Week 5 tuesday

Question 1

2 factors lead to autoimmune:

Answer 1

1. Inheritance of susceptible gene

2. Environmental triggers

Question 2

Infection can cause autoimmune by:

Answer 2

1. Upregulate expression of costimulators on APC
2. Molecular mimicry
3. Cause polyclonal activation

Question 3

Mechanism of SLE:

Answer 3

1. System lesion from antibodies on dsDNA and smith antigens form immune complex (type 3)
2. Antibodies against RBC, WBC and platelet. Opsonize and lead to cytopenia (type 2)

Neutrophil extracellulr trap in response to inflammation. release nuclear antigens

Question 4

Problems with anti-phospholipid antibodies in SLE

Answer 4

Hypercoagulation, leading to clot and ischemia

Question 5

Clinical skin features of SLE

Answer 5

1. Erythema in light exposed areas

2. immune complex on dermoepidermal junction

Question 6

Clinical kidney features in SLE

Answer 6

Nephritis due to immune complex deposition in glomeruli

Question 7

Clinical joint features in SLE

Answer 7

NON erosive and NON deforming small joint involvement

Question 8

Clinical hematologic system in SLE

Answer 8

Pericarditis, endocarditis, atherosclerosis

Question 9

SOAP BRAIN MD

Answer 9

SEROSITIS
ORAL ULCERS
ARTHRITIS
PHOTOSENSITIVITY
BLOOD CELLS
RENAL, RAYNAUDS
ANA
IMMUNOLOGIC
NEUROPSYCH
MALAR RASH
DISCOID RASH

Question 10

Mechanism of RA

Answer 10

Systemic inflammatory disorder against joints, producing NONSUPPORATIVE proliferative and inflammatory synovities that leads to DESTRUCTION of articular cartilage and ankylosis

Major genetic susceptibility

Question 11

Best test for RA

Answer 11

NOT rheumatoid factor

Test the ACCP = antibody cyclic citrullinated proteins

Question 12

Pathologic findings seen in RA joints

Answer 12

T and B cells form a PANNUS (mass of inflamed synovium) that grows over joint cartialge and leads to inflammation and destruction.

Question 13

RA nodule pathology

Answer 13

Area of central fibrinoid necrosis surrounded by a PALISADE OF MACROPHAGES and scattered chronic inflammatory cells

Question 14

Clinical presentation of Sjogren syndrome

Answer 14

DRY eyes and DRY mouth

Mostly in middle ages woman

Question 15

Pathology of sjogren syndrome and antibodies found

Answer 15

Autoimmune demediated destruction of LACRIMAL and SALIVARY glands

Often a secondary syndrome from other autoimmune. RA most common

Antibodies to ribonucleoproteins SS-A and SS-B are measured with LIP BIOPSY

Question 16

Neoplasm associated with Sjogren syndrome

Answer 16

LYMPHOMA (typically marginal zone)

Question 17

CREST syndrome

Answer 17

Can develop from limited scleroerma

Calcinosis
Raynauds phenomenom
Esophageal dysmotility
Sclerodactylyl
Telangiectasia

Question 18

Diffuse vs Limited scleroderma

Answer 18

Diffuse: WIDESPREAD skin involvement at onset, RAPID progression to visceral

Limited: Skin involvement CONFINED to fingers, forearms and face. Follow by LATE visceral involvement. Can lead to CREST

Question 19

Systemic sclerosis pathology

Answer 19

Unknown
Widespread damage to small blood vessels and progressive interstitial perivascular firbrosis of skin and organs. Patho findings are secondary to ischemic damage and fibrosis to organs

MAY have antibodies to SCL-70 (DNA TOPO 1 )
CREST may have anticentromere antibody

Question 20

Most common cause of death from Systemic sclerosis

Answer 20

Respiratory failure from interstitial fibrosis

Question 21

Clinical presentation of dermatomyositis vs myositis

Answer 21

dermatomyositis: muscle weakness and skin rash (discolor eye lids, red patches on knuckles, elbows and knees)

Myositis: Same, but WITHOUT skin involvement

Question 22

Lab findings and treatment for dermatomyositis and myositis

Answer 22

Elevated creatine kinase

Treat with immunosuppressives

Question 23

Antibody for Mixed connective tissue disease

Answer 23

Overlap of features with distinctive ANTI-U1-RNP antibody

Question 24

CATU / centromere antibodies found in:

Answer 24

CREST syndrome

Question 25

SS-A AND SS-B antibodies support:

Answer 25

Sjogren syndrome

Question 26

Jo 1 antibodies support

Answer 26

POLYMYOSITIS

Question 27

Scl 70 antibodies supports:

Answer 27

scleroderma

Question 28

Sm antibodies and dsDNA antibodies support

Answer 28

LUPUS

Question 29

primary vs secondary immunodefiency

Answer 29

primary: congenital or genetic
secondary: cancer, infection chemo, etc

Primary mainly found in INFANTS 6mo-2yr

Question 30

X-linked agammaglobulinemia (brutons)

Answer 30

failure of bcell precursors

X LINKED GENE FOR cytoplasmic tyrosine kinase

recurrent bacterial infections. Males > females

Question 31

variable immunodefiency

Answer 31

failures of bcells to differentiate. both sexes equally. later onset of symptoms

Question 32

Digeorge syndrome

Answer 32

failure of 3 and 4 pouch to develop = no thymus.
Loss of tcells
congenital heart and vessel problems
facial abnormalities

Question 33

Gene problem with digeorge syndrome

Answer 33

Sporadic deletion of chromosome 22q11

Question 34

CD40 ligand xlinked recessive mutation

Answer 34

Hyper IgM syndrome. Can not swtich to other Ig. Only IgM found

Question 35

Defect in gamma chain of cytokine receptor

Answer 35

SCID
autosomoal recessive
Defiency in enzyme adenosine deaminase
Treat with hemato cell transplant

Question 36

Loss of spleen and risk of infections?

Answer 36

Encapsulated bacteria, esp streptocossus pneumoniae

recieve vaccinations for s pneumoniae, h influenzae and n meningtidis

Question 37

Lead inhibits:

Answer 37

aminolevulinate dehydratase (ALA)

Ferrochelatase

Both reduce heme production as a result

Question 38

Lead poisoning urine results

Answer 38

elevated protoporphyrin and ALA

elevated erythrocyte protoporphyrin found early in both kids and adults