Approach to Hypersensitivity and Autoimmune Conditions Flashcards

1
Q

4 steps of Type I Hypersensitivity

A
  1. antigen exposure
  2. IgE cross linking on mast cell/basophil
  3. Mediator (histamine, PG, leukotriene, tryptase) release
  4. Sx within min of exposure & can return 4-8 hrs later
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2
Q

Sx of Type I Hypersensitivity

A

urticaria (hives), rhinitis, wheezing, diarrhea, vomiting, hypotension, and anaphylaxis

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3
Q

Examples of Type I Hypersensitivity

A

Pollen allergies, dust mite allergy, bee sting

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4
Q

3 mechanisms of Type II Cytotoxic Hypersensitivity

A
  • Opsonization (for phagocytosis)
  • Complement-mediated lysis
  • Ab-dependent cellular cytotoxicity
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5
Q

2 Ig types involved in Type II Cytotoxic Hypersensitivity related cell destruction

A

IgG and IgM

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6
Q

Examples of Type II Cytotoxic Hypersensitivity

A

ABO mismatch, Graves dz, Myasthenia gravis

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7
Q

Characteristics of Myasthenia gravis (Type II hypersensitivity)

A

Ptosis, mechanism: prevents Ach from binding Ach-R.

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8
Q

3 steps of Type III Immunocomplex Hypersensitivity

A
  1. Antigen-antibody complex formation
  2. Activates complement/neutrophil invasion into tissues
  3. Tissue inflammation leading to sx
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9
Q

Sx of Type III Immunocomplex Hypersensitivity

A

fever, urticaria (hives), generalized lymphadenopathy, arthritis, glomerulonephritis, vasculitis, SOB

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10
Q

Examples of Type III Immunocomplex Hypersensitivity

A

SLE, RA, Farmer’s Lung

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11
Q

2 steps of Type IV Cell-Mediated Hypersensitivity

A
  1. Antigen exposure activates sensitized T-cells
  2. Tissue inflammation 48-96 hrs after antigen exposure
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12
Q

Examples of Type IV Cell-Mediated Hypersensitivity

A

poison ivy rash, ppd for TB testing

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13
Q

Example of? (hint a hypersensitivity)

A

Type IV Cell-mediated hypersensitivity

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14
Q

Name the condition:

  • Autoimmune systematic inflammatory dz affecting synovial membranes
  • Granulation tissue in joint spaces and erodes articular cartilage and bone
A

RA

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15
Q

For RA:

A
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16
Q

Presentation of RA:

  • joint ____, (color) , (temperature) , and ___ ROM
  • morning, >1 hr ______
  • sites most affected: list 5
  • lab findings:
      • or - rheumatoid factor
      • or - anti CCP antibody
    • elevated or normal ESR and CRP
A
  • joint swelling (soft), erythema, warmth, decreased ROM
  • morning >1 hr stiffness
  • PIP, MCP (boutonniere deformity), wrist, knees ankles
  • lab findings:
      • rheumatoid factor
      • anti CCP antibody
    • elevated ESR and CRP
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17
Q

Patients with RA are at increased risk for 3 things:

A
  • infection from immunosuppresion
  • leukemia/lymphoma
  • CV disease
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18
Q

Name the condition:

  • Collagen vascular disorder w/ persistent inflammation in 1+ joints for 6+ weeks in patients < 16 yo
A

Juvenile idiopathic arthritis (JIA, aka juvenile RA)

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19
Q

For JIA:

A
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20
Q

Which of the 3 JIA presentations is the following?

  • Large joints, asymmetric
  • Iridocyclitis (inflammation involving ciliary body)
  • Uveitis
A

Pauciarticular

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21
Q

Which of the 3 JIA presentations is the following?

  • Large and small joints, symmetric
A

Polyarticular

22
Q

Which of the 3 JIA presentations is the following?

  • Recurrent, high fevers
  • Myalgias, pericarditis, lymphadenopathy, etc.
  • Anemia, leukocytosis
A

Systemic (Still’s Disease)

23
Q

Workup for JIA

A
  • CBC
  • ESR (erythrocyte sedimentation rate)
  • RF/ANA (rheumatoid factor/antinuclear antibody)
  • X-rays
  • Synovial fluid (check for leukocytosis/elevated protein)
24
Q

Name JIA presentation corresponding to complication

  1. Sx wax and wane over time
  2. half get destructive arthritis, complete resolution rare
  3. 70% go into remission after several yrs
A
  1. Polyarticular
  2. Systemic (Still’s disease)
  3. Pauciarticular
25
Name the condition: * Inflammatory disorder common among African Americans characterized by reccurent occurence/remissions, secondary to autoantibody formation and immune complex deposition
Systemic Lupus Erythematosus (SLE)
26
For SLE:
27
To be diagnosed with SLE how many of the following sx does a patient need to have? * Pleuritis, pericarditis, myocarditis * Oral aphthous ulcers * Arthritis * Photosensitivity * Hemolytic anemia, thrombocytopenia, leukopenia, lymphopenia * Proteinuria or urinary cellular casts * Positive ANA * Positive anti-dsDNA, anti-SM, antiphospholipid * Lupus cerebritis, seizures, psychosis * Malar rash (photosensitive butterfly rash) –Discoid rash
At least 4
28
Name the condition: * Erosions: very rare * Morning stiffness: minutes * Deforming arthritis: uncommon * ESR and CRP slightly elevated
SLE
29
Name the condition: * Erosions: common * Morning stiffness: hours * Deforming arthritis: common * ESR and CRP significantly elevated
RA
30
2 reasons patients usually die from SLE:
1. end organ damage 2. opportunistic infections due to immunosuppresion
31
Name that condition: * Chronic, hyperproliferative inflammatory disorder characterized by thick adherent scales * Males and females equally affected w/ genetic component
Psoriasis
32
Presentation of Psoriasis: * Mild \_\_\_\_\_ * *_(color)_* plaques w/ adherent *_(color)_* scales * Involves _____ surface * Bilateral or unilateral? * _____ pitting * Can potentially worsen to \_\_\_\_\_
* Mild pruritus (itch) * Salmon pink plaques w/ adherent silver white scales * Involves extensor surface * Bilateral * Nail pitting * Can potentially worsen to arthritis
33
Workup for Psoriasis
PE: Auspitz sign – pinpoint bleeding after remove scale Biopsy (sometimes)
34
Name that condition: * Demyelinating disorder of the CNS * Peak incidence around 20-40 yo * Females \> males and maybe genetic component
MS
35
Presentation of multiple sclerosis (MS): * Hint: the "MS rollercoster"
While riding the **MS** rollercoster I felt **electricified** but upon getting off, I had trouble walking. My legs were **weak, tingling and numb**. I was **dizzy**, my **vision was blurred.** I needed to **sit on the toilet for a while** to recover. * Vision changes * Vertigo * Weakness * Numbness/tingling and/or pain * Urinary incontinence or retention * Lhermitte’s sign – electrical sensation running down spine and LE with neck flexion
36
Workup for MS
* MRI * CSF
37
Clinical signs that suggest a primary immunodeficiency disease
38
Which of the 4 primary immunodeficiencies is the following? * Present in first 3-4 months of life * Disseminated intracellular diseases
T cell
39
Example of T cell primary immunodeficiency
DiGeorge Syndrome (Thymic Aplasia, Chromosome 22 del)
40
Which of the 4 primary immunodeficiencies is the following? * Present after 6 months of age (maternal antibodies disappear) * Sinopulmonary and GI infections
B cell
41
Which of the 4 primary immunodeficiencies is the following? * Combination of T-cell and B-cell features
T cell / B cell combined
42
Which of the 4 primary immunodeficiencies is the following? * Sinopulmonary and soft tissue infections
Phagocytic
43
Example of B cell primary immunodeficiency
Common Variable Immunodeficiency (CVID) * presents w/ spenolmegaly, lymphadenopathy
44
Example of T cell / B cell combined primary immunodeficiency
Severe Combined Immunodeficiency (SCID) * Onset 3 mos * Presents w/ diarrhea, pneumonia, failure to thrive, otitis
45
Example of phagocytic cell primary immunodeficiency
Chediak–Higashi syndrome * albinism, neuropathy
46
Name that condition: * Spread almost exclusively through body fluid transmission * Male \> female
HIV/AIDS
47
Presentation of Human Immunodeficiency Virus, Acquired Immunodeficiency Syndrome (HIV/AIDS): * Initially \_\_\_\_\_, followed by \_\_\_\_\_-like sx
* Initially asymptomatic, followed by flu-like symptoms
48
Workup for HIV/AIDS
* ELISA * Western Blot * HIV RNA viral load
49
Diagnosis of AIDS requires (2 possibilities):
* CD4 count \<200 OR * Presence of AIDS defining illness (virus induced sx)
50