying degrees of esophageal dysfunction; some have chronic atrophic gastritis; liver involvement can be due to primary biliary cirrhosis, nonspecific hepatitis or autoimmune hepatitis.

Question 7

What malignancies are particularly common in SS?

Answer 7

NON-HODGKIN’S LYMPHOMA (NHL) has a prevalence of 4.3% in primary SS with a median time to development from diagnosis being 7.5 yrs.;

mucosa-associated lymphoid tissue (MALT) lymphoma occurs mostly in chronic autoimmune disease such as SS

Question 8

What Lab findings are consistent with SS?

Answer 8

1. Most patients with primary SS test positive for serum ANAs (85%).
2. Approximately 1/3 to ½ have anti-Ro/SS-A and anti-La/SS-B antibodies.
3. 5% to 10% of patients with primary SS have low blood levels of C3 and C4.
4. 5% to 10% of patients with primary SS have type II or III cryoglobulinemia or a monoclonal gammopathy.
5. 5% to 15% have leukopenia or thrombocytopenia.
6. 75 to 95% have positive rheumatoid factor

Question 9

What is this?

Answer 9

Schirmer’s test using filter paper is used to document decreased tear flow in SS

Question 10

What causes this in SS?

Answer 10

keratoconjunctivitis sicca- keratitis caused by decreased lacrimal secretions

•Symptoms: dryness of the eyes, foreign body sensation, burning, photosensitivity

Question 11

How is keratoconjunctivitis sicca best detected in SS?

Answer 11

Best detected by examination of the eyes after instilling rose Bengal dye to highlight epithelial lesions.

Question 12

Oral manifestations of SS?

Answer 12

• Severe mouth dryness (xerostomia)
• Multiple dental caries
• Fissuring and ulceration of the lips, tongue, and buccal membranes
• Difficulty chewing and swallowing
• Parotid and/or submandibular salivary gland enlargement occurs in 50% of patients and is most often unilateral and episodic

Question 13

What underlying pathologies place individuals at increased risk of Sjogren’s Syndrome?

Answer 13

• Rheumatoid arthritis
• SLE

these would be called secondary SS

Question 14

What is happening in this salivary gland in SS?

Answer 14

Mononuclear inflammatory infiltrates, interstitial fibrosis, and acinar atrophy of a minor salivary gland in a biopsy of lip is typical for long-standing Sjögren syndrome

Question 15

How does the salivary gland appear grossly in SS?

Answer 15

the salivary gland is enlarged, white and sometimes admixed with cysts.

Question 16

What are some other things on the DDx?

Answer 16

• antimuscarinics can cause xerostomia
• diabetes can cause parotid swelling

need all three

Question 17

Other symptoms of SS?

Answer 17

skin and vaginal dryness

CNS involvement can be involved (anti-aquaporin Abs present with these)

pulmonary involvment also possible

Question 18

Question 19

DDx histo for SS

Answer 19

In some cases the lymphocytic infiltrate is polytypic, in keeping with a reactive process (SS). Howebeer, this population can undergo small clonal proliferations and develop into full blown lymphomes (shown here)