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Board Review
Taylor Doherty M.D.
Contributors Drew White, M.D.
and Mark Tuker, M.D.
Pearls
• Pregnancy B inhaled steroid: budesonide
• Asthma with mononeuritis multiplex: CSS
• ABPA: central bronchiectasis, high IgE
• Asthma symptoms with insp loop plateau:VCD
• Nasal polyps + Asthma: Aspirin sensitivity
• Allergic rhinitis: 1st line=nasal steroid
• Eczema in adult: biopsy to r/o cutaneous lymphoma
Pearls
• Chronic urticaria: assoc with thyroid abs and IgE receptor
antibodies (FcER1)
• Hereditary Angioedema: no hives, low C1 esterase inhibitor,
low C2/C4, normal C1Q
• Aquired Angioedema: above but low C1Q
• Anaphylaxis after eating fish: consider scromboid (high
histidine fish) vs IgE mediated
• Urticarial vasculitis:lesions last >24hr , leave a
hyperpigmented spot, and can be painful
Pearls
• Insect anaphylaxis: refer for testing and IT
• Anapylaxis: serum tryptase within hours
• PCN cephalospoin xreactivity: 2-5%
• Abacavir hypersensitivity:HLA-B5701
• Redman syndrome with Vancomycin-slow infusion rate if not
severe
• Vaccines with Egg: Influenza (both), yellow fever. MMR
negligible
Pearls
• Adult onset recurrent/chronic sinusitis, bronchiectasis, giardia,
autoimmunity, lymphomas- Common Variable (CVID), check
QIGS/specific titers
• IgA deficient: can anaphylaxis to IgA containing products
• Recurrent/sever Neisserial infections- complement deficiency (check
CH50) usually late pathway.
• Dysphagia, male, allergic: biopsy for EE
• Hypereosinophilia: >1500 AEC, check strongyloides (O+P and ELISA)
Gell and Coombs Classification
• I – IgE on Mast cells – anaphylaxis
• II – antigens on cell surface lead to cell
destruction (IgG) – PCN binds to RBC, IgG binds
to PCN/RBC and RBC destroyed
• III – soluble antigen Ag:Ab aggregates
(immune complexes) deposit in tissues (serum
sickness)
• IV – T cell mediated, delayed hypersensitivity.
Direct T cell toxicity (PPD, Poison Ivy)
Two ways to fire a Mast Cell
• Cross link specific IgE on the surface
QuickTimeª and a
decompressor
are needed to see this picture.
AERD: Aspirin exacerbated respiratory
disease
• Asthma (moderate-severe), nasal polyposis, ASA and
NSAID (naso-ocular and bronchial) reactions (can be
severe and delayed)
• Usually adult onset, female>male, many have atopy as
well
• NSAIDS (Cox-1) and ASA reactions are only a marker of
a progressive disease
• Refer to allergy
Differential Dx for adult asthma
• Vocal cord dysfunction (VCD)
• COPD (overlap?)
• Parenchymal lung disease
• LV failure
• Panic Attacks
• Don’t forget that asthma is part of other diseases
(CSS and ABPA)
• Be aware that asthma variants may have expanded
differentials (cough, chest tightness predominant)
Vocal Cord Dysfunction
• Female 2:1
• Athletes
• Bronchodilator non
responsive
• Many (up to 1/3) can
have coexist asthma
• Difficulty getting
breath in
Asthma Pharmacotherapy
Rescue medicine
SABA (short acting β agonists)
Anticholinergics
Controller medicine
ICS (inhaled corticosteroids)
LABA (long acting β agonists)
LTRA (leukotriene receptor antagonists)
Anti-IgE
Others
Risk Factors for Asthma Death
• Previous life-threatening asthma, respiratory arrest
• Psychosocial disturbance
Pregnancy and Asthma
• 1/3 worsen, 1/3 improve, 1/3 no change
• Helminth infections
– Ascaris
– Hookworms
– Strongyloides
ID-Eosinophilic lung dz
• Coccidiodal infection – up to 25% with
eosinophilia
• Lung fluke/echinococosis/cysticercosis
Meds/Tox –Eosinophilic lung dz
• Meds – NSAIDS, minocycline,
nitrofurantoin, ampicillin
• Treatment
– Interferon α
– Gleevac (imatinib) - for FIP1L1-PDGFRα gene
rearrangement
– Steroids
Food Allergy
• Oral Allergy Syndrome
– Pollen cross reactivity
– Ragweed – melons, banana
– Birch – apple, peach, plum, cherry, almond,
potato, carrot
– Mugwort – celery
• Oral itching only, no anaphylaxis
Food Allergy
• Peanut, tree nut, fish, shellfish most
common
• Skin test or RAST test for diagnosis
• Treatment – avoidance
– No role for immunotherapy
Drug Allergy
• Beta – lactam
– Cross reactivity to cephalosporins - ~2-5%
– More common with first generation cephalosporins
– Carbapenams highly cross reactive with PCNs
– Monobactams (aztreonam) – not cross reactive with
PCNs
– Aztreonam and ceftazidime share side chain and may
cross react
Vancomycin
• “red man syndrome” – pruritis and
erythema of face, neck, upper torso,
occasionally with hypotension
• Non-immunologic release of histamine
• Not IgE mediated
• Tx: slow the infusion and pre-treat
Sulfa
• Most common rxn – maculopapular eruption with
fever beginning 7-12 days after treatment in HIV.
• Fever and urticaria may be present
• Anaphylaxis is rare
– IgE mediated reactions to sulfa antibiotic are not cross
reactive with non-antibiotic sulfa drugs.
• Can usually still desensitize
Drug desensitization
• Universally successful for IgE mediated
reactions
• Nearly always successful for non-IgE
reactions
• Contraindicated in Stevens Johnson
Syndrome, TEN, and serum sickness
reactions
Local anesthetics
• In general IgE hypersensitivity does not
occur.
• Reactions are either Toxic or vasovagal
Anticonvulsant Hypersensitivity
• Deficiency of epoxide hydrolase
• Fever, maculopapular rash, generalized
lymphadenopathy
• Resembles serum-sickness reaction
• LN bx resembles Hodgkin’s
• Phenytoin, carbamazepine, phenobarb
Radiocontrast
• Anaphylactoid
– Non-IgE mediated mast cell histamine degranulation
• Angioedema 0.1-0.2%
• Does not always resolve with switch to
ARB
EM, SJS, TEN
• EM – target lesions, usually caused by
HSV, mild self limited. Oral lesions
• SJS – diffuse, severe mucocutaneous
eruption (<10% of skin detached)
– Usually drug induced
– Fever/malaise
– 2 mucosal surfaces involved
TEN
• >30% BSA has epidermal detachment
• >90% linked to causative drug
• Treatment for SJS, TEN – supportive, burn
unit. (lots or meds studied, none proven)
• Pathophysiology – CD8+ mediated
keratinocyte destruction through fas ligand
Drugs and SJS/TEN
(usually predates symptoms by 2-3 weeks)
• Sulfonamides allopurinol
• Carbamazepine penicillins
• Phenytoin NSAIDs
• Phenobarb Quinolones
• Chlormezanone cephalosporins
• Acetaminophen valproic acid
Serum Sickness
• Pruritic (sometimes more burning) hive-like rash. May last
longer than hives.
• Fever
• Rheumatic symptoms (MCP, knee, wrist, ankles)
• Some swelling can occur of extremities or around joints
• Neutropenia with reactive lymphocytes
• +/- thrombocytopenia and eosinophilia
• High ESR/CRP
Serum sickness
• Ceclor (kids), penicillins, sulfa
• Any animal protein (antisera, monoclonal
abs)
• Very long list of uncommon drugs that
cause serum sickness
• Treatment – remove antigen, antihistamines
+/- steroids
• Avoid culprit in future
Immunodeficiency
• T- cell (cellular) – virus, fungi, protozoa,
mycobacteria
• Humoral (antibody) – extracellular
pyogenic organisms
– Haemophilus
– Pneumococcus
– Streptococcus
Immunodeficiency
• Increased infections
• Recurrent respiratory infections
• Multiple systems involved
• Unusual organisms
• Malabsorption
• Big LNs or absent LNs
Common Variable
Immunodeficiency
• Various immune and genetic defects
• Lymph tissue present or enlarged
• High incidence of autoimmune disease (22%)
• Increased risk of adenocarcinoma and lymphomas
• In addition to encapsulated organisms
– Giardia, yersinia, H. pylori, and H. jejuni are common
IgA deficiency
• Most common of primary
immunodeficiency (1:333)
• Need IgA <5mg/dL (VERY LOW)
• Most patients with IgA def. are NORMAL
• Have increased risk of infections, collagen
vascular dz, allergic dz, GI dz, and
malignancy
IgA deficiency
• Can make anti-IgA antibodies
– Leads to anaphylaxis with IgA containing
blood products
Specific Antibody Deficiency
• Normal QIGs, but do not appropriately
respond to infection or vaccination with
appropriate rise in antibody titer.
• To diagnose, check pre-post vaccination
titers.
• i.e. check strep ab level, vaccinate with
pneumovax, recheck ab level in 4-6 weeks.
Should increase >4x.
IVIG
• Indicated for CVID and specific antibody
deficiency
• NOT indicated for IgA deficiency
• Best ONE SCREENING TEST FOR
HUMORAL IMMUNODEFICIENCY
• IgA
IVIG
• Side effects
– Never been transmission of HIV
– Fever, chills, HA, muscle pain.
– Aseptic meningitis
– Renal failure (was due to osmotic load, not as
common now), Stroke, MI
Complement Deficiency
• C2 deficiency (most common) – sepsis,
pneumonia, meningitis, pyogenic arthritis with
Strep pneumo
• C2 deficiency – increased risk of rheumatoid
arthritis
• Terminal Complement Components – Neisseria sp
infections
– Think about if recurrent meningitis or if unusual strand
Nutritional Immunodeficiency
• Severe protein calorie malnutrition –
profound B and T cell defects
• Autosomal recessive inability to absorb zinc
– acrodermatitis enteropathica
• Eczema around mouth and rectum
• Chronic diarrhea, malabsorption
• Recurrent sinopulmonary infections
Vaccine
• Live Viral Vaccines –
– Measles
– Mumps
– Oral Polio
– Rubella
– Varicella
– MMR
– Smallpox (vaccinia)
– Flumist
Egg allergy and Vaccines
• Influenza and Yellow fever
• A 56 year old man comes to your office for
evaluation of scrotal swelling and abdominal
cramping of recent onset. He has no other pain or
pruritus. Two months ago, he noted swelling of the
right hand. The rest of the findings are unremarkable,
except for a recent onset of night sweats.
• Which of the following laboratory findings is most
likely?
• A. Low serum levels of C3 and C4
• B. Low serum levels of C1q
• C. Elevated serum tryptase level
• D. Normal serum levels of C1q and decreased serum
levels of C2 and C4.
• A 22 year old woman comes to your office
with hives. For the past 4 months, she has
had recurrent pruritic lesions that occur on
various areas of her body and last for
several hours. Extensive review does not
indicate any physical or other specific
triggers. Review of systems is
unremarkable. Examination shows several
raised erythematous lesions on the trunk.
Which of the following
statements about this patient is
true?
• A. She has an autosomal dominant disease
• B. The skin lesions are probably associated with
exposure to allergens
• C. The serum complement level will be low.
• D. She may have histamine-releasing
autoantibodies that bind the alpha chain of the high
affinity IgE antibody receptor on mast cells
• A 32 year old woman who lives in a
suburban neighborhood and recently was
admitted to the ICU for asthma tells you
that her asthma is under control, although
you can hear wheezing from across the
room. She states that she takes her
medications exactly as prescribed, without
missing a dose. You notice a pack of
cigarettes in her open purse.
Which of the following factors
most significantly increases this
patient’s risk of asthma death?
• A. Exposure to tobacco smoke during childhood
• B. Recent admission to the ICU
• C. Female gender
• D. Non-compliance with her medication regimen
• E. Wheezing that is audible form across the
room
B. Recent Admission to ICU
• Recent admission to hospital, ED or
particularly to ICU
• 2 or more short acting beta agonist
canisters/month
• Poor perception of asthma symptoms
• Psychosocial disturbance
Which of the following is/are
NOT part of the innate immune
system
• A. C5
• B. Neutrophil
• C. IgG
• D. Macrophage
Which of the following vaccines
would not be advised in
pregnancy?
• A. Tetanus
• B. Influenza (injection)
• C. Varicella
• D. Hepatitis B
• E. Pneumovax
Varicella
• Live virus which is thus contraindicated in
pregnancy due to the increased risk of
congenital varicella syndrome
A 35 yo male with a history of mild
persistent asthma, presents with the
following pruritic rash. The most
appropriate next step would be?
• A. Topical hydrocortisone 2.5% ointment
• B. Oral Antihistamine
• C. Topical pimecrolimus cream
• D. Skin Biopsy
• E. Solumedrol oral taper.
A 19 yo college student survives
meningitis which was culture
positive for N. meningitidis
• Which test is the most appropriate?
• A. C5 level
• B. Quantitative immunoglobulins
• C. IgA level
• D. CH50
• E. N. meningitidis antibody titer
A 28 yo female presents after her 3rd
X-ray proven pneumonia. An IgA
level was drawn and was 60 mg/dL
(80-350)
• What is the next best course of action?
• A. Start IVIG therapy at 0.5 gm/kg q month
• B. Place an anergy panel to assess T cell
function
• C. Get Chest CT to eval for bronchiectasis
• D. Check CH50
• E. Check anti-pneumococcal IgG titers,
administer pneumovax and recheck in 4
weeks
• F. Place on antibiotic prophylaxis
• During a urologic surgical procedure, a 62
year old man has sudden hypotension and
cardiac dysrhythmia. The anesthesiologist
believes that she saw some raised wheals on
the patient’s trunk, but is unsure whether he
was having an anaphylactic reaction or an
acute cardiac event. Which of the
following would be most helpful in
identifying intraoperative anaphylaxis?
• A. Measurement of the serum tryptase
level within 4 hours of the incident.
• B. A 24- hour urine collection to detect
leukotriene C4 beginning immediately after
the procedure.
• C. An in vitro serologic test to detect
specific IgE to natural rubber latex.
• D. Measurement of plasma levels to detect
histamine and its metabolites within 4 hours
of the incident.
• A 21 year old woman with persistent
asthma and allergic rhinitis is in her 6th week
of pregnancy. She is using an albuterol
inhaler and budesonide inhaler for her
asthma. She uses Claritin-D for her rhinitis.
• What do you recommend?
• A. Stop budesonide and use albuterol as
needed for wheezing. Switch to
pseudophed for nasal symptoms.
• B. Continue budesonide and albuterol, use
plan pseudophe for nasal symptoms
• C. Continue budesonide and albuterol,
avoid Claritin-D during the first trimester.
• D. Stop all drugs and initiate
immunotherapy
• E. Stop budesonide and use albuterol and
benadryl prn.
Which of the following would be the
medicine most likely to be effective
in treating allergic rhinitis from dust
mites?
• A. Cetirizine
• B. Montelukast
• C. Diphenydramine
• D. Fluticasone nasal spray
• E. Ipratroprium 0.06% nasal spray