Review of SLE/Antiphospholipid Syndrome - Postlethwaite/Gupta Flashcards Preview

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Flashcards in Review of SLE/Antiphospholipid Syndrome - Postlethwaite/Gupta Deck (51):
1

4 Forms of Lupus discussed?

SLE (systemic lupus erythematosus)

DLE (discoid lupus erythematosus)

DILE (Drug induced lupus erythematosus)

Neonatal Lupus erythematosus

2

Broad definition of SLE?

Multisystem inflammatory disorder, with autoantibodies to numerous self antigens

3

What is the difference between Discoid Lupus (DLE) and SLE?

- Discoid is confined to the skin; no other symptoms involved.

- However, discoid lesions can be seen in SLE (roughly 5-10% of SLE patients)

4

What is a less severe form of SLE that can be easily resolved? Means of resolving?

Drug Induced Lupus Erythematosus (DILE) resolves once offending drug removed

5

Epidemiology of Neonatal Lupus (dont miss this one)? 

2 Clinical findings? Which one is permanent?

Occurs in newborns of mothers with SLE (hence the term neonatal)

Clinical findings: skin rash (transient), heart block (permanent)

 

6

Epidemiology of SLE

1. Risk of SLE in general population? Was this more or less than RA (fun fact)? 

2. What family relation has the highest risk of having SLE? 

3. Gender, Race most associated with SLE? Others? 

 

Risk of SLE in general population 0.05%. RA was 1%

Monozygotic twins have 25-50% concordance 

Blacks 3-6x more likely, Hispanic and Native American 2-3x, Asians 2x. All as compared to the whites. First you take their land, then they have a 2-3x more likely risk of getting SLE. Silver lining though: they have casinos. No sun in there

Oh, females more likely then men. 9:1 in adults. 2:1 in kids

7

Common causes of death in SLE?

Cardivascular disease (leading cause), Malignancy, Infection, Renal disease

8

Several factors contribute to increased mortality. Dr. P mentioned 3 that can be greatly improved, what are they?

-poor patient adherence

- inadequate patient support system

- limited patient education

9

How many criteria are there for Lupus classification? How many do you need to diagnose them with Lupus? Specificity/Sensitivity associated?

List as many as you can

11 criteria, need 4 to diagnose, if have 4/11 = 95% specificity, 85% sensitivity

First Aid has the mnemonic RASH OR PAIN:

  • Rash (malar or discoid)
  • Arthritis
  • Soft tissues/serositis
  • Hematologic disorder (immune mediated hemolytic anemia, leukonpenia, lymphopenia, thrombocytopenia, smallpenisia)
  • Oral/nasopharyngeal ulcers
  • Renal disease (glomerulonephritis, proteinuria)
  • Photosensitivity/Positive VDRL/RPR
  • Antinuclear antibodies (ANA)
  • Immunologic disorder (antiDNA, antiSm, Antiphospholipid)
  • Nerulogic disorder (seizures and/or psychosis)

sorry for the long slide

 

10

What do most/all lupus patients have in common?

They will have +ANA (95-98% of patients)

11

Relate ANA and Lupus to sensitivity and specificity.

 

Highly sensitive, low specificty for SLE. Presents in many autoimmune disorders as well as some healthy subjects

12

What test is most sensitive for testing for ANA? 

Immunofluoresscence

13

When does the low specificity of ANA in lupus still provide useful information?

 

If you have high suspicion based on symptoms and sign, and +ANA, most likely Lupus

14

When does high sensitivity of ANA in lupus provide useful information?

Patient with negative ANA is unlikely to have lupus

15

Hi, im Dr. Satterfield, I'm going to talk to you about sensitivity and specificity. 

fuck off satterfield. Snout and Spin. we get it. 

16

Besides Lupus, what disorder is also associated with ANA? Percentage stats? 

Scleroderma (95%). Dont forget, lupus 95-98%. 

Others: Hashimotos thyroiditis 50%, Idiopathic pulmonary fibrosis 50%

Normal subjects = 3-4%, know this

17

What antibodies are associated with High specificity for Lupus? Which one has a clinical association

Anti-dsDNA (associated with Lupus nephritis)

anti-Sm (nonspecific)

18

What antibodies are associated with low specificity for Lupus? 

 

ANA, Anti-RNP, Anti-SSA, Anti-SSB

19

What antibody is associated with intermediate specificity for lupus? What clinical association? 

Antiphospholipid, associated with clotting diathesis

20

Anti-SSA and Anti-SSB antibodies are associated with clinical manifestations of lupus?

Also photosensitivity and dry mouth/dry eyes

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21

What complements are decreased with SLE? Which one is classical pathway?

C3, C4, CH50 all decreased. Due to immune complex deposition. C4 part of classical pathway

22

What arthritic disorder is associated with SLE? What does it resemble? What are some distinguishing features?

Jaccoud's like arthropathy, resembles RA. However, it is nonerosive, and deformity is reducible. Unlike RA hand 

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23

Forms of Serositis seen in Lupus? What subclassifcations within these?

  • Pleura
    • Pleuritic chest pain
    • Pleural effusion
  • Pericardium
    • Chest pain
    • Pericardial effusion
  • Peritoneal cavity
    • Abdominal pain
    • Fluid accumulations usually subclinical

24

Kidneys and SLE. 

What % of SLE patients affected?

what antibody is most commonly associated with renal involvment? 

Which renal structures are affected?

50% of SLE patients

anti-DNA antibodies

Any renal structure can be affected (glomerulus, tubules and interstitum, vasculature)

25

Kidney and SLE. Where do immune complexes form. 

Can form in the circulation and deposit in kidney or form in situ

26

Heme and SLE. What cell lines are affected?

All cell lines affected

  • RBC
    • Anemia of chronic inflammation
    • Hemolytic anemia
  • WBC
    • autoimmune lymphopenia
  • Platelets
    • autoimmune thrombocytopenia

27

What neuropsychiatric disorders can be seen in SLE?

All kinds. Psychiatric (depression,  psychosis, cognitive abnormalities). Neurologic (brain, spinal cord, peripheral nerves)

28

Of the 11 clinical manifestations, which one is less commonly affected?

GI tract

29

Fertility and SLE?

Usually not affected.

However 

- small for gestational age fetuses

- recurrent fetal loss (antiphospholipid antibody)

30

Rash, leukopenia, heart block. What are you thinking? 

Neonatal lupus

31

Heart and SLE. Which is more common, pericarditis or myocarditis?

Pericarditis more common

32

What valve disorder is associated with Lupus? Key features of it?

Libman Sacks Endocarditis

- nonbacterial (i.e. no fever. so no cowbell)

- involves both sides of valve

33

Heart and SLE. SLE is a strong risk factor for? What factors can worsen this risk?

premature coronary artery disease. Use of corticosteroids and inflammation can worsen risk

34

What vasculature disorders are associated with Lupus?

Vasculitis, Raynauds

35

What feature is least present in Drug induced lupus? What is the exception to this?

Nephritis, unless patient was taking anti-TNFalpha meds (Embrel, Humera)

36

What drugs are most likely to produce DILE?

Ones he mentioned: Hydralazine, Procainamide, Isoniazid, Minocycline, TNF inhibitors, Interfeuron-alpha

Others: Hydantoins, chlorpromazine, methyldopa, penicillamine,

37

Drugs used to treat SLE?

Corticosteroids, Cyclophosphamide, MTX

38

There are many side effects of corticosteroid use in SLE. Dr. P mentioned one that was important. What is it?

Avascular necrosis of femoral head

39

Antiphospholipid antibody syndrome is defined as? 

hypercoaguable state due to antiphospholipid antibodies (especially lupus anticoagulant)

40

Antiphospholipid syndrome consists of a family of autoantibodies directed againsts what? Most common?

directed against phospholipid-binding plasma proteins, most commonly Beta 2 glycoprotein I

41

Diagnosis of Antiphospholipid antibody syndrome requires what?

Patient must have both:

  1. A clinical event such as:
    • Thrombosis
    • Pregnancy morbidity
  2. Persistent presence of an antiphopholipid antibody (aPL) such as:
    • lupus anticoagulant
    • IgA anticardiolipin antibody
    • IgA anti Beta2 glycoprotein antibodies

42

Relate anticardiolipin to the general population (hint, low and high values)

Low titer anticardioplin found in 10% of normal blood donors

High titer anticardiolipin or a positive lupus anticoagulant test occurs in less than 1%. 

KEY FACT: Just bc they test + for anticardiolipin doesnt necessarily mean they can be diagnosed with antiphospholipid syndrome. Remember, they must have a clinical presentation as well (thrombosis or pregnancy morbidity)

43

In antiphospholipid syndrome, what is the most common presentation of arterial involvement? of venous involvement?

Arterial = stroke. Venous = DVT

44

Path of SLE. What 2 sites are common biopsy sites? What is not?

Skin and renal biopsy = common, Joint is not

45

Path of SLE. What 2 skin histologic findings are seen in SLE? 

Superficial and deep perivascular inflammation

Mucin from degeneration is seen in dermis

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46

Path of SLE. What immunofluorescent finding on skin is seen in SLE. Specific for SLE?

Granular IgM, IgG, and complement at the dermal-epidermal junction. Healthy people can also have this. Can also be seen in scleroderma or dermatomyositis

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47

How many classes of Lupus Nephritis? Most common and involvement of this class?

6 Classes. Class IV most common: diffuse proliferative GN (>50% of glomeruli)

48

What class is this? Describe what you see here?

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Class III: focal proliferative GN (<50% of glomeruli involved). Extracapillary proliferation is not prominent in this case

 

49

What are the black arrows pointing at?

What class is this associated with?

What is the pathophysiology behind these structures?

Q image thumb

Wire loops

class IV (diffuse proliferative GN)

Due to extensive subendothelial deposits of immune complexes

50

Immunofluoresence of Lupus Nephritis shows?

Deposition of IgG antibody in a granular pattern

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51

Treatment for Antiphospholipid syndrome?

Place them on heparain, then convert them to coumadin