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D2 Fall > Gen Path Exam 3 - Autoimmune Disease - Sjogrens > Flashcards

Gen Path Exam 3 - Autoimmune Disease - Sjogrens Flashcards

1
Q

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

A

Sjogren’s

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2
Q

Xerophthalmia

A

Dry eyes

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3
Q

Sjogren’s effects what people?

A

35-45 yr old women

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4
Q

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

A

isolated; sicca

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5
Q

Isolated disorder; primary form of Sjogren’s

A

Sicca syndrome

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6
Q

Most common disorder associated with Sjogren’s

A

RA

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7
Q

Other 6 disorders associated with Sjogren’s

A

Systemic lupus
Polymyositis
Scleroderma
Vasculitis
Mixed CT disease
Thyroiditis

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8
Q

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

A

Sjogren’s (secondary)

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9
Q

What is the possible initiating factor of Sjogren’s?

A

Viral infection of salivary glands

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10
Q

What does a viral infection of the salivary glands cause?

A

Local cell death
Release of tissue self-antigens

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11
Q

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

A

EBV
Coxsackie

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12
Q

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

A

CD4 T cells
B cells

Result = inflammation, tissue damage, fibrosis

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13
Q

What are the major targets of Sjogren’s?

A

Lacrimal + salivary glands

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14
Q

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

A

extraglandular; lymphoma

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15
Q

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

A

Dry eyes
Dry mouth

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16
Q

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

A

Parotid

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17
Q

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

A

Chronic salivary gland enlargement (usually bilateral)

18
Q

What are the 4 ways pts usually describe dry mouth?

A

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

19
Q

What are the 3 ways pts usually describe dry eyes?

A

Red
Itchy
Painful

20
Q

What is the most typical complaint of dry eyes?

A

Gritty/sandy sensation in eyes

21
Q

Which clinical manifestation of Sjogren’s?

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

A

Neurologic

22
Q

Which clinical manifestation of Sjogren’s?

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

A

Skin

23
Q

Xerosis

A

Dry skin

24
Q

Which clinical manifestation of Sjogren’s?

Interstitial nephritis
Renal tubular acidosis
Hyposthenuria w/ glomerulonephritis

A

Renal

25
Q

Hyposthenuria

A

Urine w/ an osmolality < plasma

26
Q

Which clinical manifestation of Sjogren’s?

Tracheobronchitis
Bronchiectasis
Interstitial pneumonitis
Fibrosis

A

Lung

27
Q

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

A

Non-erosive arthritis
Polyarthralgia
Raynaud’s

28
Q

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

A

Schirmer test

29
Q

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

A

Sialometry

30
Q

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

A

Minor salivary gland biopsy

31
Q

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

A

Periductal + perivascular lymphocytic infiltration

32
Q

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

A

follicles; germinal centers

33
Q

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

A

positive; Rheumatoid factor (RF)

34
Q

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

A

ANAs

35
Q

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

A

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

36
Q

9 complications of Sjogren’s

A
  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
37
Q

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

A

Keratoconjunctivitis sicca

38
Q

What are the following 4 complications in Sjogren’s secondary to?

Oral mucosal atrophy
Increased caries/perio
Dysphagia + dysgeusia
Oral candidiasis

A

Xerostomia

39
Q

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

A

Lymphoid

40
Q

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

A

MALT lymphomas

(mucosa-associated B cell lymphomas)

41
Q

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

A

Salivary glands
Lymph nodes

D2 Fall (37 decks)

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