Antibody Disorders (CVID and Selective IgA)

Question 1

What are some indications of immunodefiency?

Answer 1

Recurrent infections

• age of onset
• too many
• too severe
• long lasting
• unusual complications (scarrig, abscess, DIC)
• fail to resolve with standard therapy

Question 2

What are the classical signs of diGeorge Syndrome?

Answer 2

Congenital Heart Defects

Absence of thymic shadow on CXR

Hypocalcemia

• absent thymus and parathyroid
  22q11. 2 deletion, failure to develop 3rd and 4th phagyngela pouches

Question 3

Does Primary immunedef. affect humoral or cellular more often?

Answer 3

PID is more often humoral (1 in 10K people)

Secondary Immunodef. is more often cellular

Question 4

What is the memory antibody?

Answer 4

IgG

Question 5

What antibody crosses the placenta?

Answer 5

IgG

Question 6

What antibody fixe complement?

Answer 6

IgM and IgG

Question 7

What important signs of ID should we look for on H &P?

Answer 7

Growth and development

-immunization history including adverse events (Diarrhea following rotavirus)

-family history

Question 8

What are values for Lymphopenia on CBC?

Answer 8

If under age 5 lymphopenia is <2500

if older lymphopenia is <1500

Question 9

What diagnostic info would exlude almost all hereditary compement immunodeficiencies?

Answer 9

A normal CH50

Question 10

What can show you the absolute and percentages of T,B and NK cells?

Answer 10

Flow cytometry

Question 11

Patient with recurrent sinus infections has labs done.

Come back and IgA is abest (<7mg/dL), but IgM and IgG are normal

He is mid 30s

Whats your dx?

Answer 11

Selective IgA defiency.

Unkown cause

-most common primary ID

Think A’s

Majority Asymptomatic, Can see Airway and GI infections, Autoimmune disease, Atopy, Anaphylaxis to IgA-containing products

Question 12

What are the treatments for selective IgA?

Answer 12

Antibiotics including acute and phophylatic therpay (low doses of antibiotic daily or 3 time weekly

• treat allergies with anti-histamines and nasal corticosteroids
• supportive care of associated symptoms like diarrhea, and control of autoimmune disease

Question 13

When can a dx for Selective IgA be made?

Answer 13

Only after puberty, because IgA doesn’t reach adult levels until then.

Question 14

What are pulmonary/sinus/airway infections a sign of?

Answer 14

Humoral deficiency

Question 15

In pre-vaccination antibody levels vs. post-vaccination antiboy levls what would you expect for a normal adult?

Answer 15

About a 70% increase in antibody production.

Example was in humoral defiency, pre-vac to pneumococcal was 6/23 and post was 7/23. This is not good

Question 16

What are some causes of Bronchiectasis?

Answer 16

Repeated pulmonary infections causes scarring of the airways. The scarring of the airways cause them to get bigger, and it makes it so the cilia cannot reach the mucus anymore and you end up getting more infection because the mucus justs sits there.

-also seen in CF becuase they have recurrent infections

Question 17

What are the diagnostics for CVID?

Answer 17

Low levels of Ig with insufficient response to vaccinations

• low IgG with low IgA and/or IgM
• insufficient response to vaccines
• recurrent infections, some sever
• severity is mild to severe

1 in 50K affected

chilldhood or adult (IgG reach adult levels around age 6)

• recurrent sinalpulmonary infections
• can present with autoimmune diseases (part of immune system is underactive and cant fight infection, and other part is trying to compensate and is over active and causes auto immunity

Question 18

What is the treatment of choice of CVID?

Answer 18

IVIg

• given typically every 4 weeks
• also exists are sub cutaenous Ig which is infused weekly