00 Immunology And Allergy Usmle Lange Outline

Front 1

ANAPHYLAXIS
differential diagnosis

Back 1

ANAPHYLAXIS
1. Type I Immediate
hypersensitivity reaction:
a. Food or food-
additive allergy.
b. Hymenoptera (bee
sting) allergy.
c. Drug allergy.
2. Disorders associated
with sudden collapse
but without urticaria and
angioedema:
a. Arrhythmia.
b. Myocardial infarction.
c. Food aspiration.
d. Pulmonary
embolism.
e. Seizure disorders.
f. Vasovagal collapse.
3. Miscellaneous:
a. Hereditary
angioedema.
b. Serum sickness.
c. Cold urticaria.
d. Idiopathic urticaria.

Front 2

Epidemiology
Includes a wide range of reactions (minimal to anaphylactic) to any
food, medication, or foreign substance. No increased incidence by
gender or geographic location. Shellfish, nuts, and milk products
are common offenders (drugs include antibiotics, vaccines, hor-
mones, dyes, and allergic extracts; foreign substances include venom
from Hymenoptera insects).

Back 2

General information:
• Type I immediate hypersensitivity reaction: anaphylaxis.
• Type II cytotoxic reaction: drug and transfusion reactions.
• Type III immune complex–mediated reaction: serum sickness,
glomerulonephritis.
• Type IV cell-mediated delayed hypersensitivity reaction: tuberculin
skin test reactions, contact dermatitis

Hint 2

Prevention
Includes allergy testing, allergen avoidance, and desensitization to
inhalant allergens, drugs, and hymenoptera venom. Accurate history
taking and skin testing prior to medication use are important. Test-
ing includes wheal-and-flare prick (epicutaneous), intradermal skin
testing and radioallergosorbent test (RAST) serum testing. Patients
with known allergies are advised to wear Medic Alert bracelets. Med-
ical facility protocols to avoid drug allergy reactions, transfusion re-
actions, and so on. Use of preventive medication when risk of aller-
gic reaction is significant (prednisone, benadryl, and an H2 blocker prior to contrast dye injection)

Front 3

Allergic (Eosinophilic) Gastroenteropathy

Back 3

Rare disorder of
immunoglobulin E (IgE) production and rapid-onset food allergy
(nausea/vomiting, etc.)

Front 4

Anaphylactic Reactions

Back 4

IgE antibody mediated (type 1), wide-
spread organ disorder

Front 5

Contact Dermatitis

Back 5

Mediated via T-cell hypersensitivity.

Front 6

Drug Reaction

Back 6

—Mediated via IgE (penicillin anaphylaxis), T cell
(contact dermatitis), immune complex (serum sickness), or cytotoxic-antibody systems (nephritis and Coombs’-positive hemolytic anemia).

Front 7

A presumptive diagnosis of can be made if certain opportunistic infections are present such as candidiasis of the esophagus, tra-
chea, bronchi, or lungs or toxoplasmosis of the brain is present, for
example. Definitive diagnosis incorporates finding antibody to HIV.
Viral load is then usually checked.

Back 7

Human Immunodeficiency Virus (HIV)
Infections and Acquired Immune Deficiency
Syndrome (AIDS)
Epidemiology
Ribonucleic acid (RNA) retrovirus HIV-induced pandemic (possible
15–20% population infected in Africa, and cases throughout the world), with increased frequency in Blacks/Hispanics (compared to
population figures), and pockets of increased frequency among
drug users noted (New York). Adult risk of infection by needle shar-
ing, broken skin/mucosal exposure, unprotected vaginal/anal inter-
course, and by transfusion of blood products. Newborns and chil-
dren by birth most often (infected mother), child abuse, and blood
products.
There is an estimated one in a million chance of transmission of
HIV via receipt of a blood transfusion due to donation from people
who are HIV-infected but have not yet developed antibodies to HIV.
These donors are therefore not picked up by enzyme-linked im-
munosorbent assay (ELISA) screens, by may be identified by lifestyle
screens. However, newer blood testing involves checking for HIV
antigens (like p24).
HIV infects lymphocytes and other cells bearing the CD4 surface
marker. HIV infection leads to impaired cell-mediated and humoral
immunity. AIDS is due to HIV infection and is characterized by im-
mune dysfunction, opportunistic infections, and unusual malignan-
cies. The incubation period between HIV infection and the onset of
AIDS is about 10 years in adults. (See Fig. 7–1.)

Hint 7

Screening
Screening includes detection of HIV infection by an ELISA, and
confirmation by Western blot.
Screening of high-risk patients and blood products/blood
donors is critical. Screening via voluntary screening sites, and by
most insurance companies for life insurance policies, is in effect.
Universal premarital or populace screening may prove costly, with
low yield and increased false-positive reports, but research is ongo-
ing to see if more widespread screening could save lives.
U.S. blood donation centers began screening in March 1985.
Minimal transfusion-related HIV/AIDS risk remains. Initial lab test-
ing for patients who test positive for HIV should include absolute
CD4 lymphocyte count and CD4 lymphocyte percentage. Counts less
than 500 cells/mm3 may indicate HIV-associated immunodeficiency.
CD4 < 200 is associated with a high risk for opportunistic infections
or malignancy.
Prevention
Cornerstone is education (including safe sex and effective use of
condoms, types of high-risk behavior). Special effort to address high-
risk groups (homosexuals, intravenous (IV) drug users/parenteral
exposure, prostitutes, promiscuous individuals, hemophiliacs, part-
ners of high-risk individuals) are needed. Education and regulation
to protect health care workers and their patients (decontamination, gloves, reduction of needlesticks). Screening will provide both pa-
tient education/treatment and partner tracing/treatment. Distribu-
tion of condoms and bleach has been attempted. Distribution of
sterile needles has been proposed.

Treatment Steps
Antiretroviral therapy inhibits viral replication by inhibiting tran-
scriptase. Nucleoside analogue (stavudine, abacavir) and non-nucle-
oside (delaviridine, efavirenz) reverse transcriptase inhibitors can be
used in conjunction with protease inhibitors (amprenavir, indi-
navir). (See also Chapter 9.)

Front 8

Fetal red blood cell antigens at-
tacked by maternal immunoglobulin G (IgG) (isoimmune disease)
or anti-A or anti-B (ABO disease) antibodies, after transplacental
passage.

Back 8

Newborn Hemolytic Disease / erythroblastosis fetalis

Hint 8

Prevention includes maternal testing for type, Rh, and antibody
screen; Rho(D) immune globulin (RhoGAM) injection; more accu-
rate screening for maternal sensitization/fetal disease (amniocente-
sis), with intervention (labor induction) as necessary.
Give Rh immune globulin injection for Rh-negative mothers (see
Table 7–1).
Kleihauer test: determines amount of fetal cells in maternal serum. Perform after traumatic delivery to determine amount of Rh immune globulin to give.
RhoGAM INJECTIONS
If mother is Rh negative: Give injection at 28 weeks, within 3 days of delivery, after any
bleeding, after amniocentesis.
If baby is Rh positive: Give another dose postpartum.
Standard dose: 1 mL (300 µg); counteracts 10 mL antigenic fetal cells

Front 9

Usually ABO incompatibility. Prevention includes proper blood-
bank specimen handling and technique, along with accurate patient
and specimen identification. Other factors include close patient ob-
servation during transfusion (major hemolytic reactions occur early
during transfusion), and observation of the blood itself (abnormal
color may indicate bacterial contamination)

Back 9

Prevention of Transfusion Reactions
Patient history of atopy or asthma may predispose to allergic
transfusion reactions, so additional caution/observation is indicated.
Pretransfusion treatment with antihistamines may be of value, along
with the use of washed erythrocytes.
Patient history of multiple transfusions or pregnancies may pre-
dispose to febrile transfusion reactions. May try infusing leukocyte-
depleted infusions (donor leukocyte antigens may result in the reac-
tion).

Hint 9

Patient history and examination will assist in dictating rate and
amount of volume to be infused (thus avoiding congestive heart fail-
ure).

Front 10

Immunization Time
Hepatitis B

Back 10

#1 from birth to 2 months
#2 from 1 month to 4 months (at least 1 month after the first
dose)
#3 from 6 months to 18 months (at least 4 months after the first
dose and at least 2 months after the second dose, but not before
6 months of age for infants)
Infants born to hepatitis B surface antigen (HbsAg)-positive
mothers should receive the hepatitis B vaccine and hepatitis B immune globulin (HBIG) within 12 hours of birth
Infants born to mothers whose HBsAg status is unkown should
receive the hepatits B vaccine within 12 hours of birth

Front 11

Immunization Time
Diptheria and Tetanus Toxoids and Acellular Pertussis
(DTaP)

Back 11

#1 at 2 months
#2 at 4 months
#3 at 6 months
#4 at 15–18 months
#5 at 4–6 years

Hint 11

Difference between DT
and Td—Children with
pertussis vaccine
reaction are given DT.
Children older than 7 and
adults are given Td
vaccine (less diphtheria
toxoid dose).

Front 12

Immunization Time
etanus and Diptheria Toxoids (TD)

Back 12

11–12 Years
of Age and Every 10 Years

Hint 12

Difference between DT
and Td—Children with
pertussis vaccine
reaction are given DT.
Children older than 7 and
adults are given Td
vaccine (less diphtheria
toxoid dose).

Front 13

Immunization Time
H. influenzae Type b (Hib)

Back 13

#1 at 2 months
#2 at 4 months
#3 at 6 months
#4 at 12–15 months

Front 14

Immunization Time
Inactivated Polio (PV)

Back 14

#1 at 2 months
#2 at 4 months
#3 at 6–18 months
#4 at 4–6 years
Oral polio vaccine (OPV) should only be used in selected situa-
tions.

Front 15

Immunization Time
Pneumococcal Conjugate (PCV)

Back 15

#1 at 2 months
#2 at 4 months
#3 at 6 months
#4 at 12–15 months

Front 16

Immunization Time
Measles–Mumps–Rubella (MMR)

Back 16

#1 at 12 to 15 months
#2 at 4 to 6 years

Front 17

Immunization Time
Varicella

Back 17

12–18 months
Susceptible patients 13 years of age or older should receive two doses at least 4 weeks apart.

Front 18

Immunization Time
Hepatitis A Vaccination

Back 18

Recommended in selected states and for high-risk groups after 2 years of age.

Front 19

Immunization Time
Adults

Back 19

• Tetanus, diphtheria (Td) every 10 years.
• Influenza (yearly in elderly or chronic cardiopulmonary disease
patients).
• Pneumococcus vaccine (high-risk chronic cardiopulmonary dis-
ease, asplenia, diabetes, and elderly).
• Hepatitis vaccine if in higher-risk group
Other specific vaccines for travel and/or work: rabies, cholera, typhoid

Front 20

Postexposure Therapy
Hepatitis: .
Rabies:
Tetanus:
Varicella:

Back 20

Postexposure Therapy

Front 21

Compromised Immune System Patients
Immunization

Back 21

Give zoster immune globulin (ZIG) to prevent chickenpox, if exposed.
Avoid live, attenuated vaccines (increased risk of paralysis with
trivalent oral polio vaccine [TOPV]). Avoid bacillus Calmette–Guérin (BCG) vaccine.
Okay to give influenza, inactivated polio, and pneumococcal vaccines.

Front 22

Severe Egg Allergy
Immunization

Back 22

ontroversy exists about avoiding vaccines grown on chick or duck
embryos, which include vaccines for yellow fever and influenza. In-
fluenza vaccines may be tolerated by egg-allergic individuals. Measles
and mumps vaccines are grown on chick or duck fibroblast tissue
cultures and are generally free of egg albumin. Measles and mumps
vaccines can therefore be used in the severe egg allergic individual
but caution should be taken

Front 23

cough, fever, chills,
dyspnea; peripheral neutrophilia (no eosinophilia); nodular or fibrotic radiograph.

Back 23

Hypersensitivity pneumonitis—IgE or IgG-mediated reaction to organic dust

Front 24

history of asthma; fever, wheezing, productive cough; leukocytosis,
sputum and blood eosinophilia, elevated total serum IgE, serum precipitins for Aspergillus;
radiographic infiltrates from proximal bronchiectasis.

Back 24

Allergic bronchopulmonary aspergillosis—IgE-mediated and immune complex–mediated
reaction to Aspergillus

Front 25

pulmonary hemorrhage and
nephritis; hemoptysis, dyspnea, lethargy, hematuria, proteinuria; positive serum anti-GBM antibody; acinar consolidation or reticular radiographic pattern; restrictive lung defect.

Back 25

Goodpasture’s syndrome—Type II or antigen–antibody reaction

Front 26

eosinophilia
with migratory infiltrates; mild cough, wheeze, low-grade fever, myalgias;

Back 26

Pulmonary eosinophilia (Löffler syndrome)—IgE-mediated reaction or idiopathic

Hint 26

good prognosis

Front 27

Symptoms
Slow-onset, swelling attacks without hives (lasting 1–4 days)

Diagnosis
History and physical examination, lab studies, no hives present, no
pruritus.
C4 levels are diminished during asymptomatic periods and are
undetectable during an attack. C2 levels are normal during asympto-
matic periods and are diminished during an attack. C1-
esterase inhibitor levels (functional or qualitative rather than quanti-
tative levels) are most specific. C1 may be low in acquired angioneu-
rotic edema states but not in hereditary angioneurotic edema.

Back 27

Hereditary Angioedema

Pathology
Autosomal dominant; C1 esterase inhibitor deficiency. Rare.

Hint 27

Treatment Steps
1. Supportive.
2. Danazol or stanozolol
Attacks may affect
laryngeal area resulting in
airway obstruction.
Epinephrine/
corticosteroids of little
value

Front 28

Symptoms/Diagnosis
Swelling; may be associated with hives and pruritus. Clinical diagnosis.

Back 28

Angioedema

Pathology
Cutaneous anaphylaxis resulting from IgE-mediated reaction (though may also result from unknown causes).

Hint 28

Treatment Steps
1. Antihistamines (Benadryl, Claritin).
2. H2 blockers (Zantac).
3. Leukotriene modifiers.
4. Tricyclic antidepressants in low doses (Sinequan).
5. Oral adrenergics (Proventil Repetab).
6. Corticosteroids.
7. Epinephrine

Front 29

Symptoms/Diagnosis
Hives (wheal and flare), pruritus. Clinical diagnosis.

Back 29

Urticaria (Hives)
Other urticarial triggers.
Cold, heat, sun,
vibration/pressure.
Most common physical
urticaric disorder:
dermographism

Hint 29

Treatment Steps
1. Antihistamines (Benadryl, Claritin).
2. H2 blockers (Zantac).
3. Leukotriene modifiers.
4. Tricyclic antidepressants in low doses (Sinequan).
5. Oral adrenergics (Proventil Repetab).
6. Corticosteroids.
7. Epinephrine.

Front 30

Symptoms
Itchy nose/eyes, rhinitis, dry cough, and sneezing

Back 30

Allergic Rhinitis

Description
Seasonal or continuous eye, nose, and throat symptoms. Caused by histamine release (and other immune mediators) in response to allergens.

Hint 30

Diagnosis
History and physical examination, allergy testing (skin and/or
RAST, including IgE level), nasal smear for eosinophils

Treatment Steps
1. Avoidance techniques.
2. Medicines:
a. Oral antihistamines (Benadryl or Zyrtec) may be added to oral
decongestants (Sudafed).
b. Oral antihistamine/decongestant combination preparations
(Allegra D or Claritin D) may be used instead of individual an-
tihistamines and decongestants.
c. Nasal corticosteroids (Nasonex).
d. Nasal antihistamines (Astelin).
e. Nasal ipratropium bromide (Atrovent 0.03%).
f. Nasal cromolyn sodium (Nasalcrom).
g. Nasal decongestants (Afrin) should be avoided for extended
periods as they may cause rhinitis medicamentosa.
h. Oral corticosteroids (prednisone).
3. Desensitization, also known as immunotherapy, to inhaled aller-
gens

Front 31

Nasal smear may show
eosinophils, but allergic
rhinitis may not be
present

Back 31

nonallergic rhinitis
with eosinophilia syndrome
(NARES), also known as
eosinophilic nonallergic
rhinitis (ENR).

Front 32

Symptoms
Wheezing, cough, and dyspnea.

Back 32

Asthma

Description
Usually, reversible airway obstructive disorder, characterized by in-
flammation and bronchospasm. Asthma can be classified into the
following categories depending on the degree of symptomatology
such as frequency of awakenings, absenteeism, need for inhalers and
emergent intervention:
1. Mild intermittent—symptoms ≤ 2/week, nighttime symptoms ≤
2/month, forced expiratory volume in 1 second (FEV1) ≥ 80% of
predicted.
2. Mild persistent—symptoms > 2/week but < 1/day, nighttime
symptoms > 2/month, FEV1 ≥ 80% of predicted
3. Moderate persistent—daily symptoms, symptoms affect activity,
daily use of short-acting β2-agonist, nighttime symptoms
> 1/week, FEV1 > 60% but ≤ 80% of predicted.
4. Severe persistent—continual symptoms, limited physical activity, frequent nighttime symptoms, FEV1 ≤ 60% of predicted.

Pathology
Airway inflammation and hyperreactivity (triggered by allergen exposure or emotional/environmental factors).

Diagnosis
History and physical examination, chest x-ray, pulmonary function
testing (pre- and postbronchodilator), provocation testing (with
methacholine), allergy testing.

Hint 32

Treatment Steps
Education, avoid inciting agents, home peak flow monitoring. Phar-
maceutical treatment consists of quick-relief and long-term-control
medicines. Start with a quick-relief medicine (e.g., Proventil HFA
prn), and then consider adding a long-term-control medicine (e.g.,
Pulmicort Turbuhaler) if the quick-relief medicine is necessary more
than twice weekly.
Quick-Relief Medicines
1. Inhaled short-acting β2-adrenergic (Proventil HFA or al-
buterol solution via nebulizer or inhaler).
2. Inhaled ipratropium bromide (Atrovent).
3. Subcutaneous epinephrine.
4. Oral corticosteroids.
5. Intravenous corticosteroids (prednisone, methylprednisolone).
6. Intravenous aminophylline.
7. Oxygen.
Long-Term-Control Medicines
1. Inhaled corticosteroid (fluticasone, beclomethasone).
2. Inhaled long-acting β2-adrenergic (salmeterol).
3. Inhaled cromolyn sodium (Intal).
4. Inhaled nedocromil sodium (Tilade).
5. Oral sustained-release theophylline (UniDur).
6. Oral leukotriene modifiers (Singulair, Accolate, Zyflo).
7. Combination inhaled corticosteroid and long-acting β-adren-
ergic (Advair).
All patients should always have a metered-dose inhaler (MDI)
with a short-acting β2-agonist (albuterol) to be used as a rescue medication.
Management of Asthma Exacerbations
• Often occurs in the hospital. Oxygen will be utilized, as well as
nebulized solutions of albuterol, usually with ipratropium bro-
mide (Atrovent).
• Systemic corticosteroids (prednisone or solumedrol) used as
well as empiric antibiotics.
• Peak flows should be followed, as well as oxygenation and CO2
retention. Due to rapid respiratory rate, CO2 levels on arterial
blood gases (ABGs) should be low. If patient is acutely ill and
CO2 is returning to normal or is elevated, mechanical ventila-
tion may be needed, as the patient may be heading toward
acute respiratory failure.
• Subcutaneous epinephrine and magnesium are sometimes
given in the emergency department.
• Alternative methods used include heliox, a mixture of oxygen
and helium, to improve oxygenation.
Adjunctive Therapy in Asthma
• Seasonal allergies/allergic rhinitis can worsen asthma. Nasal
corticosteroids are very effective, as well as newer antihista-
mines, which are less sedating: loratidine (Claritin), cetirizine
(Zyrtec), or fexofenadine (Allegra) (see prior section, Allergic
Rhinitis).
• Gastroesophageal reflux disease (GERD) can worsen asthma,
particularly causing nighttime cough. Treatment with proton
pump inhibitors can be very effective.
• Educating patients on proper use of inhalers, careful monitor-
ing of peak flows and symptoms, what to do in an acute attack,
avoidance of triggers, and importance of maintenance medications is very important to minimize morbidity and mortality.

Front 33

Asthma symptoms ≤ 2/week, nighttime
symptoms ≤
2/month, forced expiratory volume in 1 second (FEV1) ≥ 80% of predicted

Back 33

Mild intermittent Asthma

Hint 33

Treatment of Mild Intermittent Asthma
1. Daily medication not needed. Inhaled short-acting β2-adrener-
gic (albuterol) 2 puffs qid, prn.
2. For exercise-induced asthma, treat with an inhaled short- or
long-acting β2-adrenergic (as above) 15–20 minutes prior to
exercise.

Front 34

Asthma symptoms > 2/week but < 1/day, nighttime
symptoms > 2/month, FEV1 ≥ 80% of predicted.

Back 34

Mild persistent Asthma

Hint 34

Treatment of Mild Persistent Asthma
Daily medication for long-term control: low-dose inhaled cortico-
steroids. Alternatives include cromolyn, leukotriene-modifying
drugs, nedocromil, or theophylline.

Front 35

daily symptoms, symptoms affect activity,
daily use of short-acting β2-agonist, nighttime symptoms
> 1/week, FEV1 > 60% but ≤ 80% of predicted.

Back 35

Moderate persistent Asthma

Hint 35

Treatment of Moderate Persistent Asthma
Daily medications for long-term control should include low- to
medium-dose inhaled corticosteroid and long-acting inhaled β2-
agonist (can be accomplished using one inhaler, Advair). Alter-
natives include increasing inhaled corticosteroid dose or adding either a leukotriene modifier or theophylline.

Front 36

continual symptoms, limited physical activity,
frequent nighttime symptoms, FEV1 ≤ 60% of predicted.

Back 36

Severe persistent Asthma

Hint 36

Treatment of Severe Persistent Asthma
1. Daily mediations for long-term control should include high-
dose inhaled corticosteroids and long-acting inhaled β2-agonist.
2. If needed, oral corticosteroids can be used with repeated at-
tempts to reduce the systemic dose and maintain control with
inhaled corticosteroid.

Front 37

ASTHMA
differential
diagnosis

Back 37

1. Emphysema
2. Chronic bronchitis
3. Congestive heart
failure
4. Pulmonary embolism
5. Intraluminal lesions
(e.g., foreign body,
carcinoma, adenoma)
6. Carcinoid
7. Mastocytosis
8. Parasitic infestations
9. Extraluminal lesions
(e.g., lymphoma, aortic
aneurysm)
10. Gastroesophageal
reflux
11. Allergic rhinitis/sinusitis
12. Vocal cord dysfunction
13. Cystic fibrosis
14. Cough secondary
to drugs (e.g.,
angiotensin-converting
enzyme [ACE]
inhibitors)

Front 38

Child with purulent
rhinitis

Back 38

rule out foreign
body.

Front 39

Child with nasal polyps

Back 39

rule out cystic fibrosis.

Front 40

Head injury with rhinitis:

Back 40

rule out cerebrospinal fluid
(CSF) leak.

Front 41

Continuous rhinitis with
normal nasal smear:

Back 41

rule out vasomotor rhinitis

Front 42

Child with large foul
stools and wheezing:

Back 42

rule out cystic fibrosis.

Front 43

Wheezing with diarrhea
and flushing

Back 43

rule out carcinoid

Front 44

Child with unilateral
sudden wheezing

Back 44

rule out foreign body.

Front 45

Symptoms
Cough, fever, chills (4–6 hours after exposure to offending allergen).

Back 45

Hypersensitivity Pneumonitis

Description
Allergic pulmonary disorder, from organic dust inhalation (see Table 7–2).

Pathology
IgE- or IgG-mediated reaction to dust inhalation. Includes wide vari-
ety of antigens (wood, hair, mold, trees).

Hint 45

Diagnosis
History and physical examination, chest x-ray, pulmonary function
testing, inhalation challenge testing, lung biopsy.

Treatment Steps
1 Avoid inciting agent.
2. See the treatment of asthma.

Front 46

Description/Symptoms
Autoimmune disease causing both pulmonary and renal disease.
Symptoms include hemoptysis, hematuria, cough, anemia, lethargy,
proteinuria

Diagnosis
History and physical examination, renal biopsy, chest x-ray, positive
serum anti–glomerular basement membrane (anti-GBM) antibody, and antialveolar basement membrane antibody (see Fig. 7–2)

Back 46

Goodpasture’s Syndrome

Pathology
Linear immunoglobin deposits noted on glomerular basement membrane.

Hint 46

Treatment Steps
1. Plasmapheresis.
2. Prednisone (1 mg/kg/day).
3. Cyclophosphamide (1–2 mg/kg/day).

Front 47

Symptoms
Eosinophilia, cough, pulmonary infiltrates, or asymptomatic

Back 47

Pulmonary Eosinophilia (Löffler Syndrome)

Pathology
IgE-mediated reaction to various agents, or idiopathic.

Hint 47

Treatment Steps
1. Eliminate exposure to the offending agent.
2. Corticosteroids—a brief tapering course of prednisone.

Front 48

Symptoms/Diagnosis
Shock, hypotension, airway obstruction/bronchoconstriction, urticaria, angioedema. Clinical diagnosis.

Back 48

Anaphylactic Shock

Description
Acute, life-threatening allergic reaction
Anaphylactoid reactions:
Etiology is direct
mediator release
(contrast dye, for
example), presentation/
treatment similar to
anaphylaxis.

Hint 48

Treatment Steps
1. Control airway.
2. Cardiopulmonary resuscitation (CPR).
3. Epinephrine 1:1,000 (0.3–0.5 cc SQ).
4. Repeat epinephrine twice every 15–20 minutes.
5. Volume replacement.
6. Corticosteroids.
7. Antihistamines.
8. β-Agonists.

Front 49

Symptoms
Dyspnea, hypotension, lethargy (symptoms of anemia).

Back 49

Hemolytic Drug Reactions

Description
Hemolysis secondary to medication ingestion.

Pathology
Etiology includes immune complex formation, medication antibod-
ies, and autoimmune hemolytic anemia, methyldopa (Aldomet).

Hint 49

Diagnosis
Clinical, serologic studies (direct Coombs’)

Treatment Steps
1. Stop offending medication.
2. Corticosteroids have limited benefit in autoimmune-type hemolysis.

Front 50

THYROIDITIS TYPES
(fever/pain)

Back 50

Acute: bacterial

Front 51

THYROIDITIS TYPES
(fever/pain/large
gland/high sed rate).

Back 51

Subacute (de
Quervain’s): viral?

Front 52

THYROIDITIS TYPES
Chronic

Back 52

Chronic (Hashimoto’s):
autoimmune.
Increased incidence of
systemic lupus
erythematosus (SLE),
scleroderma and
pernicious anemia with
Hashimoto’s disease.
also includes
Reidel’s thyroiditis
(unknown cause).

Front 53

Hashimoto’s disease
plus Addison’s
disease.

Back 53

Schmidt syndrome:

Front 54

thyroid inflammation; female preponderance; hyper- or
euthyroid at first; positive antimicrosomal antibody

Back 54

Chronic thyroiditis—Hashimoto’s

Hint 54

treat with thyroid hormone replacement.

Front 55

hyperthyroidism; thyrotoxicosis; antithyroid antibodies, homogeneous thyroid
scan;

Back 55

Graves’ disease

Hint 55

treat with β-adrenergic blockers followed by antithyroid medications.

Front 56

weight loss, lethargy, metabolic acidosis

Back 56

Addison’s disease—adrenocortical insufficiency

Hint 56

diagnose with adrenocorticotropic hormone (ACTH) stimulation test; treat with corticosteroids.

Front 57

anemia, antiparietal cell and anti-intrinsic factor
antibodies;

Back 57

Pernicious anemia—autoimmune B12 deficiency

Hint 57

treat with parenteral vitamin B12.

Front 58

thrombocytopenia due to antiplatelet antibodies;
purpura; petechia;

Back 58

Idiopathic thrombocytopenic purpura
Acute ITP: usually
children, often postviral,
self-limited disease usually.
Chronic ITP: usually
adults.

Hint 58

treat with corticosteroids, possibly splenectomy

Front 59

hemolytic anemia (most common), (cold induced), (cold induced), (due to D antigen Rh).

Back 59

Immune hemolysis
includes warm autoimmune hemolytic anemia (most common), paroxysmal
cold hemoglobinuria (cold induced), cold-agglutinin disease (cold induced), and newborn
hemolytic disease (due to D antigen Rh).

Front 60

jaundice, ascites, extrahepatic symptoms;

Back 60

Chronic active hepatitis
due to virus, medication, or
other etiology

Hint 60

treat with corticosteroids.

Front 61

hematuria, edema, hypertension due to poststreptococcal infection, SLE,
and vasculitis.

Back 61

Glomerulonephritis

Front 62

vertigo, nystagmus,
incoordination;

Back 62

Multiple sclerosis—demyelinating central nervous system (CNS) disease

Hint 62

diagnose with magnetic resonance imaging (MRI), lumbar puncture; treat with
immunosuppressives.

Front 63

ptosis, diplopia;
positive acetylcholine receptor antibodies

Back 63

Myasthenia gravis—neuromuscular disorder;

Hint 63

diagnose with electromyogram
(EMG), tensilon test
treat with thymectomy,
corticosteroids.

Front 64

Symptoms
Early-onset jaundice, anemia, kernicterus.

Back 64

Newborn—Hemolytic Disease

Description
Newborn immune hemolytic disorder (erythroblastosis fetalis). See
Table 7–1.

Pathology
Mother’s antibodies (most often D antigen Rh), attack fetal blood cells.

Hint 64

Diagnosis
History and physical examination, prenatal lab studies, amniocente-
sis, positive direct Coombs’.

Treatment Steps
1. Prevention.
2. Predelivery: fetoscopic transfusion.
3. After delivery: exchange transfusion, phototherapy

Front 65

For exam purposes, the patient is often a young female with joint pain.
Also may have malar rash (see Fig. 7–4), symptoms of fatigue.
• Malar rash
• Discoid rash
• Photosensitivitiy
• Oral ulcers
• Arthritis (nonerosive, in
two or more joints)
• Serositis (pleuritis or
pericarditis)
• Renal disorder
(proteinuria or casts)
• Neurologic disorder
(seizures or psychosis)
• Hematologic disorder
(hemolytic anemia,
leukopenia,
lymphopenia,
thrombocytopenia)
• Positive ANA
• Immunologic disorder (positive anti-doublestranded DNA, positive anti-SM, positive antiphospholipid antibody, or false + syphilis test([VDRL])
malar rash, arthritis, proteinuria,
vasculitis, alopecia; positive ANA, antiphospholipid antibodies

Back 65

Systemic Lupus Erythematosus (SLE)

Description
Multisymptom inflammatory immune disorder.

Symptoms
Since SLE is a multisystem disorder, symptomatology is variable

Pathology
Immune mediated with increased incidence in females, and those
with positive family histories of SLE.
Disorders that may
coexist with SLE:
psoriasis, porphyria
cutanea tarda, Sjögren’s
syndrome
Systemic lupus erythematosus—multisystem disorder; malar rash, arthritis, proteinuria,
vasculitis, alopecia; positive ANA, antiphospholipid antibodies; treat with corticosteroids.

Hint 65

Treatment Steps
1. Corticosteroids.
2. Immunosuppressives.
3. Antimalarials

Front 66

Symptoms
Asymmetric joint pain, tenderness and swelling, for > 6 weeks’ duration, fever, morning stiffness
asymmetric joint pain, fever, morning stiffness; abnormal
erythrocyte sedimentation rate (ESR), RF, ANA, joint x-rays

Back 66

Juvenile Rheumatoid Arthritis (JRA)

Description
Most frequent childhood rheumatic disorder (peak age 1–2 years).
Also called juvenile chronic arthritis or Still’s disease.

Pathology
Autoimmune-induced synovitis—three types: systemic, polyarticular,
and pauciarticular
Systemic JRA: Still’s
disease; adenopathy,
fever.
• Polyarticular: ≥ 5 joints
affected.
• Pauciarticular: ≤ 4
joints, most frequent
type, iridocyclitis is
most significant
complication.
Juvenile rheumatoid arthritis—asymmetric joint pain, fever, morning stiffness; abnormal
erythrocyte sedimentation rate (ESR), RF, ANA, joint x-rays; systemic, polyarticular or
pauciarticular; treat with salicylates, NSAIDs, gold, corticosteroids.

Hint 66

Diagnosis
History and physical examination, lab studies (sed rate, rheumatoid
factor [RF], antinuclear antibody [ANA], etc.), x-ray.

Treatment Steps
1. Salicylates.
2. Nonsteroidal anti-inflammatory drugs (NSAIDs).
3. Gold.
4. Antimalarials.
5. Corticosteroids.
3. Adult Rheumatoid

Front 67

Symptoms
Symmetric joint swelling, morning stiffness, subcutaneous nodules, extra-articular symptoms (episcleritis, neuropathy, pulmonary fibrosis/pleuritis, vasculitis, lethargy) (see Fig. 7–5
hronic synovitis, symmetric joint swelling, subcutaneous nodules, extraarticular symptoms; abnormal ESR, RF, x-rays

Back 67

Adult Rheumatoid Arthritis

Description
Chronic adult immune-mediated synovitis.

Pathology
Chronic synovitis resulting from immune complex formation with joint/tissue injury.
Adult rheumatoid arthritis—chronic synovitis, symmetric joint swelling, subcutaneous nodules,
extraarticular symptoms; abnormal ESR, RF, x-rays; immune-complex formation; treat with
salicylates, NSAIDs, gold, corticosteroids

Hint 67

Diagnosis
History and physical examination (with attention to American
Rheumatism Association criteria), lab (RF, sed rate), x-ray (marginal
erosions/osteoporosis), synovial fluid analysis.

Treatment Steps
1. NSAIDs.
2. Corticosteroids.
3. Disease-modifying antirheumatic drugs (DMARDs) including
penicillamine, gold salts, immunosuppressants (methotrexate,
immuran), and hydroxychloroquine.
4. Physical therapy/occupational therapy (PT/OT).
5. Surgery.
6. New “biologic” medications (see Cram Facts).
RHEUMATOID ARTHRITIS
TREATMENT
Three new biologic
medications are used in the
treatment of rheumatoid
arhritis. All three work to
block TNF-α:
• Etanercept (Enbrel)
• Adalimumab (Humira)
• Infliximab (Remicade)

Front 68

RA,
granulocytopenia, and
splenomegal

Back 68

Felty syndrome

Front 69

Symptoms
polyarthritis, chorea, carditis, erythema marginatum, subcutaneous nodules; abnormal ASO, CRP, ESR, leukocytosis
mj: polyarthritis, chorea, carditis, erythema marginatum, and
subcutaneous nodules.
mn: fever, abnormal lab studies (antistreptolysin-O [ASO] titer, Creactive
protein [CRP], sed rate, leukocytosis).

Back 69

Rheumatic Fever

Description
Poststreptococcal multisystem inflammatory disorder.

Pathology
Multisystem tissue inflammation; a possible result of host versus streptococcal antigen reaction.
Rheumatic fever—poststreptococcal multisystem inflammatory disease; polyarthritis, chorea,
carditis, erythema marginatum, subcutaneous nodules; abnormal ASO, CRP, ESR,
leukocytosis; treat with penicillin, NSAIDs, corticosteroids.

Hint 69

Diagnosis
History and physical examination, streptococcal infection plus Jones
criteria (two major symptoms or one major plus two minor).

Treatment Steps
1. Penicillin (eliminates strep, but will not alter RF disease course).
2. NSAIDs.
3. Prednisone (for carditis).

Front 70

Symptoms
Affects skin/vascular system (Raynaud’s phenomenon), GI tract, renal, pulmonary, and cardiac systems. Also anemia, dyspnea, and arthritis.
multisystem, anemia, dyspnea, arthritis;
abnormal ANA, single-stranded RNA, biopsy;

Back 70

Scleroderma

Description
Multisystem connective tissue disorder (progressive systemic sclerosis).

Pathology
Increased skin and organ collagen deposition, along with vascular
narrowing. May be localized (morphea), or generalized.
• Most frequent cause for
scleroderma death:
renal disease.
• Most frequent internal
organ affected:
esophagus.
Scleroderma—progressive systemic sclerosis; multisystem, anemia, dyspnea, arthritis;
abnormal ANA, single-stranded RNA, biopsy; treat with corticosteroids.

Hint 70

Diagnosis
History and physical examination, lab studies (single-strand RNA,
ANA), biopsy

Treatment Steps
1. Supportive.
2. Corticosteroids.

Front 71

high sed rate/back pain in elderly
severe shoulder and pelvis pain/stiffness in elderly, fever, lethargy; no decreased joint motion; abnormal cortisol secretion rate, human lymphocyte antigen (HLA)-
associated;

Back 71

POLYMYALGIA RHEUMATICA
Temporal arteritis may
coexist (diagnosis by
temporal artery biopsy).
Be sure to rule out
multiple myeloma

Hint 71

Polymyalgia rheumatica—severe shoulder and pelvis pain/stiffness in elderly, fever, lethargy; no
decreased joint motion; abnormal cortisol secretion rate, human lymphocyte antigen (HLA)-
associated; treat with corticosteroids.

Front 72

Description
Genetic disorder characterized by abnormal facies, congenital heart
disease, hypocalcemia, and increased susceptibility to infections.

Back 72

DiGeorge Anomaly


Pathology
Hypoplasia or aplasia of the thymus occurs, causing defective T cells.

Hint 72

Treatment Steps
Early thymus transplantation may promote immune reconstitution.

Front 73

Severe Combined Immunodeficiency Disease
(SCID)

Back 73

SCID refers to several genetic diseases that result in B- and T-cell disorders.
The most common type is X-linked. The other forms are autosomal
recessive or due to spontaneous mutations. One of these
forms is linked to a deficiency of the enzyme adenosine deaminase
(ADA).
Patients are at increased risk of infection and if SCID is not diagnosed
and treated early, it can result in severe infection and death
by age 2.

Hint 73

Treatment includes prophylaxis against PCP (Pneumocystis
carinii pneumonia). In certain genetic causes, bone marrow transplantation
is offered. If caused by ADA deficiency, ADA replacement
may be warranted. Gene therapy is being investigated

Front 74

Symptoms
Fever, pain, lethargy. Dysfunction of transplanted organ.

Back 74

Transplantation Rejection

Description
Immunological transplant rejection.

Pathology
Lymphocyte- and antibody-induced reaction, with T-cell graft tissue
injury (acute reaction). Renal transplant may also present as hyperacute
or chronic reaction.

Hint 74

Diagnosis
History and physical examination, additional studies (kidney: blood
urea nitrogen [BUN], creatinine, renal biopsy).

Treatment Steps
Immunosuppressive medication (corticosteroids, cytotoxic medications,
antimetabolites).

Front 75

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 75

Thermophilic actinomycetes

Front 76

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 76

Bacillus subtilis

Front 77

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 77

Streptomyces albus

Front 78

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 78

Aspergillus spp.

Front 79

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 79

Aureobasidium, Graphium spp

Front 80

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 80

Cryptostroma corticale

Front 81

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 81

Penicillium casei

Front 82

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 82

Sacchoromonospora viridis

Front 83

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 83

Various undetermined puffball spores

Front 84

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS

Back 84

Alternaria, Penicillium spp

Front 85

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS
Infested flour

Back 85

Sitophilus granarius (wheat weevil)

Hint 85

Wheat miller’s lung

Front 86

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS
Various industries

Back 86

Isocyanates

Hint 86

Chemical worker’s lung

Front 87

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS
Medication

Back 87

Pituitary snuff

Hint 87

Pituitary snuff taker’s lung

Front 88

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS
Coffee bean dust

Back 88

Coffee bean protein

Hint 88

Coffee worker’s lung

Front 89

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS
Laboratory rats

Back 89

Rat urine protein

Hint 89

Laboratory worker’s lung

Front 90

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS
Animal pelts

Back 90

Animal fur protein

Hint 90

Furrier’s lung

Front 91

ALLERGENS CAUSING HYPERSENSITIVITY PNEUMONITIS
Contaminated tap water

Back 91

Unknown

Hint 91

Tap water hypersensitivity pneumonitis