Pathology Of Autoimmune Diseases

Front 1

What is the main pathogenesis pattern of Sjögren Syndrome?

Main population affected?

Back 1

Lymphocyte infiltration and fibrosis of salivary and lacrimal glands mainly by CD4+ cells, some infiltration by B cells and plasma cells.

Females 35-45 (90%)

Front 2

Antibody involvement with Sjögren?

Eliciting factors?

Back 2

No direct evidence for antibody involvement or which antigen(s) elicit Sjögren Syndrome.

Potential role for alpha-fodrin autoantigen or retrovirus infection for initiating events.

Front 3

Chief symptoms include _____ of eyes and mouth. Eyes tested with _______'s Test.

Back 3

dryness (keratoconjunctivitis and xerostomia); Schirmer's Test

Front 4

Extra-glandular symptoms include (3):

Back 4

1) pulmonary fibrosis (30-40% of patients, can progress to complete respiratory failure)

2) synovitis

3) peripheral neuropathy

Front 5

Is Sjögren Syndrome associated with malignancy?

Back 5

Yes: Follicular B cell hypertrophy leads to lymphadenopathy and 40x risk for malignant lymphoma

Better recognize niggas.

Front 6

Most specific antibodies for Sjögren Syndrome?

High ____ associated with ________.

Back 6

SSA (Ro) and SSB (La) - elevated in 90% of Sjögren patients

High SSA (Ro) levels more likely to exhibit extraglandular disease. enG

Front 7

RF levels in Sjögren Syndrome:

Back 7

Elevated in 75% Sjögren patients (in absence of RA)

Front 8

ANA levels and immunofluorescence pattern?

Back 8

Elevated in 50-80% of Sjögren patients overall.

Speckled Pattern specific for Sjögren Syndrome.

Front 9

Part of glands affected first by lymphocyte infiltration?

Back 9

Acini obliterated before more sturdy ducts

Front 10

HLA alleles associated with Sjögren Syndrome (3):

Back 10

B8, DR3 and DRW52

Front 11

Main pathogenesis pattern for scleroderma (systemic sclerosis)?

Back 11

Excessive fibrosis throughout the body - skin especially, then GI, kidneys, heart, muscles, lungs.

Front 12

Cells and factors involved with epithelial scleroderma pathogenesis:

Back 12

Antigen-activated CD4+ T cells induce inflammatory cells recruitment and release of inflammatory cytokines (IL-4, IL-13, PDGF, TGF-B) that stimulate myofibroblasts and lead to excessive collagen deposition.

Front 13

Cells and factors associated with endothelial scleroderma pathogenesis:

Back 13

Endothelin: endothelial injury associated with irreversible intimal fibrosis and platelet activation that can lead to ischemia

Front 14

Skin-related symptoms of scleroderma:

Back 14

- Sclerotic atrophy of skin beginning distally

- Thickening/occlusion of small vessels can lead to ischemia

- Loss of rete pegs/dermal appendages can lead to ulcers

Front 15

Chief histologic dermal findings with scleroderma (3):

Back 15

- dense collagen deposition in dermis

- eccrine sweat gland degeneration

- hair follicles missing (only arrector pili muscles left behind)

Front 16

Most common extra-dermal finding of scleroderma is?

Back 16

GI/Esophagus (90%)

- Collagen deposition at the esophagus that disrupts the muscularis --> GERD and Barrett's Esophagus

- Loss of villi in small bowel --> malabsorption

Front 17

Fibrosis in small vessels of the ______ can lead to hypertension and _____ failure

Back 17

Kidney involvement for 2/3 of patients:

- Hypertension (30%)

- Renal failure (50% deaths)

Front 18

The most common causes of morbidity/mortality in scleroderma are:

Back 18

Pulmonary HTN and pulmonary fibrosis

Front 19

Heart symptoms associated with scleroderma (2):

Back 19

More fibrosis - myocarditis and myocardial fibrosis

Front 20

Diffuse scleroderma characteristics:

Back 20

- Affects all skin

- Nucleolar ANA pattern

- Anti-DNA topoisomerase 1 (Scl-70) in 28-70% of patients

Front 21

Limited scleroderma characteristics:

Back 21

- Only affects skin at distal limbs (acral skin)

- Does not affect skin at trunk or proximal limbs

- Ant-centromere ANA

- Extracutaneous CREST symptoms

Front 22

What does CREST stand for?

Back 22

- Calcinosis

- Raynaud's

- Esophageal dysmotility

- Sclerodactyly

- Telengectasia

O'Hara says Calcinosis not an associated finding anymore, internet says otherwise. God FTW

Front 23

Basic pathogenesis pattern of Rheumatoid Arthritis (RA):

Back 23

Chronic inflammatory autoimmune disorder that attacks joints, can lead to destroyed articular cartilage and ankylosis

Front 24

Chief factor elevated in 80% of those with RA? Role in pathogenesis?

Back 24

Rheumatoid factor (RF) = anti-IgG IgM

Circulating IgG-IgM complexes that deposit in vessels though to to contribute to extra-articular symptoms.

Not 100% responsible for symptoms - found in healthy people and not found in some with RA

Front 25

T cell type related to RA? Cells and enzymes they acticvate?

Back 25

CD4+ T cells

Activate endothelial cells to express adhesion molecules

Activates collagenase, elastase, stromelysin, PGE2 and other enzymes that help break down joint tissue

Front 26

Result of immune complex deposition and CD4+ T cell activation =

Back 26

Pannus: tumor-like mass of granulation tissue that destroys articular cartilage and bone. Can lead to ankylosis

Front 27

Primary joint affected by RA? Spared joints?

Back 27

Primarily affects small joints - MCP, PIP, wrist, ankles, elbows

Large joints usually spared

Front 28

______ deviation of the wrist and ______ deviation of the phalangeal joints is a sign of progressed RA

Back 28

Radial deviation of the wrist, ulnar deviation of the phalangeal joints

Front 29

Effects at synovium with RA due to CD4+, macrophage and plasma cell infiltration (5):

Back 29

- synovial hyperplasia

- edema

- synovial membrane thickening

- increase vascularity

- fibrin deposition

Front 30

Fibrinoid necrosis of collagen surrounded by inflammatory cells =

Back 30

Rheumatoid nodules

Can be found at skin (usually extensor surfaces) or lung, heart, spleen

Front 31

Name 3 organ specific autoimmune diseases:

Back 31

1) Hasimoto's thyroiditis

2) Atrophic gastritis

3) Hemolytic anemia

All directed against one tissue

Front 32

4 examples of systemic auto-immune diseases:

Back 32

1) SLE

2) RA

3) Scleroderma

4) Sjögren Syndrome

Front 33

Indirect immunofluorescence test technique

Back 33

Introduce anti-IgG antibodies with fluorescent component after permeabilizing cell membrane and nuclear membrane

Front 34

Auto-antibodies and ANA pattern for SLE

Back 34

- anti-dsDNA and anti-Sm antigen

- homogenous ANA pattern

Front 35

Auto-antibodies and ANA pattern for Sjögren Syndrome

Back 35

- SSA (Ro) an SSB (La) anti-RNPs

- Speckled ANA pattern

Front 36

Auto-antibodies and ANA pattern for scleroderma

Back 36

- anti-DNA topoisomerase 1 (Scl-70)

- Nucleolar ANA pattern (most sensitive of the ANA patterns)