Immuno path III - Autoimmune disorders continued. Flashcards Preview

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Flashcards in Immuno path III - Autoimmune disorders continued. Deck (19)
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1
Q

This autoimmune disorder is characterized by the destruction of exocrine glands with the ocassional affect on GI, GU, and Respiratory tracts.

A

Sjorgens syndrome

Occurring 90% in females.

2
Q

What is Sicca syndrome and how common is it?

A

This is Sjorgens syndrome causing damage only to the lacrimal and salivary glands.

Occurs in 40% of Sjorgens patients.

3
Q

What is a possible mechanism for the cause of Sjorgens syndrome?

A

Infection by SuperAg may trigger CD4 cells to react against glandular epithelial cells.

4
Q

ANAs to SS-A (RO) and SS-B (La) are indicitive of what disease?

A

90% of the time are related to Sjorgens syndrome.

5
Q

Sjorgens patients are at 40% increased risk of what cancer?

A

B-cell lymphoma possibly due to development of neoplastic, monoclonal cell line via polyclonal B cell activation.

6
Q

Which ANA is related to causing systemic Sjorgens syndrome ?

A

Ro AKA SS-A Ab.

7
Q

What causes the pathogenesis of systemic sclerosis?

A

B-cells become activated for unknown reason which then activate T cells to produce cytokines that promote fibrogenesis and excessive collagen in the skin and viscera.

8
Q

B cells in systemic sclerosis produce which ANAs?

A

anti-DNA topoisomerase I (anti-Scl-70) ab.

9
Q

The limited variant of SS is positive to which Ab in 90% of cases?

A

Positive to anticentromere Ab!

Leads to limited involvement and late, slow progressing visceral involvement.

10
Q

What does CREST syndrome relate to and what is it a variant of?

A
C - Calcinosis
R - Reynauds
E - Esophageal dysmotility
S - Sclerodactly
T - Telangiectasia
A limited variant of Systemic Sclerosis.
11
Q

Esophageal atrophy and collagenization is a clinical sign of what defect?

A

CREST syndrome, the limited form of Systemic Sclerosis.

12
Q

Cor pulmonale of the heart is a clinical manifestation of what systemic disorder?

A

Systemic Sclerosis occuring in >50% of patients.

13
Q

What is the 10 year survival rate for Systemic Sclerosis?

A

35-70%
COD:
Renal failure, cardiac failure, pulmonary insuffciency, GI malabsorption.

14
Q

What is the male to female ratio of infection by RA?

A

2-3F:1M within ages 40-70.

15
Q

The pathogenesis of rheumathoid arthritis is as follows:

A

Activation of CD4 T cells leading to production of damaging cytokines and proliferation of synovial cells and fibroblasts.
TNF leads to leukocyte recruitment.

16
Q

What is Rheumatoid factor?

A

Production of IgM auto-ab that binds to IgG causing type III synovitis.

17
Q

What is Pannus formation and what is it related to?

A

Related to rheumatoid arthritis.

Accumulation of grannular tissue, synovial and inflammatory cells as well as connective tissue in the affected joint.

18
Q

What is seen more frequently in juvenile arthritis?

A

Systemic complications including pericarditis, myocarditis, pulmonary fibrosis and GN.

NO RF SEEN IN THES PTS.

Clinical remission can occur in up to 50% of cases.

19
Q

What is the ANA ab associated with mixed connective tissue disease?

A

Ribonucleoprotein (Anti-U1 RNP)