Allergy/Immunology

Question 1

Describe the presentation and treatment of anaphylaxis.

Answer 1

• presents with a urticarial rash, respiratory distress, and hypotension
• treat with airway management, epinephrine, antihistamines, and glucocorticoids

Question 2

Describe the etiology, presentation, diagnosis, and treatment of angioedema.

Answer 2

• most often due to a C1 esterase inhibitor deficiency
• presents as sudden swelling in the absence of pruritus and urticaria, often after starting an ACEi or the occurrence of minor trauma
• best initial test is for low C2 and C4 but most accurate is for low C1 esterase inhibitor activity
• treat acutely with airway maintenance, C1 esterase inhibitor concentrate, the kallikrein inhibitor ecallantide, or the bradykinin antagonist icatibant
• use antifibinolytics, androgens, or C1 esterase inhibitor infusions for maintenance therapy

Question 3

What is the treatment for urticaria?

Answer 3

antihistamines or leukotriene receptor antagonists

Question 4

Describe the presentation and treatment of allergic rhinitis.

Answer 4

• presents with recurrent episodes of watery and itchy eyes, inflamed and boggy nasal mucosa, nasal polyps, and pale or violaceous turbinates
• treat with avoidance, intranasal corticosteroids, antihistamines, anticholinergics, and desensitization

Question 5

Describe the pathophysiology, presentation, diagnosis, and treatment of common variable immunodeficiency.

Answer 5

• a defect in B cell differentiation leads to low levels of plasma cells and all Ig isotypes
• presents with recurrent sinopulmonary infections, seronegative autoimmune disease, and normal amounts of lymphoid tissue
• diagnosis is based on decreased response by B cells to antigen stimulation
• treat with scheduled IVIG infusions

Question 6

Describe the pathophysiology, presentation, and treatment of X-linked agammaglobulinemia.

Answer 6

• an X-linked defect prevents maturation of B cells
• presents with recurrent sinopulmonary bacterial and enteroviral infections with absent lymphoid tissue
• treat with maintenance IVIG

Question 7

Describe the pathophysiology, presentation, diagnosis, and treatment of SCID.

Answer 7

• an IL-2R or adenosine deaminase deficiency leads to a deficiency of both B and T cells
• presents with recurrent sinopulmonary infections and many AIDS defining infections (candida, PCP, varicella, etc.)
• diagnosis is based on diminished TRECs, absent thymic shadow, and no germinal centers on lymph node biopsy
• treat with antimicrobial prophylaxis and IVIG until a bone marrow transplant can be performed

Question 8

Describe the presentation and treatment of IgA deficiency.

Answer 8

• presents with the 4 A’s: airway and GI infection, atopy, autoimmune disease, and anaphylaxis to blood products
• IVIG does not help and infections are treated as they areise

Question 9

Describe the pathophysiology, presentation, and treatment of hyper-IgE syndrome

Answer 9

• it is a disease caused by impaired neutrophil recruitment
• presents with FATED: coarse Facies, Abscesses (staph), retained primary Teeth, increased IgE, and Dermatologic problems in the form of eczema
• consider prophylactic antibiotics for Staph infections including dicloxacillin or cephalexin

Question 10

What are the features of Wiskott-Aldrich syndrome?

Answer 10

WATER

• wiskott
• aldrich
• thrombocytopenia
• eczema
• recurrent infection

Question 11

Describe the pathophysiology, presentation, diagnosis, and treatment of chronic granulomatous disease.

Answer 11

• a deficiency of NADPH oxidase prevents formation of reactive oxygen species like superoxide, leaving individuals susceptible to catalase positive organisms
• presents with recurrent infection by Staphylococcus, Burkholderia, Nocardia, and Aspergillus
• diagnosis is based on a negative nitroblue tetrazolium test and abnormal dihydrorhodamine test
• patients need bactrim and itraconazole prophylaxis