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2020 Flashcards

1
Q

3 investigations that would help with HES diagnosis, and 2 mutations

A

Hypereosinophila

3 investigations

  1. Bone marrrow biopsy for specific stains such as PDGFRA-1 and PDGFRB2
  2. IgE level
  3. LSUB for CD3-CD4+ which is associated with L-HES subtypes

Mutation

  1. JAK2
  2. FGFR
  3. PDGFRA-1 and PDGFRB-2
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2
Q

Role of filaggrin mutation, how does it contribute to AD?

A
  • filaggrin mutation leads to decreases integrity of the skin barrier function
  • this contributes to AD bc of impaired moisturizuration of the skin enhanced infectious inflammation and breaches in the skin barrier
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3
Q

4 reasons for poor AD control

A
  1. poor compliance
  2. poor understanding of the treatment plan
  3. Underlying contributers to the diagnosis such as PID or nutritional deficiencies (zinc)
  4. inadequate dosing
  5. wrong diagnosis
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4
Q

Scoring systems in AD

A
  1. EASI - measures extent of body SA affected
    - uses number score for redness, thickening, scratching, lichenification
  2. SCORAD - uses rule of 9s to measure extent of eczema
  3. POEM - (pt oriented eczema measure)
    - questionaries that measure days of the week involvement of eczema sx
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5
Q

Global suppression after measles, what lab changes do you expect

A
  1. T cell lymphopenia
  2. poor T cell response to mitogens
  3. ?decreases Abs
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6
Q

investigations in sarcoidosis

A
  1. HRCT
  2. BAL
  3. bronchoscopy and biopsy
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7
Q

biologic used in sarcoidosis

A

Inflixumab

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8
Q

stages of HP and imaging finding

A
  1. Acute
    - CXR normal
    - PFTs restrictive,
    - HRCT shows mediastinal lymphadenopathy and ground glass opacities
  2. Subacute
    - HRCT: micronodular pattern on HRCT
  3. Chronic
    - fibrotic lung disease, w clubbing and hypoxia
    - HRCT: honey combing, air trapping, and micronodules
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9
Q

histology of HP

A
  • poorly formed non caseating granulomas or mononuclear infiltrates with giant cells
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10
Q

Target and MOA of Dupilumab

A

IgG4 binds to IL-4R, which inhibits IL4 and IL13

MOA - by binding to IL4 and IL13 it decreases the cytokines that mediate AD

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11
Q

CRSwNP - what are two cytokines involved and two tx other than INCS

A

IL4, IL5
mab treatment -

  1. Xolair (18+)
  2. Dupilumab (18+)
  3. Mepolizumab (18+ w CRwNP)
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12
Q

Indications for Nucala (Mepolizumab)

A
  1. add on maintenance tx for > 6 year olds with severe asthma and with an eosinophilic phenotype
  2. add on maintenance for of adult patients 18+ with CRSwNP
  3. treatment of adults with EGPA
  4. treatment of adults and peds > 12 years with HES for > 6 months without an identifiable non-hematological 2 cause
dosing 
12+ - 100 mg SC Q4weeks 
6-11 - 40 mg SC  Q4weeks
CRSwNP 100 mg SC Q4 weeks 
EGPA 300 mg (100mg injections sep) Q4 weeks
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13
Q

occupational asthma from nail salon

  1. what is the allergen
  2. 3 mechanisms
A
  1. acrylamides
  2. immune mediated - IgE mediated
    non-immune mediated - reactive airway dysfunction syndrome
    irritant - irritant chemical, dust, fumes
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14
Q

OIT major antibody and mechanism of tolerance

A

Major antibody - IgG4

  1. shift from TH2 cytokine release to TH1
  2. increase in IgG4
  3. increase in IL-10 and TGF b
    these actions lead to T cell anergy which contributes to OIT
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15
Q

what are the consequences of venom IT

A

decreased IgE
increased IgG4
increased IL10 and TGFB

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16
Q

when would you not skin test in VIT? different methods?

A
  1. Overlying skin infection
  2. Patient did not d/c appropriate medications (AH or INAH)
  3. Patient has previous anaphylaxis to skin testing
  4. Dermatographism
  • SPT with 100 mcg/ml
  • ID with 1 mcg/ml
  • sIgE
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17
Q

persistent hypotension with Epi admin, pt on BB what drug do you give and dosing

A

Glucagon 1-5mg over 5 mins

18
Q

4 adverse reactions to SCIT

4 CI to HDM SLIT

A

SCIT AE

  1. Anaphylaxis
  2. LLR
  3. systemic hives
  4. syncope?
  5. asthma exacerbation

4 CI to HDM SLIT

  1. serve uncx asthma
  2. uncontrolled cardio resp disease
  3. hypersens rxns to any ingredients in SLIT
  4. Pregnancy
  5. EoE
19
Q

disadvantages and advant to SCIG over IVIG

A
  1. requires adherent patient
  2. requires equipment at home
  3. requires weekly injections instead of monthly
  4. requires some tricky equipment

advantages

  1. no IV access
  2. slower admin leads to consistent peak of IgG
  3. home access
  4. fewer sx SEs
20
Q

Pemphigus vulgaris IIF findings

A
  • IgG antibodies DSG1, and 3, desmoglein ab and IgG deposits
  • affects the surface layer of the skin and epidermis
21
Q

Bullous pemphigus IIF findings

A
  • IIF shows IgG and complement deposits at the epidermal basement membrane
  • blisters form in the dermoepidermal junction
22
Q

dermatitis heptiformis IIF findings

A
  • IIF shows IgA deposits in the dermal papilla
23
Q

ALPS list 3 mutations

A
  1. CASP10, FAS, FASL
  2. GOF mutations - CTLA4, RAS, NRAS

characteristic abnormality: splenomegaly and lymphadenopathy, and AI

Defect in apoptosis of lymphocytes

24
Q

CTLA4 - list two normal mechanisms

A
  1. Binds to CD80 and CD86 (B7.1, and B7.2) and inhibits T cell proliferation and activation
  2. Expressed by Tregs to maintain tolerance
25
Q

HIV and Sulfa allergies

A
  • HIV patients have increased risk of developing cutaneous reactions compared to the general population bc of changes in their immune system such as increased IgE, immune system hyperactivation and changes in drug metabolism
  • mgmt: desensitization
    chemical structure is sulfa non antibiotics is different and do not CR, exception is sulfalazine which becomes sulfapyridine with degradation which is like sulfa
26
Q

3 mechanisms for FDEIa

A
  1. increased gut permeability
  2. increased absorption of partially digested allergens into circulation
  3. NSAIDs, illness or other cofactors may also contribute
27
Q

what is GPC?

A
  • GPC is presence of giant papillae on the upper tarsal conjunctiva
  • type 1 and 4 rxn
  • due to contact lens wear
28
Q

GVHD

A

3 Main concepts (Billinghams Criteria)

  1. Donor must have competent immune system
  2. Host has antigens that the donor does not, donor and host have a discrepancy (HLA discrepancy)
  3. Host is immunosuppressed
29
Q

list 4 mast cell mediators

A
  1. histamine
  2. tryptase
  3. PAF
  4. NO
30
Q

what feature of anaphylaxis is associated with PAF

A

severity of anaphylaxis

31
Q

what is the utility of anti-EM in Celiac disease? what about anti TTG?

A

anti EM is highly SPECIFIC
[has moderate sensitivity (85-98) and high specificity (97-100) for untx celiac disease]

anti TTG is highly SENSITIVE

32
Q

in IgA def what is the best ab test for celiac?

A

anti DGP (deaminated gliadin peptide)

33
Q

Name the rubber accelerators that account for contact dermatitis

A
  1. Benzothiazoles
  2. Carba chemicals
  3. Thiurams
34
Q

Name the chemical in vinyl gloves

A

Bisphenol A

35
Q

Filaggrin gene functions

A
  1. Chr 1q21
  2. involved in keratinocyte bundling (epithelial barrier)
  3. filagrin degradation products are involved in maintaining pH of the skin
36
Q

3 main mechanisms of AD

A
  1. defects of skin barrier
  2. immune dysregulation - defective innate immune response, th2 then th1 predominant, results in cytokine release
  3. enviromental/ infectious agents
37
Q

arguments for and against the role of allergy in AD

A
  1. For
    - involvement of certain cytokines IL4, IL3, IL5, which promotes eos survival and production
    - 70% of pts have a pos fam hx
  2. Against
    - many pts do not have pos SPT and high IgE
    - possible genetic basis
    - early AD begins in the absence of detectable IgE
38
Q

name some medium potency TC steroid options

A
  1. Clobetasone Butyrate 0.05%
  2. Betamethasone Valerate 0.05%
  3. Mometasone furoate 0.1% (Elocom)
39
Q

name some high potency TCS options

A
  1. Mometasone furoate 0.1% (Elocom)
  2. Clobetasol (0.05%)
  3. Betamethasone diproprionate (0.025%)
40
Q

Name 5 contact irritants of eyelids

A
  1. Nickel
  2. Nails - tosylamide, acrylides, formaldehyde
  3. Hair products - PPD (paraphenylenediamine), CAPB (cocoamidopropyl betaine)
  4. Make up - parabens, botanical ingredients
41
Q

Cell types in allergic contact dermatitis (6)

A
  1. Langerhans cells
  2. mast cells
  3. CD8s – major effector cell, source of INF-g
  4. CD4s
  5. Tregs
  6. NK cells - member of the innate immune system

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