Lupus and Sjogrens

Question 1

Systemic Lupus Erythematosus definition?

Answer 1

Multi-system inflammatory disorder

Autoantibodies to numerous self antigens

Question 2

Discoid lupus

Answer 2

only involves the skin though these Discoid lesions CAN be seen in SLE

Question 3

Drug induced Lupus

Answer 3

Less severe than SLE

Self-limiting once the offending drug is removed.

Question 4

Neonatal lupus

Answer 4

Newborns of mothers with SLE

-skin rash, heart block (permanant), thrombocytopenia

Question 5

What are the three problems of neonatal lupus

Answer 5

skin rash, heart block(which is permanent), and thrombocytopenia

Question 6

Female to male ration in lupus

Answer 6

13:1

Question 7

Peak incidence of lupus?

Answer 7

Childbearing years

Question 8

Racial discrimination

Answer 8

Blacks 3-4 times more likely

Question 9

Lupus is called the disease of a thousand faces. WHy

Answer 9

Can present in tons of different ways

Question 10

Common general symptoms of lupus

Answer 10

Malaise, fever, fatigue

Question 11

Cutaneous manifestations of lupus

Answer 11

Malar rash, alopecia

Question 12

Malar rash, along with other symptoms, get worse upon exposure to what?

Answer 12

SUN

Question 13

Discoid lesions heal with…..

Answer 13

scars

Question 14

Musculoskeletal complaints

Answer 14

Arthralgias, arthritis, myalgias, myositis

Arthralgia and myalgia are just joint and muscle pain. The -itis’ are inflammation

Question 15

Ulnar deviation that can be corrected occurs in what dz?

Answer 15

Lupus

Question 16

Ulnar deviation that cannot be corrected (fixed ulnar deviation) occurs in

Answer 16

Rheumatoid Arthritis

Question 17

DrP Key: what kind of genetic deficiency confers the highest risk of dz

Answer 17

Complement Deficiency

Question 18

DrP Key: This mutation is important in lupus and it points to impaired regulation of endogenous nucleic acids

Answer 18

TREX1

Question 19

DrP Key: The production and affect of …… has emerged as central to Lupus Pathogenesis

Answer 19

Type 1 Interferon

Question 20

Renal Disorders in lupus are correlated with the presence of what?

Answer 20

anti-dsDNA antibodies

If renal dz is active the anti-dsDNA is high

Question 21

What is the most common renal structure affected

Answer 21

Glomerulus….they all CAN be affected.

• – RBC casts in Urine
• – And proteinuria…….this is a glomerulonephritis, dawg

Question 22

Psychiatric abnormalities in Lupus?

Answer 22

Depression, psychosis, cognitive abnormalities

Question 23

Neurological dz

Answer 23

STROKE, myelitis,

Question 24

What do you first do if a pt presents with neurological disease like a seizure and they have lupus?

Answer 24

EXCLUDE OTHER CAUSES like a brain tumor

Question 25

Heme manifestation

Answer 25

Anemia, thrombocytopenia, leukopenia

Question 26

Most common pulmonary maifestation

Answer 26

pleuritis with pleural effusion

Question 27

Most common cardiac manifestation

Answer 27

pericarditis

Question 28

What can happen on the valves

Answer 28

Libman sacks endocarditis

Question 29

Obstetrical complications of Lupus?

Answer 29

Small babies, and receurrent fetal loss

Question 30

Anticardiolipin/ antiphospholipid antibody syndrome can cause what?

Answer 30

blood clots, can lead to multiple miscarriages.

Question 31

Lupus classification mnemonis

Answer 31

``` RASH PAIN O MD!! R- renal A- Arthritis S- erositis (inflammation of the serous tissues of the body, pericardium, pleura, inner lining of abdominal cavity) H- hematologic ``` P- Photosensitivity A- ANA positive I- Immunologic N- Neurologic O- Oral ulcers M-- Malar rash D- discoid rash 4/11 needed

Question 32

Neonbatal lupus is due to the transplacental transfer of

Answer 32

Ro (SSA) and La (SSB)

Question 33

WHat is the antibody that everyone with Lupus has?

Answer 33

ANA (anti-nuclear antibody)

Question 34

Lupus antibodies

Answer 34

ANA- everyone has it dsDNA- renal disease in lupus RoSSA- neonatal lupus La SSB- neonatal lupus Anti SM- (smith antibody) very sensitive for lupus Anti phospholid (anticardiolipin)- clots, abortions

Question 35

Clinical lupus check-up

Answer 35

Are you having any problems with allopecia, rashes arthritis? Do you have any trouble taking a deep breath (pleurisy) We're gonna do a urnalysis (rbc cast, proteinuria) CBC (-penia, complements)

Question 36

What is key in the lupus pathogenesis

Answer 36

DEFECTIVE CLEARING OF ANTIGEN-ANTIBODY COMPLEX GOTTA KNOW THAT We don't know what really causes itCDomplement

Question 37

WHere are the autoantibodies coming from in SLE

Answer 37

B cells....these suckers avoid apoptosis and produce bad abs T cells....increased helpers and decreased suppresors

Question 38

Complement activation is huge in Lupus, for example, in the kidney

Answer 38

Immune complexes can form in the circulation and then deposit in the kidney, they may also form in situ

Question 39

What is the major cause of the hemolytic anemia, thrombocytopenia, and vasculitis in SLE

Answer 39

Antibody directed Cell mediated Cytotoxicity (ADCC) Basically, antibody binds to cells, Fc receptors on NATURAL KILLER CELLS bind, target cell dies

Question 40

Anytime you hear ADCC

Answer 40

think NK cells

Question 41

What the fuck is anti-phospholipid syndrome

Answer 41

You get antibodies to the phospholipids involved in clotting. Leads to a jacked up PTT time but somehow a hypercoaguable state. It is significant because it can lead to thrombosis and recurrent fetal loss

Question 42

Sjogren's syndrome is divided into what two types

Answer 42

primary and secondary

Question 43

What are teh three big clinical hallmarks

Answer 43

kerratoconjunctivitis sicca parotid gland swelling xerostima

Question 44

Extraglandular features of sjogrens

Answer 44

fatigue, Raynaud's, polyarthritis, arthralgias, ILD, neuropathy, purpura

Question 45

Classic histological finding in Sjogren's disease is?

Answer 45

mononuclear cell infiltrates in the lacrimal and salivary glands

Question 46

90% of sjogrens pts are what sex?

Answer 46

female

Question 47

What is Schirmer's test?

Answer 47

a test which uses filter paper to document tear flow

Question 48

Sjogren's disease leads to a 44 fold increase in

Answer 48

risk for non-hodgkins lymphoma

Question 49

What type of immune cell is the predominant player in sjogren's syndrome?

Answer 49

T cells (CD4)

Question 50

Common lab findings in Sjogren's syndrome?

Answer 50

ANA, high rheumatoid factor, Anti-RoSSA, Anti- LA/SSB