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PID Flashcards

(4 cards)

1
Q

Name at least 3 complications of CVID

A
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
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2
Q

True or False

CVID is the most frequent symptomatic antibody deficiency

A

True

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3
Q

Describe inheritance pattern of CVID and the most common genetic defect

A
  • 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
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4
Q

Discuss management of CVID

A
  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
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