2020 Flashcards
3 investigations that would help with HES diagnosis, and 2 mutations
Hypereosinophila
3 investigations
- Bone marrrow biopsy for specific stains such as PDGFRA-1 and PDGFRB2
- IgE level
- LSUB for CD3-CD4+ which is associated with L-HES subtypes
Mutation
- JAK2
- FGFR
- PDGFRA-1 and PDGFRB-2
Role of filaggrin mutation, how does it contribute to AD?
- 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
4 reasons for poor AD control
- poor compliance
- poor understanding of the treatment plan
- Underlying contributers to the diagnosis such as PID or nutritional deficiencies (zinc)
- inadequate dosing
- wrong diagnosis
Scoring systems in AD
- EASI - measures extent of body SA affected
- uses number score for redness, thickening, scratching, lichenification - SCORAD - uses rule of 9s to measure extent of eczema
- POEM - (pt oriented eczema measure)
- questionaries that measure days of the week involvement of eczema sx
Global suppression after measles, what lab changes do you expect
- T cell lymphopenia
- poor T cell response to mitogens
- ?decreases Abs
investigations in sarcoidosis
- HRCT
- BAL
- bronchoscopy and biopsy
biologic used in sarcoidosis
Inflixumab
stages of HP and imaging finding
- Acute
- CXR normal
- PFTs restrictive,
- HRCT shows mediastinal lymphadenopathy and ground glass opacities - Subacute
- HRCT: micronodular pattern on HRCT - Chronic
- fibrotic lung disease, w clubbing and hypoxia
- HRCT: honey combing, air trapping, and micronodules
histology of HP
- poorly formed non caseating granulomas or mononuclear infiltrates with giant cells
Target and MOA of Dupilumab
IgG4 binds to IL-4R, which inhibits IL4 and IL13
MOA - by binding to IL4 and IL13 it decreases the cytokines that mediate AD
CRSwNP - what are two cytokines involved and two tx other than INCS
IL4, IL5
mab treatment -
- Xolair (18+)
- Dupilumab (18+)
- Mepolizumab (18+ w CRwNP)
Indications for Nucala (Mepolizumab)
- add on maintenance tx for > 6 year olds with severe asthma and with an eosinophilic phenotype
- add on maintenance for of adult patients 18+ with CRSwNP
- treatment of adults with EGPA
- 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
occupational asthma from nail salon
- what is the allergen
- 3 mechanisms
- acrylamides
- immune mediated - IgE mediated
non-immune mediated - reactive airway dysfunction syndrome
irritant - irritant chemical, dust, fumes
OIT major antibody and mechanism of tolerance
Major antibody - IgG4
- shift from TH2 cytokine release to TH1
- increase in IgG4
- increase in IL-10 and TGF b
these actions lead to T cell anergy which contributes to OIT
what are the consequences of venom IT
decreased IgE
increased IgG4
increased IL10 and TGFB
when would you not skin test in VIT? different methods?
- Overlying skin infection
- Patient did not d/c appropriate medications (AH or INAH)
- Patient has previous anaphylaxis to skin testing
- Dermatographism
- SPT with 100 mcg/ml
- ID with 1 mcg/ml
- sIgE
persistent hypotension with Epi admin, pt on BB what drug do you give and dosing
Glucagon 1-5mg over 5 mins
4 adverse reactions to SCIT
4 CI to HDM SLIT
SCIT AE
- Anaphylaxis
- LLR
- systemic hives
- syncope?
- asthma exacerbation
4 CI to HDM SLIT
- serve uncx asthma
- uncontrolled cardio resp disease
- hypersens rxns to any ingredients in SLIT
- Pregnancy
- EoE
disadvantages and advant to SCIG over IVIG
- requires adherent patient
- requires equipment at home
- requires weekly injections instead of monthly
- requires some tricky equipment
advantages
- no IV access
- slower admin leads to consistent peak of IgG
- home access
- fewer sx SEs
Pemphigus vulgaris IIF findings
- IgG antibodies DSG1, and 3, desmoglein ab and IgG deposits
- affects the surface layer of the skin and epidermis
Bullous pemphigus IIF findings
- IIF shows IgG and complement deposits at the epidermal basement membrane
- blisters form in the dermoepidermal junction
dermatitis heptiformis IIF findings
- IIF shows IgA deposits in the dermal papilla
ALPS list 3 mutations
- CASP10, FAS, FASL
- GOF mutations - CTLA4, RAS, NRAS
characteristic abnormality: splenomegaly and lymphadenopathy, and AI
Defect in apoptosis of lymphocytes
CTLA4 - list two normal mechanisms
- Binds to CD80 and CD86 (B7.1, and B7.2) and inhibits T cell proliferation and activation
- Expressed by Tregs to maintain tolerance
HIV and Sulfa allergies
- 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
3 mechanisms for FDEIa
- increased gut permeability
- increased absorption of partially digested allergens into circulation
- NSAIDs, illness or other cofactors may also contribute
what is GPC?
- GPC is presence of giant papillae on the upper tarsal conjunctiva
- type 1 and 4 rxn
- due to contact lens wear
GVHD
3 Main concepts (Billinghams Criteria)
- Donor must have competent immune system
- Host has antigens that the donor does not, donor and host have a discrepancy (HLA discrepancy)
- Host is immunosuppressed
list 4 mast cell mediators
- histamine
- tryptase
- PAF
- NO
what feature of anaphylaxis is associated with PAF
severity of anaphylaxis
what is the utility of anti-EM in Celiac disease? what about anti TTG?
anti EM is highly SPECIFIC
[has moderate sensitivity (85-98) and high specificity (97-100) for untx celiac disease]
anti TTG is highly SENSITIVE
in IgA def what is the best ab test for celiac?
anti DGP (deaminated gliadin peptide)
Name the rubber accelerators that account for contact dermatitis
- Benzothiazoles
- Carba chemicals
- Thiurams
Name the chemical in vinyl gloves
Bisphenol A
Filaggrin gene functions
- Chr 1q21
- involved in keratinocyte bundling (epithelial barrier)
- filagrin degradation products are involved in maintaining pH of the skin
3 main mechanisms of AD
- defects of skin barrier
- immune dysregulation - defective innate immune response, th2 then th1 predominant, results in cytokine release
- enviromental/ infectious agents
arguments for and against the role of allergy in AD
- For
- involvement of certain cytokines IL4, IL3, IL5, which promotes eos survival and production
- 70% of pts have a pos fam hx - Against
- many pts do not have pos SPT and high IgE
- possible genetic basis
- early AD begins in the absence of detectable IgE
name some medium potency TC steroid options
- Clobetasone Butyrate 0.05%
- Betamethasone Valerate 0.05%
- Mometasone furoate 0.1% (Elocom)
name some high potency TCS options
- Mometasone furoate 0.1% (Elocom)
- Clobetasol (0.05%)
- Betamethasone diproprionate (0.025%)
Name 5 contact irritants of eyelids
- Nickel
- Nails - tosylamide, acrylides, formaldehyde
- Hair products - PPD (paraphenylenediamine), CAPB (cocoamidopropyl betaine)
- Make up - parabens, botanical ingredients
Cell types in allergic contact dermatitis (6)
- Langerhans cells
- mast cells
- CD8s – major effector cell, source of INF-g
- CD4s
- Tregs
- NK cells - member of the innate immune system
