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Path Exam III > Immunopathology III > Flashcards

Immunopathology III Flashcards

1
Q

What is Sjorgrens syndrome marked by?

A

Destruction of exocrine glands in the body

i.e.: Lacrimal and salivary glands.

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2
Q

What sex does sjorgens disease most commonly affect?

A

90% of cases are wtihin women.

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3
Q

What is sicca syndrome?

A

A type of sjorgens syndrome in which there is a decrease in tears and saliva.

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4
Q

What is the pathogenesis for sjorgens syndrome?

A

CD4 T cells kill self Ag in glands.

Systemic B cell hyperactivity occurs with 75% of cases positive for RF

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5
Q

Which specific ANAs does sjorgens syndrome have?

A

Specific ANAs to ribonucleoproteins
SS-A (Ro)
and
SS-B (La) - Very common.

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6
Q

Systemic Sjorgens syndrome is associated with what ANA?

A

SS-A ab

Ro

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7
Q

What is the male to female ratio for systemic sclerosis?

A

3:1 F:M

typically occurring around 50-60 years.

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8
Q

What cytokine causes mass activation of fibroblasts in Systemic Sclerosis?

A

TGF-B

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9
Q

What is the limited varient of systemic sclerosis?

A

CREST syndrome with late visceral involvement in a rather benign course and limited skin involvmenet.

Look for anti-centromere ab.

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10
Q

What is the major characteristic test for CREST syndrome identification?

A

Anti centromere antibody

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11
Q

What does CREST stand for?

A

Calcinosis - Calcium deposits in skin
Raynauds phenomenon
Esophageal dysfunction
Sclerodactyly (Thickening and tightening of skin on hands)
Telangiectasis (Dilation of capillaries on skin)

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12
Q

What is the diffuse variant of CREST?

A

Widespread skin involvement at onset with rapid progression due to Ab to DNA topoisomerase I (Anti-ScL-70)

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13
Q

What is the reactive antibodies produced to the diffuse variant of CREST?

A

Typically Ab to DNA topoisomerase I (anti-Scl-70) occuring in 70% of cases.

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14
Q

Which disease ca cause vascular obliteration and fibrosis within the esophagus?

A

CREST

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15
Q

Cor pulmonale can be caused by what?

A

Systemic Sclerosis within the lungs occuring >50% of the time.

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16
Q

What is the ten year survival rate for Systemic sclerosis?

A

Varies from 35-70% depending on which organs are involved.

17
Q

What are the typical organ problems that result in death caused by systemic sclerosis?

A

Renal
Cardiac
Pulmonary
GI dysfunction or failure.

18
Q

What genetic issue leads to RA?

A

HLA-DRB1 and PTPN22 gene for tryrosine phosphatase begins the synovitis that is then continued by CD4 T cells making cytokines.

19
Q

What exactly is rheumatoid factor?

A

An auto antibody IgM that has an Fc portion autologous to IgG in 80% of cases.

20
Q

Citrullinated peptides contribute to what disease state?

A

Rheumatoid arthritis.

21
Q

What is pannus formation and what is it linked to?

A

Granulation tissue, synovial and inflammatory cells with fibrous conective tissue.

Linked to RA.

22
Q

In 25% of RA cases, rheumatoid nodules will appear at whcih locations?

A

Appear at skin pressure points such as the elbow or occiput.

Also appear in the viscera of the lungs, spleen, and heart.

23
Q

In cases of severe rheumatoid arthritis what can occur in the blood vessels??

A

Small vessel vasculitis with high tighters of RF

24
Q

How does RF damage the body?

A

RF binds with IgG and forms antibody antigen complexes causing type III damage to small vessels in severe cases of RA.

25
Q

Rheumatoid nodules histologically show what pattern?

A

Show necrosis surrounded by fibrosis

26
Q

What is unique about juvenile idiopathic arthritis occuring in children

A

Ends up systemic causing pericarditis, myocarditis, and pulmonary fibrosis.
Also can undergo remission.

27
Q

What is Still disease?

A

A Juvenile idiopathic arthritis variant presenting with febrile ilness, hepatosplenomegaly, rash, and an increased WBC count.

28
Q

Does Juvenile idiopathic arthritis show RF?

A

No rheumatoid factor but is ANA+.

29
Q

What are the characteristics of Mixed connective tissue disease?

A

Overlapping features of SLE and Systemic sclerosis

30
Q

What specific Ab is linked to mixed conective tissue disease?

A

anti-U1RNP to ribonucleoprotein.

Path Exam III (6 decks)

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