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A 39-year-old woman comes to the emergency department due to mild shortness of breath and right-sided
pleuritic chest pain that began this morning. The patient underwent a mastectomy for left-sided breast
cancer 2 weeks ago. She has a history of hives after eating seafood.
Blood pressure is 148/96 mm Hg, pulse is 114/min, and respirations are 21∕min. Oxygen saturation is 91%
on room air.
Chest x-ray is unremarkable and urine pregnancy test is negative. Creatinine is 0.8 mg∕dL.
Which of the following is the most appropriate diagnostic approach?
A. CT pulmonary angiography after pretreatment with glucocorticoids and antihistamines
B. CT pulmonary angiography using a high-osmolar radiocontrast material
C. CT pulmonary angiography without specific precautions
D. Lower extremity compression ultrasonography
E. Ventilation-perfusion scan

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A 39-year-old woman comes to the emergency department due to mild shortness of breath and right-sided
pleuritic chest pain that began this morning. The patient underwent a mastectomy for left-sided breast
cancer 2 weeks ago. She has a history of hives after eating seafood.
Blood pressure is 148/96 mm Hg, pulse is 114/min, and respirations are 21∕min. Oxygen saturation is 91%
on room air.
Chest x-ray is unremarkable and urine pregnancy test is negative. Creatinine is 0.8 mg∕dL.
Which of the following is the most appropriate diagnostic approach?
A. CT pulmonary angiography after pretreatment with glucocorticoids and antihistamines [25%]
B. CT pulmonary angiography using a high-osmolar radiocontrast material [4%]
C. CT pulmonary angiography without specific precautions [59%]
D. Lower extremity compression ultrasonography [3%]
E. Ventilation-perfusion scan [7%]

Omitted
Correct answer
Ihi 59% 25 SecondS 05/06/2019
Answered correctly Time Spent Last Updated
C

Explanation

This patient’s presentation with dyspnea, tachypnea, tachycardia, chest pain, and hypoxia, along with a
history of breast cancer, is highly suggestive of acute pulmonary embolism (PE). CT pulmonary
angiography (CTPA) using intravenous, usually iodinated radiocontrast, is the first-choice imaging test for
diagnosing acute PE.
The exact mechanism of radiocontrast-induced anaphylaxis is not known. However, the widely held
notion that seafood allergy increases the risk of anaphylaxis to iodinated contrast has no basis in medical
literature. Therefore, patients with seafood allergy can receive iodinated contrast and do not require
pretreatment with glucocorticoids and antihistamines (Choice A). The converse is also true—patients
who have had anaphylaxis due to iodinated contrast do not need to avoid seafood.
(Choice B) High-osmolar radiocontrast is associated with increased rates of allergic reaction and contrast-
induced nephropathy. Therefore, low- or iso-osmolar radiocontrast is used in most settings regardless of
allergy history.
(Choice D) Compression ultrasonography can diagnose deep venous thrombosis (DVT) and suggest the
presence of PE. However, the absence of DVT does not rule out PE. CTPA is more diagnostically
definitive and is the preferred study in patients without contraindication.
(Choice E) Ventilation-perfusion scanning may be used in evaluation of PE in patients with a
contraindication to CTPA (eg, renal insufficiency, true contrast allergy) or in those with an inconclusive
CTPA. It is also the initial test of choice for pregnant patients with suspected PE and a normal chest x-ray.
Educational objective:
Seafood allergy does not confer any increased risk of anaphylaxis from iodinated contrast media and does
not necessitate pretreatment with glucocorticoids and antihistamines.

References
• 2014 ESC guidelines on the diagnosis and management of acute pulmonary embolism.
• The relationship of radiocontrast, iodine, and seafood allergies: a medical myth exposed.
• Seafood allergy and radiocontrast media: are physicians propagating a myth?

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A 60-year-old man is hospitalized for acute pancreatitis. He reports swelling and tenderness in his jaw that
appeared 6 months ago. He has no medical problems, takes no medications, and does not use tobacco,
alcohol, or illicit drugs. There is no history of trauma to the abdomen. He does not have dry eyes or dry
mouth. On admission, ultrasound of the abdomen shows no gallstones. CT of the abdomen reveals a 3x4-
cm solid mass in the mid-portion of his pancreas with some surrounding edema. Chest CT reveals bilateral
hilar adenopathy. He is started on intravenous fluids, opioids for pain control, and nothing by mouth, and
his abdominal pain resolves.
On examination, he is well-nourished and appears comfortable. His blood pressure is 133/78 mm Hg,
pulse is 90∕mi∩, and oxygen saturation is 98% on room air. There is bilateral enlargement of his parotid
glands and some enlargement of his lacrimal glands. The remainder of his examination is within normal
limits.
Laboratory results are as follows:
Complete blood count
Hematocrit 38%
Mean corpuscular
88 fL
volume
Leukocytes 8,000/L
Neutrophils 60%
Eosinophils 8%
Lymphocytes 30%
Monocytes 2%

Serum chemistry and fasting lipid panels, Iiverfunction tests, urinalysis, and erythrocyte sedimentation rate
are all within normal limits. HIV testing is negative.
Which of the following conditions is the most likely cause of this patient’s presentation?
A. lgG4-related disorder
B. Large B cell lymphoma
C. Occult alcoholism
D. Pancreatic carcinoma
E. Sjogren’s syndrome

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A 60-year-old man is hospitalized for acute pancreatitis. He reports swelling and tenderness in his jaw that
appeared 6 months ago. He has no medical problems, takes no medications, and does not use tobacco,
alcohol, or illicit drugs. There is no history of trauma to the abdomen. He does not have dry eyes or dry
mouth. On admission, ultrasound of the abdomen shows no gallstones. CT of the abdomen reveals a 3x4-
cm solid mass in the mid-portion of his pancreas with some surrounding edema. Chest CT reveals bilateral
hilar adenopathy. He is started on intravenous fluids, opioids for pain control, and nothing by mouth, and
his abdominal pain resolves.
On examination, he is well-nourished and appears comfortable. His blood pressure is 133/78 mm Hg,
pulse is 90∕mi∩, and oxygen saturation is 98% on room air. There is bilateral enlargement of his parotid
glands and some enlargement of his lacrimal glands. The remainder of his examination is within normal
limits.
Laboratory results are as follows:
Complete blood count
Hematocrit 38%
Mean corpuscular
88 fL
volume
Leukocytes 8,000/L
Neutrophils 60%
Eosinophils 8%
Lymphocytes 30%
Monocytes 2%

Serum chemistry and fasting lipid panels, Iiverfunction tests, urinalysis, and erythrocyte sedimentation rate
are all within normal limits. HIV testing is negative.
Which of the following conditions is the most likely cause of this patient’s presentation?
A. lgG4-related disorder [44%]
B. Large B cell lymphoma [28%]
C. Occult alcoholism [2%]
D. Pancreatic carcinoma [8%]
E. Sjogren’s syndrome [16%]

Omitted
Correct answer
Ihi 44% 11 SecondS 01/04/2019
Answered correctly Time Spent Last Updated
A

Explanation

Clinical presentation of lgG4-RD

Clinical features

• Asymptomatic generalized Iymphadenopathy

• Type 1 autoimmune pancreatitis
• Sclerosingcholangitis
• Bilateral salivary and/or lacrimal gland involvement without
significant symptoms (eg, dry mouth & eyes)
• Thyroid disease (eg, RiedeIjSthyroiditis, fibrous
Hashimoto’s)
• Interstitial pneumonitis/pulmonary pseudotumors
• Tubulointerstitialnephritis
• Retroperitoneal fibrosis
Diagnosis

• Tissue infiltration of mainly lgG4-positive plasma cells

and small lymphocytes
• Elevated serum lgG4 levels
• Peripheral eosinophilia
Treatment

• Immunosuppressionwith glucocorticoids

This patient’s presentation of acute idiopathic pancreatitis, bilateral salivary and lacrimal gland
enlargement, and Iymphadenopathy suggests immunoglobulin lgG4-related disease (lgG4-RD), a fibro-
Inflammatory disease causing fibrosis and tumor-like swelling of affected organs. lgG4-RD is an emerging
disease of unknown etiology. It most commonly affects middle-aged men and should be suspected in
patients presenting with any of the following:
• Idiopathic pancreatitis
• Sclerosing cholangitis
• Bilateral salivary and/or lacrimal gland enlargement
Up to 40% of patients may have peripheral eosinophilia. Diagnosis is confirmed with tissue biopsy showing
lgG4-positive plasma cells and lymphocytes. Elevated serum lgG4 can be helpful as well although serum
levels can be normal. Patients typically respond to glucocorticoids.
(Choice B) Patients with lymphoma can present with a pancreatic mass and Iymphadenopathy, but they
often present with B symptoms (eg, fever, weight loss, night sweats) as well. This patient’s presentation of
acute pancreatitis and bilateral parotid and lacrimal gland swelling is not typical for lymphoma.
(Choice C) Alcoholism is sometimes associated with parotid gland enlargement in malnourished patients.
Alcoholism sufficient to cause acute pancreatitis is often associated with increased mean corpuscular
volume and elevated transaminases. This patient has none of these features and denies alcohol use.
(Choice D) Autoimmune pancreatitis is a prototypical form of lgG4-RD that can mimic pancreatic cancer
with a pancreatic mass and painless jaundice. However, there is usually no history of weight loss.
(Choice E) Patients with Sjogren’s syndrome usually complain of dry mouth and eyes, and/or arthralgia.
They also have antibodies to SSA and SSB proteins and do not have recurrent episodes of pancreatitis, as
is common in lgG4-RD. Patients with lgG4-related salivary and lacrimal gland disease do not have severe
sicca symptoms despite glandular enlargement.
Educational objective:
lgG4-related disorders can present with autoimmune pancreatitis, Iymphadenopathy, and bilateral salivary
and/or lacrimal gland enlargement. Diagnosis is confirmed by peripheral eosinophilia, elevated serum lgG4
levels, and tissue biopsy showing lgG4-positive plasma cells. Treatment involves glucocorticoids and
possibly azathioprine, mycophenolate mofetil, or rituximab for refractory disease.

References
• Comprehensive diagnostic criteria for lgG4-related disease (lgG4-RD), 2011.
• Outcome of patients with type 1 or 2 autoimmune pancreatitis.
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A 26-year-old primigravid woman at 36 weeks gestation is admitted to the hospital because of vaginal
bleeding and lower abdominal pain. Her other medical problems include selective IgA deficiency, allergic
rhinitis, and recurrent sinusitis. Her anti-lgA antibody screening is positive.
Evaluation reveals placental abruption. She is scheduled to undergo urgent cesarean section and requires
blood transfusion.
Which of the following should be administered to this patient?
A. Blood product desensitization therapy
B. Irradiated red cells
C. Leukoreduced red cells
D. Normal packed red cells
E. Washed red cells

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A 26-year-old primigravid woman at 36 weeks gestation is admitted to the hospital because of vaginal
bleeding and lower abdominal pain. Her other medical problems include selective IgA deficiency, allergic
rhinitis, and recurrent sinusitis. Her anti-lgA antibody screening is positive.
Evaluation reveals placental abruption. She is scheduled to undergo urgent cesarean section and requires
blood transfusion.
Which of the following should be administered to this patient?
A. Blood product desensitization therapy [3%]
B. Irradiated red cells [10%]
C. Leukoreduced red cells [19%]
D. Normal packed red cells [5%]
E. Washed red cells [61 %]

Omitted
Correct answer
Ihi 61% 8 SecondS 03/06/2019
Answered correctly X—√ Time Spent Last Updated
E

Explanation

Indications for specialized RBC treatments

• Bone marrow transplant (BMT) recipients

• Acquired or congenital cellular immunodeficiency.
Irradiated
• Blood components donated by first or second
degree relatives

• Chronically transfused patients

• CMV seronegative at-risk patients (e.g, AIDS,
Leukoreduced transplant patients)
• Potential transplant recipients
• Previous febrile nonhemolytic transfusion reaction

• IgA deficiency
• Complement-dependent autoimmune hemolytic
Washed anemia
• Continued allergic reactions (eg., hives) with red
cell transfusion despite antihistamine treatment

OUWorld

When red cells and plasma are separated from whole blood, small amounts of residual plasma remain in
the red cell concentrate. This plasma contains proteins, including IgA. IgA deficient patients develop
antibodies against IgA, which can react with IgA-containing donor products and induce an anaphylactic
reaction. To reduce this risk, red cells should be washed to remove as much of the plasma as possible.
(Choices B & C) Packed red cells can also be contaminated with leukocytes or leukocyte debris during
the separation process. This can induce febrile transfusion reactions and HLA alloimmunization.
Cytomegalovirus (CMV) also resides in the leukocytes and can be transmitted through transfusion. Thus,
Ieukoreduction with filters to remove WBCs reduces the risk of HLA alloimmunization, febrile nonhemolytic
transfusion reactions, and transmission of CMV.
However, Ieukoreduction alone cannot remove all lymphocyte components. But, gamma radiation can
essentially kill all the living lymphocytic components (T-lymphocytes). This is particularly useful in patients
at risk for transfusion-associated graft-versus-host disease, a rare, but fatal complication caused by the
engraftment and proliferation of donor T lymphocytes in the transfused recipient.
(Choice A) Desensitization to HLA and ABO antigens is indicated for certain patients who are difficult to
transfuse secondary to their broad immunogenicity.
Educational objective:
Patients with IgA deficiency should receive washed RBCs to remove as much plasma (containing IgA) as
possible. Patients at risk for transfusion-associated graft-versus-host disease (e.g., bone marrow
transplant) require irradiated RBCs. To reduce CMV transmission (e.g., patients waiting for transplant or
those with AIDS), patients should receive Ieukoreduced RBCs.

References
• Red blood cell transfusion in clinical practice.

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A 31-year-old farmer is hospitalized with a severe reaction to a bee (Hymenoptera) sting. He developed
respiratory distress requiring endotracheal intubation in the field. He was treated with epinephrine and
corticosteroids with rapid recovery. The patient has no prior similar incidents and no allergies. He is given
an epinephrine autoinjector and instructed on its use.
Which of the following is the most important part of discharge planning in this patient?
A. Antihistamine therapy
B. Prolonged course Ofcorticosteroids
C. Referral for immunotherapy
D. Serum total immunoglobulin E levels
E. Serum tryptase levels

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A 31-year-old farmer is hospitalized with a severe reaction to a bee (Hymenoptera) sting. He developed
respiratory distress requiring endotracheal intubation in the field. He was treated with epinephrine and
corticosteroids with rapid recovery. The patient has no prior similar incidents and no allergies. He is given
an epinephrine autoinjector and instructed on its use.
Which of the following is the most important part of discharge planning in this patient?
A. Antihistamine therapy [14%]
B. Prolonged course Ofcorticosteroids [9%]
<s C. Referral for immunotherapy [59%]
D. Serum total immunoglobulin E levels [8%]
E. Serum tryptase levels [7%]

Omitted
Correct answer
Ihi 59% 6 SecondS 03/06/2019
Answered correctly Time Spent Last Updated
C

Explanation

Hymenoptera stings can cause life-threatening anaphylaxis. One study found that up to 50% of patients
with anaphylaxis had no previous reaction or known allergy. Sixty percent of adult subjects with a history of
anaphylaxis from a previous sting will have anaphylaxis with a repeat sting. Venom immunotherapy (VIT)
reduces this risk to less than 5% by producing venom-specific immunoglobulin (Ig) G, changing T cell
responses, and decreasing venom-specific IgE. VIT is indicated in patients with a history of systemic
allergic reaction to an insect sting and evidence of venom-specific IgE, either by skin or in vitro test.
Discharge care should also include education on avoidance and an epinephrine autoinjector.
(Choices A and B) Acute medical treatment for anaphylaxis includes intramuscular epinephrine, H-1
receptor antagonists, corticosteroids, and supportive therapy for shock. However, these agents are not as
beneficial after resolution of the acute episode.
(Choice D) Serum IgE levels rise during the acute anaphylaxis stage and during the first few months of
immunotherapy before decreasing over the next 3-5 years of VIT. However, these levels do not reliably
predict protection from future anaphylaxis reactions.
(Choice E) Tryptase is a mediator released from mast cells and basophils. Serum tryptase levels may be
elevated in anaphylaxis but also in other mast cell disorders (eg, systemic mastocytosis) and
myelodysplastic syndromes. In addition, tryptase decreases after the anaphylaxis reaction and would not
be as useful to monitor as immunotherapy in this patient.
Educational objective:
Venom immunotherapy reduces the risk of anaphylaxis recurrence from 60% to 5%. It is indicated for
patients with history of systemic reaction to an insect sting and venom-specific immunoglobulin E.

References
• Chapter 4: Stinging insect allergy and venom immunotherapy.
• A controlled trial of immunotherapy in insect hypersensitivity
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A 45-year-old woman comes to the physician because of chronic nasal congestion. Her symptoms began
years ago, but they have worsened lately. She often has paroxysms of 10-12 sneezes, especially when
waking up in the morning. She also complains of chronic rhinorrhea and post-nasal drip, and she
occasionally has itchy eyes. She is unsure if there is a seasonal component to her symptoms, but they
may be worse in the Spring. She does not use aspirin and has not had any episodes of sinusitis. She has
tried chlorpheniramine in the past, but it made her drowsy. She does not smoke tobacco or drink alcohol
and she denies cocaine or other illicit drug use. She has mild hypertension but is in good health otherwise.
Her blood pressure is 134/84 mm Hg and pulse is 67∕min. Her nares are patent with a clear discharge.
There are no nasal polyps or septal deviation. The turbinates are pale and her conjunctivae appear
normal. Neck, pulmonary, and cardiovascular examinations are within normal limits.
Which of the following is the best next step in this patient's management?
A. Leukotriene inhibitor
B. Nasal corticosteroid spray
C. Nasal decongestant spray
D. Oral decongestant
E. Oral non-sedating antihistamine

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A 45-year-old woman comes to the physician because of chronic nasal congestion. Her symptoms began
years ago, but they have worsened lately. She often has paroxysms of 10-12 sneezes, especially when
waking up in the morning. She also complains of chronic rhinorrhea and post-nasal drip, and she
occasionally has itchy eyes. She is unsure if there is a seasonal component to her symptoms, but they
may be worse in the Spring. She does not use aspirin and has not had any episodes of sinusitis. She has
tried chlorpheniramine in the past, but it made her drowsy. She does not smoke tobacco or drink alcohol
and she denies cocaine or other illicit drug use. She has mild hypertension but is in good health otherwise.
Her blood pressure is 134/84 mm Hg and pulse is 67∕min. Her nares are patent with a clear discharge.
There are no nasal polyps or septal deviation. The turbinates are pale and her conjunctivae appear
normal. Neck, pulmonary, and cardiovascular examinations are within normal limits.
Which of the following is the best next step in this patient's management?
A. Leukotriene inhibitor [3%]
B. Nasal corticosteroid spray [70%]
C. Nasal decongestant spray [4%]
D. Oral decongestant [1%]
E. Oral non-sedating antihistamine [20%]

Omitted
Correct answer
Ihi 7o% 11 SecondS 04/23/2019
Answered correctly Time Spent Last Updated
B

Explanation

This patient's presentation is consistent with typical allergic rhinitis, which affects a large percentage of the
normal population. For many individuals, the symptoms are disturbing; they can lower quality of life and
impair sleep. Patients typically present with sneezing, watery nasal discharge, and pale turbinates.
Although most patients have seasonal exacerbations, this is not always the case. Clinical history and
examination are sufficient to establish a diagnosis, and skin testing or other tests to detect allergens are not
necessary at this time. Randomized controlled trials have demonstrated that nasal corticosteroids are
superior to all other listed modalities in relieving symptoms and improving quality of life.
(Choice A) Leukotriene antagonists may be of some benefit, but they are not as effective as nasal
corticosteroids.
(Choice C) Nasal decongestants are effective for short-term relief of symptoms but should be avoided due
to desensitization and rebound symptoms, which can lead to overuse and rhinitis medicamentosa.
(Choice D) Oral decongestants can be effective for short periods to decrease rhinorrhea and nasal
congestion, but they have no impact on sneezing and itching eyes. In addition, they can increase blood
pressure.
(Choice E) Sedating and non-sedating anti-histamines often relieve symptoms and are commonly used in
patients with paroxysmal and mild symptoms. However, they are inferior to nasal steroids in patients with
persistent, moderate-to-severe symptoms (such as this patient).
Educational objective:
Nasal corticosteroids are the most effective and best tolerated treatment for typical allergic rhinitis.

References
• Comparison of nasal glucocorticoid, antileukotriene and a combination antileukotriene and
antihistamine in the treatment of seasonal allergic rhinitis.
• Intranasal corticosteroids versus H1 receptor antagonists in allergic rhinitis: systematic review of
randomized controlled trials.
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 A 36-year-old man comes to the physician with decreased appetite, right-sided facial pain, and fever. He
also complains of scant nasal discharge. He has had 3 episodes of sinusitis over the last year that
responded well to amoxicillin-clavulanate. Six months ago, he was hospitalized with pneumonia and
treated with intravenous antibiotics. His past medical history is unremarkable. He does not use tobacco,
alcohol, or illicit drugs. He lives with his girlfriend and has 1 child.
His temperature is 38.3o C (101o F), blood pressure is 144/90 mm Hg, and pulse is 90∕min. The right
maxillary sinus is tender to palpation. He has 3 small, mobile submandibular nodes. Cardiopulmonary
examination is within normal limits. Both upper extremities and the chest have areas of hypopigmented
skin.
Laboratory results are as follows:
400,000/
Platelets
μL
Leukocytes 14,000∕μL
Creatinine 0.8 mg/dL

Urinalysis is unremarkable. Rapid HIV test is negative.

Which of the following conditions is the most likely diagnosis?
A. Common variable immunodeficiency
B. Cysticfibrosis
C. Granulomatosis with polyangiitis (Wegener's granulomatosis)
D. Myeloperoxidase deficiency
E. Primary ciliary dyskinesia

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A 36-year-old man comes to the physician with decreased appetite, right-sided facial pain, and fever. He
also complains of scant nasal discharge. He has had 3 episodes of sinusitis over the last year that
responded well to amoxicillin-clavulanate. Six months ago, he was hospitalized with pneumonia and
treated with intravenous antibiotics. His past medical history is unremarkable. He does not use tobacco,
alcohol, or illicit drugs. He lives with his girlfriend and has 1 child.
His temperature is 38.3o C (101o F), blood pressure is 144/90 mm Hg, and pulse is 90∕min. The right
maxillary sinus is tender to palpation. He has 3 small, mobile submandibular nodes. Cardiopulmonary
examination is within normal limits. Both upper extremities and the chest have areas of hypopigmented
skin.
Laboratory results are as follows:
400,000/
Platelets
μL
Leukocytes 14,000∕μL
Creatinine 0.8 mg/dL

Urinalysis is unremarkable. Rapid HIV test is negative.

Which of the following conditions is the most likely diagnosis?
A. Common variable immunodeficiency [59%]
B. Cysticfibrosis [6%]
C. Granulomatosis with polyangiitis (Wegener's granulomatosis) [9%]
D. Myeloperoxidase deficiency [13%]
E. Primary ciliary dyskinesia [10%]

Omitted
Correct answer
Ihi 59% 16 SecondS 03/06/2019
Answered correctly Time Spent Last Updated
A

Explanation

Clinical features of CVID

• Recurrent infections (eg, rhinosinusitis,

pneumonia, conjunctivitis)
• Chronic lung disease (eg, bronchiectasis)
• Autoimmune disorders (eg, vitiligo,
rheumatoid arthritis, thyroid disorders)
Clinical
manifestations • Gastrointestinal disorders (eg, irritable
bowel disease, malabsorption)
• Granulomatous disease in lymphoid or
solid organs
• Increased risk of malignancy
(eg, lymphoma, gastric cancers)

• Extremely persistent low IgG levels

Diagnosis • Low IgA and/or IgM levels
(requires all of • No response to vaccinations
these criteria)
• Exclusion of other immunodeficiency
disorders

• Immunoglobulin replacement therapy

Treatment and
• Age-appropriate cancer screening
management
• Monitoring for lymphoma

© UWorId

This patient's vitiligo, recurrent sinusitis, and previous history of pneumonia suggest likely common variable
immunodeficiency (CVID). CVID is the most common immunodeficiency disorder affecting children and
young adults and is due to impaired B cell differentiation and immunoglobulin (IgG, IgA, and/or IgM)
production. Patients typically present before age 20 (delayed diagnosis is common) with variable clinical
manifestations such as infections (eg, rhinosinusitis and pneumonia) and chronic lung disease. Nearly
75% of patients have a history of pneumonia that is usually due to Streptococcus pneumoniae,
Mycoplasma, or Haemophilus influenzae.
Diagnosis is suggested by low immunoglobulin levels, poor response to vaccinations, and exclusion
of other disorders. Treatment includes antibiotics for specific infections and immunoglobulin replacement
therapy. Immunoglobulin therapy can improve CVID prognosis by reducing the number of infections and
hospitalizations, reducing antibiotic use, and possibly decreasing progression of chronic lung disease.
CVID patients are more likely to develop malignancies and require age-appropriate cancer screening,
monitoring for lymphoma, or possible bronchoscopy for unresolved pulmonary findings despite treatment.
Chronic lung disease and malignancies are the most common causes of death.
(Choice B) Adult cystic fibrosis generally presents with a milder form of the disease that consists of mild
respiratory infections, gastrointestinal disease (eg, recurrent pancreatitis, biliary cirrhosis, and portal
hypertension), or abnormal reproductive function. This patient’s absence of a long history of lung infections
or gastrointestinal or fertility problems makes this less likely.
(Choice C) Patients with granulomatosis with polyangiitis (Wegener's) typically present with pulmonary
and renal disease, history of oronasal ulcers, and persistent rhinorrhea (leading to bony destruction and
"saddle-nose" deformity). In addition, the urinalysis is usually abnormal (eg, hematuria and non-nephrotic-
range proteinuria).
(Choice D) Patients with myeloperoxidase deficiency may be asymptomatic or present with recurrent
Candida infections (mucocutaneous, meningeal, osteomyelitis, and sepsis). However, this patient has no
prior history of Candida infections.
(Choice E) Primary ciliary dyskinesia typically presents with nasal polyposis, otitis, recurrent respiratory
infections, situs inversus (present in 50% of patients), and infertility. This patient is not infertile, which
makes primary ciliary dyskinesia less likely.
Educational objective:
Common variable immunodeficiency is the most common immunodeficiency disorder affecting children and
young adults and is caused by impaired B cell differentiation and immunoglobulin production (IgG, IgA,
and/or IgM). Patients typically present with respiratory infections, chronic lung disease, and increased risk
of malignancies. Management includes immunoglobulin replacement therapy and vigorous cancer
screening.

References
• The spectrum of disease manifestations in patients with common variable immunodeficiency disorders
and partial antibody deficiency in a university hospital.
• Review of gastric cancer risk factors in patients with common variable immunodeficiency disorders,
resulting in a proposal for a surveillance programme.

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