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*MHD Exam 5 > Autoimmunity > Flashcards

Autoimmunity Flashcards

1
Q

Autoimmune diseases can be divided into 2 categories:

A
  1. Organ-specific (Grave’s disease, Type I DM)

2. Systemic (SLE, systemic sclerosis)

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2
Q

What is the simplified pathogenesis behind SLE?

A

failure to maintain self-tolerance -> auto Ab formation -> tissue damage

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3
Q

What is the ACR Classification Criteria?

A

It is a way to diagnose/classify SLE. If a patient has 4/11 of the criteria, the test is 95% specific for Lupus. However, it is not very sensitive (only 85% sensitive).

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4
Q

Which signs/symptoms are part of the ACR Classification Criteria?

A 
SOAP BRAIN MD 
S=serositis
O=oral ulcers
A=ANA
P=photosensitivity 
B=blood disorder
R=renal disorder
A=arthritis
I=immunologic abnormalities (anti-Smith, anti-dsDNA, anti-phospholipid) 
N=neurologic symptoms
M=malar rash
D=discoid rash
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5
Q

What is the ACR/EULAR criteria for SLE?

A
  • ANA titer >1:80 = diagnosis of Lupus

- 10+ entry criterion (signs and symptoms) = diagnosis of Lupus

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6
Q

List examples of the Lupus intangibles.

A

fatigue, brain fog, achiness, depression, joint pain

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7
Q

List examples of external manifestations of Lupus.

A

malar rash, discoid rash, subacute cutaneous rash, Raynaud, alopecia, Jaccoud deformity

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8
Q

List examples of internal manifestations of Lupus.

A

lupus nephritis, pericarditis, pleurisy, hemorrhagic stroke

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9
Q

Explain the classic findings in Lupus nephritis.

A
  • immune deposits (primarily due to anti-dsDNA Abs; also deposition of IgG, IgA, IgM, C1q, C3)
  • immune deposits can occur in mesangium, subendothelial, and/or subepithelial components of glomerulus
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10
Q

Libman-Sacks Endocarditis (LSE) is seen with which autoimmune disorder?

A

SLE

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11
Q

What are the 5 subtypes of Lupus?

A
  1. SLE (systemic)
  2. Discoid lupus (contained to skin)
  3. Drug-induced lupus
  4. Neonatal lupus
  5. Overlap syndrome (SLE associated w/ another autoimmune disorder)
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12
Q

Describe the general sensitivity and specificity of the anti-nuclear antibody (ANA).

A

It is non-specific but very sensitive; therefore, it is most useful when negative (as a screening test) to rule out certain conditions.

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13
Q

Which antibodies have a high specificity for SLE?

A

anti-dsDNA and anti-Smith

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14
Q

What are the general treatments for SLE?

A
  • NSAIDs
  • Anti-malarials
  • Corticosteroids
  • Immunosuppressive meds
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15
Q

What are the “activity markers” of SLE that can be measured with laboratory testing?

A
  • complement 3 and 4 (decreased)

- dsDNA

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16
Q

What are adjunctive measures that are important in preventing SLE flare-ups?

A
  • avoiding sun exposure
  • not smoking
  • eating a healthy diet
  • exercising regularly
17
Q

What are the major causes of mortality in SLE?

A

cardiovascular and renal diseases

18
Q

Do males or females get SLE more commonly? Do males or females get a more severe version of SLE?

A

females get SLE more commonly (9:1 ratio), but males get a more severe version

19
Q

What is Sjogren’s syndrome characterized by?

A

exocrine glandular dysfunction due to lymphocytic infiltration

20
Q

What are the main clinical manifestations of Sjogren’s?

A
  • dry eyes (xerophthamia)
  • dry mouth (xerostomia)

(but patients can also have vaginal dryness, skin dryness, constipation, parotid enlargement, joint pain)

21
Q

What are the laboratory abnormalities associated with Sjogren’s?

A
  • (+) anti-SSA and/or anti-SSB Ab
  • (+) Rheumatoid factor
  • RTA
  • focal lymphocytic sialadenitis on labial salivary gland biopsy
22
Q

Sjogren’s patients have an increased risk of:

A

lymphoma (40x increased risk, esp. mucosa-associated tissue, aka MALT)

23
Q

Why do we worry about pregnant women with Sjogren’s?

A

We worry because it can cause congenital heart block in the newborn (newborn would need a permanent pacemaker implanted).

24
Q

Scleroderma (systemic sclerosis) is characterized by a triad of:

A
  1. Autoimmunity
  2. Noninflammatory vasculopathy
  3. Collagen deposition and fibrosis
25
Q

What is the difference b/t Raynaud disease and phenomenon?

A
  • Disease: when primary/idiopathic

- Phenomenon: when secondary to autoimmune disease (Scleroderma, SLE, mixed CT disease)

26
Q

What is the treatment for Raynaud?

A

vasodilators (like CCBs)

27
Q

What are the clinical manifestations of systemic sclerosis?

A
  • Raynaud
  • skin tightening
  • finger tip ulcers
  • joint contractures
  • renal HTN
  • dyspnea
  • GERD
  • diarrhea/malabsorption
  • weight loss
28
Q

What are the subtypes of systemic sclerosis?

A
  1. Diffuse: extensive skin involvement, interstitial lung disease, anti-Scl70 or RNA polymerase 3
  2. Limited (CREST syndrome): limited skin involvement (fingers/face) w/ Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactyly, and Telangiectasia
29
Q

What are the scleroderma antibodies?

A

anti-centromere, anti-topoisomerase (Scl70), anti-RNA polymerase III

30
Q

Which antibody is associated with scleroderma renal crisis?

A

anti-RNA polymerase III

31
Q

Which antibody is associated with CREST syndrome (limited scleroderma)?

A

anti-centromere

32
Q

Anti-phospholipid syndrome is most commonly associated with _________.

A

SLE

33
Q

Mortality from SSc is often related to what?

A

pulmonary complications (HTN, interstitial lung disease)

34
Q

What are the anti-phospholipid Abs?

A
  • anticardiolipin Abs (aCL)
  • anti-beta2-glycoprotein I Abs
  • lupus anticoagulant (LAC)
35
Q

Anti-phospholipid Abs associated with anti-phospholipid syndrome can cause abnormalities in which lab results?

A
  • false (+) VDRL/RPR test (for Syphilis)

- prolonged PTT

*MHD Exam 5 (14 decks)

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