Lupus I Flashcards

1
Q

Characteristic marker of SLE

A

Anti-nucleic acid autoantibodies (ANAs)

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2
Q

Rates of SLE have ___ in the past 40 years

A

Rates of SLE have more than tripled in the past 40 years

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3
Q

The gender ratio for SLE is __:__ female:male

A

The gender ratio for SLE is 9:1 female:male

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4
Q

Suggested reasons for gender preference in SLE

A
  • Role of estrogen in disease
  • X-linked genes associated with risk
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5
Q

Onset of lupus

A

Anywhere from ~10 to 55 years, mean age in 30’s

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6
Q

Common causes of mortality in Lupus patients

A
  • Cardiovascular disease
  • Renal failure
  • Infection
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7
Q

Typical treatments for Lupus

A
  • Corticosteroids
  • Other immunosuppressants (cyclophosphamide, etc)
  • Antimalarials
  • Appropriate treatment of comorbidities
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8
Q

__ of __ criteria are needed for the diagnosis of SLE.

A

4 of 11 criteria are needed for the diagnosis of SLE.

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9
Q

Genes which predispose to SLE

A
  • C1q and C4 (complement)
  • Lack of C4 functionality leads to decreased elimination of autoimmune B cells
  • Lack of C1q functionality leads to decreased clearance of necrotic debris
  • CD40 (necessary for B cell longevity and class-switching)
  • Fc receptors (variants which bind to Igs poorly and make ICs harder to clear)
  • Type I IFN variants
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10
Q

Criterion table for SLE

A
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11
Q

SLE patients have very high baseline levels of serum ___.

A

SLE patients have very high baseline levels of serum Type I IFNs.

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12
Q

Although the spectrum of autoantibody specificities in SLE is ___, only a few have ___.

A

Although the spectrum of autoantibody specificities in SLE is extensive, only a few have been shown to contribute to disease-related tissue injury.

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13
Q

Anti-CD3 and Anti-TCR

A
  • Suppress IL-2 production
  • Poor T cell proliferation results
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14
Q

Anti-DNA, anti-histone, and anti-chromatin

A
  • Main drivers of immune complex formation in SLE
  • Result commonly in kidney disease and neuropathy
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15
Q

Anti-Ro antibodies

A
  • Alter function of myocytes and cells in the conduction system of the heart
  • Linked to neonatal Lupus and congenital heart block
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16
Q

Anti-NMDAR antibodies

A
  • Often a result of cross-reactivity due to NMDAR and DNA similarities
  • NMDAR is expressed on CNS neurons, and so this antibody in CSF may lead to CNS neuropathy and cognitive deficits
  • Resulting inflammation also compromises the blood-brain barrier
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17
Q

Antiphospholipid antibodies

A
  • Interfere with coagulation system and endothelial cell function
  • Induce chronic expression of adhesion molecules and tissue factor, promoting thrombosis
  • May also cross-link platelets into larger aggregates
18
Q

Antibody therapies for SLE

A
  • Rituximab (B cell depletion)
  • Belimumab (Anti-B-lymphocyte-stimulator, important B cell growth/survival factor)
19
Q

The pathogenesis of SLE hinges on ___ and ___

A

The pathogenesis of SLE hinges on loss of tolerance and sustained autoantibody production

20
Q

Circulating apoptotic microparticles that cause SLE also . . .

A

. . . trigger neutrophil NETosis! This provides even more fodder for SLE autoantibody development

21
Q

Current pathogenesis model for SLE

22
Q

SLE diagram

23
Q

Podocytes

A

Cells of Bowman’s capsule with appendages that make contact with the endothelium of the glomerulus across the basement membrane.

24
Q

Tubule cells

A

Surround podocytes in Bowman’s capsule and make up the rest of the capsule.

25
\_\_% of SLE patients have positive ANA titers.
**95-99%** of SLE patients have positive ANA titers.
26
SLE "specific" antibodies
* Anti-ssDNA * Anti-dsDNA * Anti-Sm (nucleoplasmic protein)
27
SLE "nonspecific" antibodies
* Anti-histone * Anti-Ro * Anti-LA * Anti-U1RNP * Rheumatoid factor
28
One of the most difficult aspects of lupus is knowing whether. . .
. . . the patients are having a flare, an infection, or a drug eruption They require completely different modes of treatment
29
Malar rash
30
How to avoid confusing malar rash and rosacea
* Malar rashes never have papules or pustules * Malar rashes never have telangectasias * Malar rashes always spare the orolabial and nasolabial folds
31
Patients with lupus describe sunlight as. . .
. . . worsening their rashes and making them feel fatigued or ill
32
Discoid rash
33
Characteristics of discoid rash
* Areas of hypopigmentation with hyperpigmented borders * Raised/elevated * Occurs frequently on the scalp, face, and neck * Result of chronic inflammation in Lupus
34
Oral and nasal rashes in Lupus
* Often non-painful, as opposed to Herpes * Often unreported and must be observed on HEENT exam
35
Serositis
* Inflammation of lining of body cavities (pleura, pericardium, peritoneum) * Extremely painful * Causes buildup of inflammatory cells and fluid * Pericarditis may lead to **tampanade** (hydrostatic pressure resiting blood pumping) * Pleuritis may lead to shortness of breath with decreased sounds in lower lung fields
36
\_\_\_\_ nephritis in Lupus requires immediate attention
**Class III or above** nephritis in Lupus requires immediate attention
37
Jaccoud's arthropathy Finding of chronic lupus arthritis. Substantial ulnar deviation in the absence of bone erosion.
38
Lupus may lead to ___ by several different mechanisms These include:
Lupus may lead to **anemia** by several different mechanisms These include: * Hemoltyic anemia * Iron deficiency anemia * Anemia of chronic disease
39
Even if platelet counts are low in patients with Lupus. . .
. . . they are still at risk for thrombosis! This is due to the effects of autoantibodies against thrombocytes
40
Neurological symptoms in Lupus may include
* Stroke * Seizure * Psychosis * Worsening depression * Worsening executive functioning * Other neurological dysfunction
41
While not required or specific, Lupus patients often display \_\_\_.
While not required or specific, Lupus patients often display **Reynaud's phenomenon**
42
Libman-Sack's Endocarditis
* Formation of clotts and thrombosis on heart valves * **Posterior leaflets of aortic and mitral** valves involved * NOT infectious * May be a source of emboli