Systemic Lupus Erythematosus Flashcards

1
Q

What is SLE?

A
  1. A collagen vascular disease
  2. Autoimmune disease
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2
Q

What are some common histological features of SLE?

A
  1. Inflammatory damage to connective tissue and blood vessels (due to the fibrosis that collects)
  2. Fibrinoid material deposition
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3
Q

What cells are involved with the immune response that SLE causes?

A
  1. Lymphoid stem cell
  2. B-cell
  3. T-cell
  4. Macrophage, monocyte
  5. Natural killer cells
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4
Q

What is the prevalence and demographic of SLE?

A
  1. 9 to 1 female to male (85% female)
  2. More common in black women
  3. Peak age is 15-25
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5
Q

When do most causes of SLE develop?

A
  1. Most develop between menarche and menopause
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6
Q

What are some of the genetics factors that play into SLE?

A
  1. HLA DRB 2/3 classes
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7
Q

What are some bodily responses that SLE causes?

A
  1. Widespread inflammation in multiple organs, blood vessels and other connective tissues
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8
Q

What is the inflammation caused by?

A
  1. Profound immune alteration that leads to the development of T cell and antibody responses
  2. The tissue damage occurs as a result of cell mediated immune response and direct damage caused by auto-antibodies or accumulation of immune complexes
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9
Q

What causes the symptoms of SLE?

A
  1. The symptoms are a direct result of the formation of the immune complexes that deposit within various tissues
  2. The immune complexes then recruit complement and inflammatory cells to cause disease
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10
Q

What type of hypersensitive is SLE?

A

Type III
* due to the formation of and collection of the immune complexes

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11
Q

What are the different types of hypersensitivity reactions?

A

Type I: Reaction mediated by IgE
Type II: cytotoxic reaction mediated by IgG or Ig antibodies
Type III: reaction mediated by immune complexes
Type IV: delayed reaction mediated by cellular response

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12
Q

What is the etiology of SLE?

A

The cause is unknown
*Thought to be a multi-faceted etiology
*possibly due to genetics or environmental

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13
Q

What is the clear gender and cultural bias for SLE?

A
  1. Women are 10 times more likely than men to get SLE
  2. Black women and Hispanic women are more likely to get SLE than whites women
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14
Q

What are the environmental factors that can trigger SLE?

A
  1. UV light (someone in remission that was out in the sun can get a SLE flare up)
  2. Infectious agents
  3. Certain drugs
    *Drug induced lupus DOES NOT mean SLE
    *Drug induced lupus is a flare up
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15
Q

What are the SLE clinical manifestations due to?

A
  1. Trapping of antigen-antibody complexes in capillaries of visceral structures (causing damage to organs)
  2. Autoantibody-mediated destruction of host cells
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16
Q

What leads to complement-mediated lysis?

A
  1. The auto antibodies that are specific for RBC and platelets
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17
Q

What is the criteria for diagnosing SLE?

A
  1. If 4 or more of the 11 criteria are present, serial, or simultaneously during any interval of observation
    *The patient may have SLE and not have 4 of the criteria
18
Q

What is the pneumonic for the SLE criteria?

A

M D S O A P
B R A I N

19
Q

What is M for?

A

Malar rash
*Butterfly rash
*bridge of nose, checks, forehead, chin

20
Q

What is D for?

A

Discoid rash
*raised circular rash
*on sun exposed areas

21
Q

What is S for?

A
  1. Serositis
    * pleuritis, pleural effusion
    *Pericarditis, pericardial effusion
    *Ascites (fluid in the abdomen, liver involvement)
22
Q

What is O for

A

Oral ulcers

23
Q

What is A for?

A
  1. Arthritis (With swelling)
    *Not just joint pain, there will also be swelling
  2. Arthritis (jaccouds)
    *happens during a flare
    *will go away during remission
24
Q

What does P stand for?

A

Photosensitivity
* in sun exposed areas
*looks like a sunburn

25
Q

What does B stand for?

A

Blood abnormalities *bc the body is attacking itself and the blood cells
1. Leukocytopenia (<4,000 on 2+ occasions)
2. Lymphopenia (<1500 on 2+ occasions)
3. Hemolytic anemia
4. Thrombocytopenia (<100,000)

26
Q

What does R stand for?

A

Renal
1. Proteinuria (>500mg/24hrs)
2. Cellular casts

27
Q

What does A stand for?

A

Anti-nuclear antibodies (ANA)
1. Rim
2. Diffuse
3. Nuclear
4. Speckled (less conclusive)

28
Q

What does I stand for?

A

Immune abnormalities
*Get an ANA first and if that is really high order these test
1.Smith antibody
2. Anti-ds (double stranded) DNA antibody
3. Anti-phospholipid antibody
*Anti-cardiolipin antibody (IgG or IgM)
*Biologic false positive VDRL
*Lupus anticoagulant

29
Q

What does N stand for?

A

Neurologic *change in a persons baseline behavior
1. Seizure
2. Psychosis

30
Q

What are the head and neck manifestations of SLE?

A
  1. Malar rash is usually the first sign
  2. Erythematous maculopapular eruption after sun exposure
  3. Oral ulceration
  4. 3-5% perforated nasal septum
  5. Acute parotid enlargement 10%
    *if there is parotid enlargement and joint pain (90%) then SLE
    *ddx sjogren syndrome
31
Q

What is drug induced lupus?

A
  1. Nephritis and CNS features not present
  2. Hypocomplementemia and antibodies to double-stranded DNA are absent
  3. When drugs are discontinued person will return back to normal
  4. ANA is positive for anti-histone (does not happen with SLE)
32
Q

What is a common feature of SLE?

A

Joint pain
* 90% of patient have joint pain
*will not be erosive
*reversible swan neck occurs during flares goes away once in remission

33
Q

What labs should you order if you’re suspecting someone with SLE?

A
  1. CBC with diff
  2. Serum creatinine
  3. UA with urine sediment
  4. Serum protein electrophoresis (not common, will not be the first lab you order)
  5. ESR, CRP
  6. ANA
  7. Antibodies to double-stranded DNA (correlates with severity) and Smith
  8. Depressed serum complement (C3 and C4 or CH50)
34
Q

What is definite, probable, and possible SLE?

A

D: meets 4/11, 4/17 of the criteria
Probable: 2-3
Possible: 1 or 2

35
Q

What are the current therapies for SLE?

A
  1. Immunosuppressants
    *cyclophosphamide
    *Azathioprine
36
Q

Do therapies cure SLE?

A

No they only alleviate symptoms

37
Q

What is the first line treatment if SLE involves joint symptoms and skin symptoms?

A
  1. Antimalarials *hydroxychloroquine-plaquenil
    *have annual monitoring for retinal changes
38
Q

When would corticosteroids be used for SLE treatment?

A
  1. If there is a life-threatening manifestations due to major organ involvement
  2. Start with lowest dose possible
39
Q

What is the first line treatment for SLE if there is issues with the kidney or systemic issues?

A
  1. Low dose methotrexate
  2. Azathioprine, cyclophosphamide *improves renal survival but not patient survival
    *use if resistant to corticosteroids
40
Q

What are the biologics treatment for SLE?

A
  1. Belimumab
  2. Rituximb
    *only if the patient has tired everything, a last resort
41
Q

What is the prognosis for SLE?

A
  1. Usually a bimodal mortality pattern
    *infection in early yers=death
    *Atherosclerosis in later years=death
  2. Risk of AVN (Avascular necrosis)
    *Bone is dying to the lack of blood supply
    *Prednisone increases risk of AVN