Gen Path Exam 3 - Autoimmune Disease - Sjogrens

Question 1

Chronic disease characterized by dry eyes and dry mouth resulting from immunologically mediated
destruction of the lacrimal and salivary glands

Answer 1

Sjogren’s

Question 2

Xerophthalmia

Answer 2

Dry eyes

Question 3

Sjogren’s effects what people?

Answer 3

35-45 yr old women

Question 4

Sjogren’s occurs as an __________ disorder (primary form), also known as _______ syndrome, or more often in association with another autoimmune disease (secondary form)

Answer 4

isolated; sicca

Question 5

Isolated disorder; primary form of Sjogren’s

Answer 5

Sicca syndrome

Question 6

Most common disorder associated with Sjogren’s

Answer 6

RA

Question 7

Other 6 disorders associated with Sjogren’s

Answer 7

Systemic lupus
Polymyositis
Scleroderma
Vasculitis
Mixed CT disease
Thyroiditis

Question 8

Seen in ~1/3 of pts with RA or Systemic lupus

Answer 8

Sjogren’s (secondary)

Question 9

What is the possible initiating factor of Sjogren’s?

Answer 9

Viral infection of salivary glands

Question 10

What does a viral infection of the salivary glands cause?

Answer 10

Local cell death
Release of tissue self-antigens

Question 11

Name 2 viral infections of the salivary glands that may trigger Sjogren’s

Answer 11

EBV
Coxsackie

Question 12

In genetically susceptible individuals, which cells specific for self-antigens may escape tolerance and are able to react? What is the result?

Answer 12

CD4 T cells
B cells

Result = inflammation, tissue damage, fibrosis

Question 13

What are the major targets of Sjogren’s?

Answer 13

Lacrimal + salivary glands

Question 14

Primary and secondary forms of Sjogren’s can have ________________ manifestations that can effect all major organ systems and increased risk for the development of _____________

Answer 14

extraglandular; lymphoma

Question 15

Most pts with Sjogren’s present with sicca symptoms. Name them.

Answer 15

Dry eyes
Dry mouth

Question 16

Sjogren’s classically manifests with painless, intermittent enlargement of which gland? (usually unilaterally in beginning)

Answer 16

Parotid

Question 17

The clinical manifestation of Sjogren’s usually progresses to what?

Answer 17

Chronic salivary gland enlargement (usually bilateral)

Question 18

What are the 4 ways pts usually describe dry mouth?

Answer 18

Can’t eat dry food (like crackers) bc they stick to roof of mouth
Tongue sticks to roof of mouth
Can’t speak for long periods of time - develop hoarseness
Can’t wear dentures

Question 19

What are the 3 ways pts usually describe dry eyes?

Answer 19

Red
Itchy
Painful

Question 20

What is the most typical complaint of dry eyes?

Answer 20

Gritty/sandy sensation in eyes

Question 21

Which clinical manifestation of Sjogren’s?

Peripheral and cranial neuropathies
MS “look-alike” CNS disease

Answer 21

Neurologic

Question 22

Which clinical manifestation of Sjogren’s?

Palpable/nonpalpable purpura, papules, urticarial lesions, and annular lesions
Xerosis

Answer 22

Skin

Question 23

Xerosis

Answer 23

Dry skin

Question 24

Which clinical manifestation of Sjogren’s?

Interstitial nephritis
Renal tubular acidosis
Hyposthenuria w/ glomerulonephritis

Answer 24

Renal

Question 25

Hyposthenuria

Answer 25

Urine w/ an osmolality < plasma

Question 26

Which clinical manifestation of Sjogren's? Tracheobronchitis Bronchiectasis Interstitial pneumonitis Fibrosis

Answer 26

Lung

Question 27

What 3 clinical findings are typically seen in Sjogren's?

Answer 27

Non-erosive arthritis Polyarthralgia Raynaud's

Question 28

Used to measure the production of tears and helps establish the presence of xerophthalmia (dry eyes)

Answer 28

Schirmer test

Question 29

Good measure of the degree of decreased salivary flow and helps establish the presence of xerostomia (dry mouth)

Answer 29

Sialometry

Question 30

What is considered to be the best sole diagnostic criterion for the salivary component of Sjogren's?

Answer 30

Minor salivary gland biopsy

Question 31

What is the earliest histologic finding in both the major and minor salivary glands?

Answer 31

Periductal + perivascular lymphocytic infiltration

Question 32

Eventually the lymphocytic infiltrate becomes extensive and in the larger salivary glands, lymphoid _________ with ___________ _________ may be seen

Answer 32

follicles; germinal centers

Question 33

About 75% of patients are ___________ for _____________ ___________ regardless of whether coexisting RA is present or not

Answer 33

positive; Rheumatoid factor (RF)

Question 34

What is detected in a fine, speckled pattern in up to 83% of pts with primary Sjogren's?

Answer 34

ANAs

Question 35

What are the 2 ribonucleoprotein antigens that autoantibodies are directed against in Sjogren's?

Answer 35

SS-A (Ro) SS-B (La)

Question 36

9 complications of Sjogren's

Answer 36

1. Salivary gland infection 2. Salivary gland obstruction 3. Oral mucosal atrophy 4. Increased caries/perio 5. Dysphagia + dysgeusia 6. Oral candidiasis 7. Keratoconjunctivitis sicca 8. Perforation of septum due to dry nose 9. Non-hodgkin lymphomas

Question 37

Drying of corneal epithelium which may become inflamed, eroded, and ulcerated -> leads to corneal vascularization, opacification, perforation

Answer 37

Keratoconjunctivitis sicca

Question 38

What are the following 4 complications in Sjogren's secondary to? Oral mucosal atrophy Increased caries/perio Dysphagia + dysgeusia Oral candidiasis

Answer 38

Xerostomia

Question 39

Patients with Sjögren’s have up to a 40-fold increased risk of developing what type of malignancies?

Answer 39

Lymphoid

Question 40

What are the most common non-hodgkin lymphomas seen in pts with Sjogren's?

Answer 40

MALT lymphomas (mucosa-associated B cell lymphomas)

Question 41

Where are non-hodgkin lymphomas seen in pts with Sjogren's?

Answer 41

Salivary glands Lymph nodes