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A/I Board Review > Clinical Diseases > Flashcards

Clinical Diseases Flashcards

1
Q

Which component in immunoglobulin products is thought to be responsible for thromboembolic events?

A

Factor XIa

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2
Q

A mismatched donor transplant has higher risk of what?

A

GVHD and rejection

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3
Q

A patient has received multiple blood transfusions in the past. Which type of transplant rejection is more likely?

  1. Acute rejection
  2. Hyperacute rejection
  3. Chronic rejection
A

Hyperacute rejection

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4
Q

What immune mechanisms underlie:

  1. Acute rejection
  2. Hyperacute rejection
  3. Chronic rejection
A
  1. Type IV HSR first (host CD8 T cells attack), followed by type II and III HSR, ADCC
  2. Type II HSR, from Ab’s formed by previous transfusions/pregnancies/transplants
  3. Chronic Type IV reactions (build up of damage from recurrent acute rejections)
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5
Q

What does Filaggrin do?

A

Pro-filaggrin turned into filaggrin, which is then broken down into natural moisturizing factors (NMFs) that maintain hydration in stratum corneum, UV protection, acidification, and anti-staph efffects

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6
Q

Mutations in filaggrin lead to what?

A

LOF mutations:

  • increased atopy
  • increased risk for eczema herpeticum
  • increased pH
  • increased IL-1beta
  • decreased natural moisturizing factors (NMFs)
  • palmar hyperlinearity
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7
Q

What is the triad of clinical findings in Netherton Syndrome (SPINK5 mutation)?

A
  1. Congenital ichthyosis
  2. Trichorrhexis invaginatum (bamboo hair)
  3. Atopic diathesis (AR, AD, asthma, food allergy, urticaria/angioedema)

AR mutation in SPINK5 which encodes for serine protease inhibitor LEKT1

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8
Q

What is Schnitzler Syndrome?

A
  • IgM monoclonal paraproteinemia
  • nonpruritic urticaria
  • intermittent fever
  • arthralgia, bone pain
  • leukocytosis and elevated ESR
  • Neutrophils on dermal biopsy
  • Anti-IL1 therapy is affective (Anakinra)
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9
Q

Which cold urticaria syndromes have a negative ice cube test?

A

PLAID - phospholipase Cgamma2-associated antibody deficiency and immune dysregulation

FCAS - Familial Cold Autoinflammatory Syndrome

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10
Q

Which complement study differentiates Acquired Angioedema from Hereditary Angioedemas?

A

C1q - low in AAE but normal in HAE

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11
Q

What are risk factors for death from an anaphylactic reaction?

A

Delayed epinephrine
young adult/teen
underlying asthma
absence of skin symptoms

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12
Q

What are risk factors for anaphylaxis?

A

Atopy (for idiopathic anaphylaxis, exercise-induced anaphylaxis, radiocontrast and latex-induced reactions)
*Atopy is not a RF for medication anaphylaxis or venom anaphylaxis

Gender - Males up to age 15y/o, then Females >15y/o

Age - children and adolescents

Route of administration - IV/IM > oral

Intermittent administration

Prolonged administration

Geography - north > south

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13
Q

What tryptase ratio is seen in mastocytosis vs systemic anaphylaxis?

A 
Tryptase ratio (total alpha+beta)/(mature beta-only tryptase)
Ratio >20 in mastocytosis (mature tryptase stays low)
Ratio <10 in anaphylaxis (both total and mature increase)
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14
Q

What compound contaminated worldwide heparin supplies in 2007-2008, causing anaphylaxis by directly activating the kinin-kallikrein pathway leading to generation of bradykinin, C3a, and C5a?

A

Oversulfated chondroitin sulfate (OSCS)

These reactions consistently lacked urticaria or pruritus!

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15
Q

What drug classes increase risk for more severe anaphylaxis?

A

Beta blockers and ACEI’s

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16
Q

What drugs increase side-effects from epinephrine administration?

A

TCA’s and MAOI’s

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17
Q

What type of cells would you see in a biopsy of a salivary gland from a Sjogren’s syndrome patient?

A

CD4 lymphocyte infiltrate

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18
Q

What are the standardized allergen extracts?

A

DF, DP
short ragweed
Cat hair and pelt
Grasses (Bermuda, Northern grasses)

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19
Q

What parameters go up in allergen immunotherapy?

A

Allergen specific IgE (initally, then eventual decrease)
early, IgG1, then later IgG4
Th1 cytokines (IL-12, IFN-gamma); Th1/Th2 ratio
IFN-gamma/IL4 ratio
IL-10 and TGF-beta (from Tregs)
Treg expression of FoxP3
IgA in respiratory secretions

*Decreases seen in low affinity IgE receptor (FcεRII or CD23)

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20
Q

What are the effective doses for standardized allergen extracts?

A 
DF/DP = 500-2000 AU
Short ragweed = 1000-4000 AU or 6-12mcg of Amb a1
Cat = 1000-4000 BAU
Northern Grasses = 1000-4000 BAU
Bermuda = 300-1500 BAU
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21
Q

What are the effective doses for nonstandardized US allergen extracts?

A

Dog = 15mcg of Can f1
Pollens = 0.5ml of 1:100; or 1:200 wt/vol
Mold and CR = highest tolerated dose

22
Q

What is the target antigen in vasculitis for cytoplasmic staining antineutrophil cytoplasmic antibodies (cANCA)? How about perinuclear ANCA (pANCA)?

A

cANCA: proteinase-3 (PR-3)
pANCA: myeloperoxidase (MPO)

23
Q

What mediators is increased/decreased in AERD?

A

Increased LTE4 (urine, BAL)
Increased LTC4 synthase expression
Increased cysLTR1 and cysLTR2 receptor expression
Decreased lipoxin
Decreased EP2 receptors (receptor for PGE2 which inhibits 5-LO)

24
Q

Increased levels of which 2 mediators correlate with severe reactions during aspirin desensitizations?

A

urinary LTE4 and PGD2

25
Q

What factors increase FeNO? What factors decrease FeNO?

A

Increase FeNO:

  • allergic rhinitis
  • URI
  • eating foods containing nitrates (cold cut meat, spinach, green beans, carrots, beets)

Decrease FeNO:

  • exercise
  • smoking
  • alcohol
  • spirometric maneuvers
  • CF
  • pulmonary HTN
26
Q 
Which of the following will increase serum theophylline levels?
A. Carbamazepine
B. Phenytoin
C. Rifampin
D. Zileuton
A

D. Zileuton. The other 3 drugs will decrease theophylline levels.

27
Q

Desmoglein antibodies to DSG1 cause what type of lesions in pemphigus vulgaris? (mucosal or skin?)
How about antibodies to DSG3?

A 
DSG1 = skin (pemphigus foliaceous)
DSG3 = mucous membranes
28
Q

In AERD, what order does the “triad” of symptoms appear?

A
  1. Chronic Rhinosinusitis with polyps
  2. Asthma
  3. aspirin/NSAID hypersensitivity
29
Q

Hypersensitivity Pneumonitis–associate the causal agent with the following context/occupations:

  1. Farmer’s Lung
  2. Detergent worker’s lung
  3. Hot tub lung
  4. Humidifier lung
  5. Bird Breeder
  6. Rodent handlers
  7. Paint refinishers
  8. Chemical plants
  9. Wood workers
  10. Cork dust
  11. Cedar worker
  12. Machine operator’s lung
A
  1. Thermophilic actinomycetes/Mycopolyspora faeni
  2. Bacillus subtilis
  3. M. avium-intracellulare
  4. T. vulgaris, Candida, M. faeni
  5. Avian proteins
  6. Urine proteins (serum proteins)
  7. Isocyanates (TDI)
  8. Anhydrides (Trimellitic), plastics, epoxy
  9. Alternaria sp.
  10. Suberosis
  11. Thuja plicata (plicatic acid)
  12. Contaminated fluids (Pseudomonas fluorescens/fungi)
30
Q

What is the CD4:CD8 ratio in acute hypersensitivity pneumonitis compared to sarcoidosis?

A

CD4:CD8 ratio is LOW (increased CD8) in HP and COPD, whereas CD4:CD8 ratio is HIGH in sarcoidosis, tuberculosis, or berylliosis

31
Q

What is the PC20? What levels are considered unlikely to have asthma, and what levels are considered highly predictive of asthma?

A

PC20 = “provocative concentration” - interpolated dose of inhaled agonist that causes FEV1 to fall exactly 20%
PC20 > 16mg/ml – unlikely to have asthma
PC20 <= 4mg/ml is highly predictive of asthma

If you get an abnormal peak flow rate (PEFR), it is validated by a PC20 <= 16mg/ml

32
Q

What is a noninfectious, febrile lung illness that occurs after exposure to dust contaminated by toxin-producing fungi (in grain, hay, textiles) and is 30-50 times more common than hypersensitivity pneumonitis, usually in young patients, shows lack of serologic response to common fungal antigens, and no prior sensitization is required?

A

Organic Dust Toxic Syndrome (ODTS) - caused by build up of endotoxins in waste product. Also known as pulmonary mycotoxicosis or swineherder’s disease.

33
Q

What are the most likely agents involved in occupational asthma for the following occupations?

  1. Polyurethane/plastics/moldings factory worker, spray painters, insulation installers
  2. Hairdressers
  3. Adhesive manufacturer, nail stylist/worker, printing inks, dental/orthopedic materials
  4. Sawmill workers, carpenters, furniture makers
  5. healthcare workers, cleaners
A
  1. isocyanates (TDI, MDI, HDI)
  2. persulfate salts, hair bleach
  3. Acrylates (cyanoacrylates, methacrylates, ditriacrylates)
  4. Wood dusts (red cedar, oak)
  5. Biocides (aldehydes, quaternary ammoniums)

*these are all low molecular weight agents and typically IgE-independent pathophysiology

34
Q

Between asthma and COPD, which disease is known for marked eosinophilia, IL-5 expression, and CD4 T cell predominance?

A

Asthma.

COPD is known for mild eosinophilia, no increase in IL-5, and CD8 predominance

35
Q

What are the serum autoantibodies and tissue immunofluorescence patterns of the following skin diseases:

  1. Pemphigus vulgaris
  2. Bullous pemphigoid
  3. Dermatitis herpetiformis
A
  1. IgG autoantibodies to desmoglein 1 and 3; Deposits of IgG and C3 in the intracellular spaces of epidermis (cells surface staining)
  2. IgG autoantibodies to BP180 and BP230; Linear basement membrane zone IgG and C3
  3. IgA autoantibody to epidermal transglutaminase; Granular basement membrane zone IgA wtih stippling in dermal papillae
36
Q

What allergic eye condition causes bilateral chronic inflammation in young atopic males residing in warm and dry climates, presenting with severe photophobia, intense ocular itching, papillary hypertrophy (>1mm), thick and ropy discharge, and peri-limbal white dots on exam?

A

Vernal Keratoconjunctivitis (VKC)

Horner-Trantas Dots - focal collections of degenerated eosinophils and epithelial cells that are peri-limbal. Also can find these dots in AKC

37
Q

What allergic eye condition causes bilateral chronic inflammation in patients with eczema, worsens in parallel with worsening eczema, presents with intense ocular itching, and can lead to keratoconus and anterior subcapsular cataracts?

A

Atopic Keratoconjunctivitis (AKC)

38
Q

Roughly 2/3rds of children with frequent wheezing and positive asthma predictive index (API) will have asthma during school years. A positive API in children younger than 4 years can be initiated on controller therapy.

What are the major and minor criteria for the API (modified)?

A

≥ 4 episodes of wheezing over past 1 year AND

Any ONE of the MAJOR CRITERIA:

  • Parental Asthma
  • Physician diagnosis of Atopic Dermatitis
  • Sensitization to aeroallergens

OR

Any TWO of the MINOR CRITERIA:

  • sensitization to milk, egg, or peanut
  • eosinophils ≥4%
  • Wheezing unrelated to colds
39
Q

What fruits are associated with Latex-Fruit Syndrome?

A

Banana, Avocado, Kiwi, Chestnut

40
Q

What diagnostic tests are used for the following physical urticarias:

  1. cold urticaria
  2. dermatographism
  3. local heat urticaria
  4. cholinergic urticaria
  5. delayed pressure urticaria
  6. solar urticaria
  7. aquagenic urticaria
  8. vibratory urticaria
A
  1. cold urticaria - ice cube test on skin for 5 min
  2. dermatographism - stroke skin with blunt, smooth object
  3. local heat urticaria - test tube water 45degC
  4. cholinergic - a) exercise 15-20min OR b) immerse in 42degC bath until core temp >0.7degC
  5. delayed pressure - sand bag test: 15lb weight 15 min
  6. solar - specific wavelength exposure
  7. aquagenic - water compress 35degC
  8. vibratory - vortex for 4min
41
Q

What are general diagnostic criteria for Allergic Bronchopulmonary Aspergillosis (ABPA)?

A
  1. Asthma or CF
  2. High IgE (>1000 IU/mL)
  3. Eos >500 in a steroid naive pt
  4. +SPT and serum IgE to Aspergillus fumigatus
  5. Central bronchiectasis
  6. Thick/brown mucus/sputum plugs, eosinophilic debris, hyphae
42
Q

What are the GOLD criteria for staging COPD?

A

GOLD 1 - mild: FEV1≥ 80% predicted
GOLD 2 - moderate: 50% ≤FEV1 <80% predicted
GOLD 3 - severe: 30% ≤FEV1 <50% predicted
GOLD 4 - very severe: FEV1 <30% predicted.

43
Q

Which immune cells play a major role in COPD?

A

neutrophils, macrophages, CD8 T cells.

Activated neutrophils and macrophages release proteases that cause parenchymal lung destruction underlying emphysema. Nicotine can enhance destruction by inhibiting tissue inhibitors of matrix metalloproteinases (TIMPs).

44
Q

Penicillin G is the treatment of choice for syphilis. For penicillin-allergic patients diagnosed with syphilis, what 3 situations is penicillin desensitization indicated?

A
  1. neurosyphilis
  2. congenital syphilis
  3. syphilis in a pregnant woman
45
Q

What is the most common form of vasculitis?

A 
Giant Cell Arteritis
>50y/o, F>M 2:1
-fever, fatigue
-Headache over vessel
-scalp tenderness
-Jaw/tongue claudication
-Polymyalgia rheumatica
-Optic nerve ischemia leading to blindness
Dx: temporal artery biopsy
Tx: pred 40-60mg daily while bx arranged
*tocilizumab (anti-IL6R) - NOT a replacement for prednisone, but could reduce need for steroids
46
Q

What small-to-medium vessel vasculitic disease presents with the classic triad of sinus, lung, and kidney involvement?

A

granulomatosis with polyangiitis (Wegener’s granulomatosis)

  • Sinuses: congestion, bleeding, pain, not responsive to AH/abx
  • Lungs: pulmonary nodules infiltrates, or singular cavitary lesions, or ground glass
  • Kidney: detected by U/A (proteinuria, hematuria, RBC casts)
  • cANCA (anti-PR3) found in 75-90% of patients
  • few to no immune complexes in kidney bx (pauci-immune glomerulonephritis, unlike Lupus)
47
Q

Which vasculitis presents with palpable purpura, arthritis, glomerulonephritis, intussusception?

A

IgA vasculitis (Henoch-Schonlein Purpura)

  • small vessel
  • Biopsy of skin: IgA deposits; Kidney: +IgA often IgG, C3
  • typically self-limited course
48
Q
  1. What trees cross-react with ash?
  2. What trees cross-react with cottonwood?
  3. What trees cross react with cedar?
  4. What trees cross react with birch?
  5. What trees cross react with pecan?
  6. What trees cross react with maple?
A
  1. ash, olive, privet (Oleaceae family)
  2. cottonwood, poplar, aspen (Populus family)
    * also cross-reacts with willow (Salicaceae)
  3. cedar, juniper, cypress (Cupressaceae family)
  4. birch, alder, hazel, hornbeam (Betulaceae family)
    * also cross-reacts with oak, beech, chestnut (Fagaceae)
  5. pecan, hickory, walnut (Juglandaceae)
  6. maple, box elder (Aceraceae)
49
Q
  1. What other weeds does mugwort cross-react with?
  2. What other weeds does kochia cross-react with?
  3. What weeds does ragweed cross-react with?
  4. Which grasses cross-react?
A
  1. mugwort, sage, and wormwood (Artemisia spp)
  2. kochia, russian thistle, lambs quarter, burning bush
  3. short/giant/false/western ragweeds (Ambrosia)
  4. Northern grasses cross-react with each other: timothy, orchard, KY bluegrass, meadow fescue, perennial rye, sweet vernal, red top (Festucoideae)
    - Bahia and Johnson cross react (Panicoideae)
    - Bermuda is by itself (Chloridoideae)
50
Q

Which allergen extracts should be separated from pollen extracts?

A

mold and cockroach (contain proteolytic enzymes that degrade pollen.
dust mite is OK with pollen in 10% glycerin or greater.
Ragweed and cat are OK with mold and cockroach if glycerin is 50%.

51
Q

What are the differences in cell types and cytokines between acute atopic dermatitis lesions vs chronic dermatitis lesions?

A

Acute AD lesions have more IL-4 expressing Th2 cells
-characterized by IL-4, 5, 13, 31

Chronic AD lesions have more IL-5 expressing Th2 cells and activated eosinophils
-characterized by IL-4, IL-5, IL-12, and IFNgamma

T cells in AD lesions express cutaneous lymphocyte-associated antigen (CLA+) - ligand for E-selectin

A/I Board Review (8 decks)

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