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General Pathology > Exam #3: Immunopathology III > Flashcards

Exam #3: Immunopathology III Flashcards

1
Q

What is Sjogren’s Syndrome?

A

Autoimmune destruction of the:

  • Exocrine glands
  • Primary lacrimal glands
  • Salivary glands
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2
Q

Is there a sex predominance to Sjogren’s Sydrome?

A

Yes, females

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3
Q

What is Sicca Syndrome?

A

Isolated form of Sjogren’s that causes a decrease in tears & saliva

*****Note that Sjogren’s Syndrome is a systemic autoimmune disease

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4
Q

Describe the progression of Sjogren’s Syndrome?

A
  • CD4+ T-cell proliferation against glandular epithelial self-antigens
  • Systemic B-cell hyperactivity to antinuclear antigens (ANA)

*****Note that the syndrome may be induced by vial infections e.g. EBV & Hepatitis C

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5
Q

What are hallmark ANAs in Sjogren’s Syndrome?

A

Anti-nuclear antigens to ribonucleoproteins called:

  • SS-A (Ro)
  • SS-B (La)

*****These are common & seen in 90% of cases; however, not that ~70% of patients are also positive for Rhematoid Factor (RF)

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6
Q

Describe the pathogenesis of Sjogren’s Syndrome?

A
  • Activated CD4+ T-cells & B-cells infiltrate ducts & vessels in target glands
  • Ductal hyperplasia leads to obstruction of the glands
  • Acinar atrophy, fibrosis, and replacement of parenchyma w/ fat occurs
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7
Q

Describe the symptomatic progression of Sjogren’s Syndrome.

A

Dry mucous membranes leads to:

  • Xerostomia (dry mouth)
  • Keratoconjunctivitis sicca (dry eyes)
  • Nasal septal erosion/ perforation
  • Dysphagia
  • Dypareunia
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8
Q

What are Sjogren’s patients at risk for?

A

B-cell lymphoma

*****Note that lymph nodes in patients with Sjogren’s Syndrome are often massively hyperplastic

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9
Q

What are the systemic symotoms associated with Sjogren’s Syndrome?

A
  • Systemic vasculitis & extraglandular involvement of the kidneys, lungs, skin, CNS, muscle

Leads to:

  • Interstitial nephritis
  • Pulmonary fibrosis
  • Peripheral neuropathy
  • Synovitis
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10
Q

What antibody is associated with more systemic manifestations of Sjogren’s Syndrome?

A

SS-A (Ro)

**Note that SS-A (Ro) is generally associated with a worse clinical course

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11
Q

What are the symptoms of Systemic Sclerosis?

A

Involvement of the skin=

  • Rigidity
  • Loss of specialized skin cells & function

Involvement of the GIT

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12
Q

Is there a sex predominance associated with Systemic Sclerosis?

A

Female

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13
Q

What is Systemic Sclerosis?

A

Autoimmune disorder characterized by excessive fibrosis throughout the body; furthermore there is:

1) Chronic inflammation
2) Destruction of small vessels
3) Progressive tissue fibrosis of not only the skin but multiple organs as well

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14
Q

Describe the pathogenesis of Systemic Sclerosis.

A

The cause of Systemic Sclerosis is unknown.

  • It is proposed that there is inappropriate activation of CD4+ T-cells that release cytokines, which activate inflammatory cells & fibroblasts
  • There is also blood vessel injury, which causes ischemia leading to fibrosis; cause is unknown
  • Also, there is inappropriate humoral activation
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15
Q

What is the predominant growth factor associated with Systemic Sclerosis?

A

TGF-B

**TGF-B leads to fibroblast activation & fibrosis

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16
Q

What is CREST Syndrome?

A

There are two subtypes of Systemic Sclerosis, limited & diffuse

  • Limited is characterized by:
    1) Skin involvement of the face, forearms, & figners
    2) Late visceral involvement

**This limited subtype of Systemic Sclerosis is called CREST Syndrome

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17
Q

What does CREST stand for?

A 
Calcinosis
Raynaud's phenomenon
Esophageal dysfunction
Sclerodactyly 
Telangiectasias
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18
Q

What is Clacinosis?

A

Ca++ deposits in the skin

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19
Q

What is Raynaud’s phenomenon?

A

Spasm of blood vessels in response to cold or stress (white fingertips)

20
Q

What is esophageal dysfunction?

A

GERD & decreased esophageal motility

21
Q

What is Sclerodactyly?

A

Thickening & tightening of the skin on the fingers & hands

22
Q

What is Telangiectasas?

A

Dilation of capillaries causing red marks on the surface of skin

23
Q

What is the severe complication of Reynaud’s Phenomenon?

A
  • Marked pallor of the fingers

- Can lead to necrosis

24
Q

What is the diffuse variant of Systemic Sclerosis? Symptoms?

A

This is the second subtype of Systemic Sclerosis that is characterized by:

  • Widespread skin involvement at onset
  • Early visceral involvement
  • Rapid progression

*****A worse clinical course compared to limited/ CREST

25
Q

What antibody is associated with the diffuse variant of Systemic Sclerosis? What about CREST Syndrome

A

Antibody to DNA topoisomerase I (anti-Scl-70) is associated with diffuse variant Systemic Sclerosis

*****vs. Anti-cenntromere antibody seen in CREST Syndrome

26
Q

What are the early skin changes seen with diffuse Systemic Sclerosis?

A
  • Edema

- Lymphocyte (CD4+) infiltrates

27
Q

What are the late skin changes seen with diffuse Systemic Sclerosis?

A
  • Epidermal thinning
  • Dermal/ appendage fibrosis
  • Subcutaneous calcifications

Ultimately these lead to contractures i.e. claw fingers & “mask facies”

28
Q

What are the vascular complications seen with Systemic Sclerosis?

A

Chronic vascular endothelial damage & fibrosis causes ischemia that leads to autoamputation of digits

29
Q

What are the esophageal changes that are seen in Systemic Sclerosis?

A

Collagenization & fibrosis of the muscularis mucosa that leads to:

  • Esophageal dysmotility
  • LES dysfunction
  • GERD
30
Q

What are the small bowel cahnges seen in Systemic Sclerosis?

A
  • Mucosal thinning
  • Loss of villi & microvilli
  • Submucosal fibrosis

**All of these pathologic changes result in MALABSORPTION

31
Q

What are the renal changes that are seen in Systemic Sclerosis? What does this cause?

A
  • Thickening of interlobular arteries by concentric proliferation of intimal cells
  • Deposition of collagenous or mucinous material & hyaline

*****These changes result in HTN in 30% of cases

32
Q

What is the genetic predisposition for RA?

A
  • HLA-DRB1

- PTPN22 (Remember, gene for tyrosine phosphatase that participates in the activation & control of T-cells )

33
Q

Describe the pathogenesis of RA.

A

1) Unknown antigenic trigger w/ underlying genetic susceptibility
2) Initiation of synovitis
3) ONGOING AUTOIMMUNE REACTION mediated by CD4+ T-cells
- Produce cytokines
- Activate macrophages
- Activate B-cells
4) Production of Rhematoid Factor

34
Q

What is RA factor?

A

IgM auto-antibody to Fc portion of IgG (in same individual)

35
Q

What are citrullinated peptides?

A

Certain enzymatic reactions in the body will convert arginine–>citrulline (seen in smokers)
- antibodies that develop to these peptides & contribute to RA

36
Q

How does RA first present?

A

Typically, there is a slow & insidious progression starting with malaise, fatigue, and generalized MSK pain
- Several weeks- months later joints become involved

**Joint involvement is typically symmetric with the small joint affected first, then progressing to more proximal joints

37
Q

What are the hallmark deformaties associated with RA?

A

Ulnar deviation
Swan-neck

**Note that joints are swollen, warm, painful, & stiff especially following inactivity

38
Q

How does RA appear on x-ray?

A
  • Loss of joint space
  • Ulnar drift of the fingers

**Patients are also diffusely osteopenic (progressing toward osteoporosis)

39
Q

What is pannus formation?

A

Inflammatory response that includes:

  • Granulation tissue
  • Synovial & inflammatory cells
  • Fibrous CT
40
Q

What are the systemic manifestations of RA?

A

Rheumatoid nodules form at the:

  • Skin pressure points
  • Viscera

Small vessel vasculitis especially in cases of severe disease w/ high RF titers

41
Q

Describe the clinical course of RA.

A

Course is variable, ranging from mild discomfort to progressive disability

42
Q

What complications are associated with RA?

A

1) Amlyoidosis
2) Vasculitis
3) Drug complications
- Bleeding
- Infection

43
Q

What is Juvenile Idiopatic Arthritis?

A

This is a diagnosis of all forms of arthritis, developing in the patient prior to 16 years-old & persisting for 6 weeks

  • Frequently involves large joints
  • ANA positive, BUT RF NEGATIVE
44
Q

What is Still’s Disease?

A

This is a variant of Juvenile Idiopathic Arthritis that is associated with:

1) Fever
2) Hepatosplenomegaly
3) Rash
4) Increased WBC count

45
Q

What is mixed connective tissue disease?

A

Clinical syndrome with overlapping features of SLE & Systemic Sclerosis

**Note that it is DISTINCTIVE b/c of minimal renal disease & good response to steroid therapy

46
Q

What antibody is associated with MCTD?

A

anti-U1RNP to ribonucleoprotein

47
Q

Why is important to remember about MCTD clinically?

A

Good response to steroid therapy

General Pathology (33 decks)

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