PID

Question 1

Name at least 3 complications of CVID

Answer 1

Autoimmunity – AIHA, ITP, RA, autoimmune thyroiditis, cytopenias (neutropenia, thrombocytopenia), psoriasis
Cancer – non-hodgkin lymphoma
Bronchiectasis 
IBD-like disease
Allergies – AR, asthma
Granulomatous disease
Lymphadenopathy
Splenomegaly

Question 2

True or False

CVID is the most frequent symptomatic antibody deficiency

Answer 2

True

Question 3

Describe inheritance pattern of CVID and the most common genetic defect

Answer 3

• Majority of CVID cases are sporadic
• Monogenic causes identified in 2-10% of patients
• 5-25% of patients have (+) family history
• AR or AD with variable penetrance
• Most common genetic mutation is in TACI gene

Question 4

Discuss management of CVID

Answer 4

1. Immune globulin replacement therapy
   - IVIG or SCIG
   - Reduces infections, antibiotic use, & hospitalizations
   - May slow progression of chronic lung disease
2. Treat infections
3. Antibiotic prophylaxis
   - Consider in those with frequent infections +/- ongoing l lung disease
   Chronic lung disease (e.g. bronchiectasis, reduced lung function)  reduce exacerbations
4. Surveillance