PID Flashcards
(4 cards)
Name at least 3 complications of CVID
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
True or False
CVID is the most frequent symptomatic antibody deficiency
True
Describe inheritance pattern of CVID and the most common genetic defect
- 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
Discuss management of CVID
- Immune globulin replacement therapy
- IVIG or SCIG
- Reduces infections, antibiotic use, & hospitalizations
- May slow progression of chronic lung disease - Treat infections
- Antibiotic prophylaxis
- Consider in those with frequent infections +/- ongoing l lung disease
Chronic lung disease (e.g. bronchiectasis, reduced lung function) reduce exacerbations - Surveillance