Sjogren's Syndrome Flashcards Preview

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Flashcards in Sjogren's Syndrome Deck (19)
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1
Q

What are the clinical hallmarks of Sjögren’s syndrome?

A

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

2
Q

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

A

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

3
Q

What is the characteristic histopathologic finding of Sjogrens?

A

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

4
Q

What is the patient population of Sjogrens?

A
  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
5
Q

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

A

Can cause:

tubular interstitial nephritis,

type I renal tubular acidosis (RIA),

glomerulonephritis OR

nephrogenic diabetic insipidus.

Not that severe

6
Q

What are some potential GI symptoms associated with SS?

A

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.

7
Q

What malignancies are particularly common in SS?

A

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

8
Q

What Lab findings are consistent with SS?

A
  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
9
Q

What is this?

A

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

10
Q

What causes this in SS?

A

keratoconjunctivitis sicca- keratitis caused by decreased lacrimal secretions

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

11
Q

How is keratoconjunctivitis sicca best detected in SS?

A

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

12
Q

Oral manifestations of SS?

A
  • 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
13
Q

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

A
  • Rheumatoid arthritis
  • SLE

these would be called secondary SS

14
Q

What is happening in this salivary gland in SS?

A

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

15
Q

How does the salivary gland appear grossly in SS?

A

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

16
Q

What are some other things on the DDx?

A
  • antimuscarinics can cause xerostomia
  • diabetes can cause parotid swelling

need all three

17
Q

Other symptoms of SS?

A

skin and vaginal dryness

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

pulmonary involvment also possible

18
Q
A
19
Q

DDx histo for SS

A

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)