ID: Primary immunodeficiencies

Question 1

How do patients with a selective IgA deficiency usually present?

Answer 1

Recurrent sinopulmonary infections, GI infections, inflammatory bowel disease, or celiac disease; and have increased frequency of autoimmune or allergic disorders

Question 2

How do patients with CVID present?

Answer 2

hypogammaglobulinemia
recurrent respiratory infections
GI tract involvement with malabsorption or chronic diarrhea

Question 3

How is CVID diagnosed?

Answer 3

It is diagnosed by low levels of total IgG and IgA or IgM as well as a poor antibody response to vaccines

Question 4

When should you screen for complement deficiency and what test do you use?

Answer 4

Screen for complement deficiency when patient presents with recurrent bloodstream infection with encapsulated bacteria or invasive meningococcal or gonococcal disease

Screen by using the total hemolytic complement activity; if total is normal then check the alternative pathway. If either are abnormal, then look at specific complement concentrations

Question 5

What should patients with complement deficiency be vaccinated for?

Answer 5

meningococcal and pneumococcal

Question 6

What is the difference between a late complement deficiency and a classic complement deficiency?

Answer 6

Late complement deficiency (C5-C9): recurrent, invasive meningococcal or gonococcal infections

Classical compliment deficiency (C1-C4): results in rheumatological disease such as SLE, vasculitis, dermatomyositis, or scleroderma