Common variable immunodeficiency: Difference between revisions

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(Created page with "== Background == * Characterized by decreased IgG immunoglobulins, with or without decreases in IgA and IgM, and decreased response to vaccination ** Diagnosis of exclusion...")
 
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== Background ==
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==Background==
   
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* Characterized by decreased IgG immunoglobulins, with or without decreases in IgA and IgM, and decreased response to vaccination
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*Characterized by decreased IgG immunoglobulins, with or without decreases in IgA and IgM, and decreased response to vaccination
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*Must exclude other causes of hypogammaglobulinemia
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** Diagnosis of exclusion
  
   
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=== Epidemiology ===
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=== Classification ===
   
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*
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* Affects up to 1 in 25,000 people
  
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===Epidemiology===
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*Affects up to 1 in 25,000 people
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== Clinical Manifestations ==
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* Wide spectrum of manifestations
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* Most common presentation is recurrent sinopulmonary infections
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** Especially [[Streptococcus pneumoniae]], [[Haemophilus influenzae]], and [[Mycoplasma species]]
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** [[Empyema]], [[bacteremia]], [[meningitis]], and [[osteomyelitis]]
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* Can eventually develop chronic lung disease including obstruction, restriction, and bronchiectasis
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* Diarrhea is common (21 to 57%)
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** [[Giardia lamblia]] is the most common cause of chronic diarrhea
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** Others include [[Cryptosporidium parvum]], [[CMV]], [[Salmonella species]], [[Clostridium difficile]], and [[Campylobacter jejuni]]
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* Can also develop [[sarcoidosis]] (8 to 22%), with granulomatous changes occurring years before the hypogammaglobulinemia
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** Mainly in lung, lymph nodes, or liver
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** Portends a higher risk of [[immune-mediated thrombocytopenia]] and [[autoimmune hemolytic anemia]]
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* Autoimmune phenomena include [[immune-mediated thrombocytopenia]], [[autoimmune hemolytic anemia]], [[Evans syndrome]], and [[autoimmune neutropenia]]
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* Higher rates of malignancies, primarily [[non-Hodgkin lymphoma]] and [[stomach cancer]]
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** The latter may be related to infection with [[Helicobacter pylori]]
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=== Prognosis ===
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* Decreased life expectancy
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* Median survival 13.7 years with granulomatous changes or 28.8 years without
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== Investigations ==
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{| class="wikitable"
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!Serum IgG (mg/dL)
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!Recommendation
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|-
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|<150
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|repeat immunoglobulins to confirm
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|-
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|150-250
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|repeat immunoglobulins to confirm
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consider tetanus and diphtheria titres
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consider pneumococcal vaccine titres pre- and post-vaccination
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|-
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|250-450
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|repeat immunoglobulins to confirm
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check tetanus and diphtheria titres
  +
  +
check pneumococcal vaccine titres pre- and post-vaccination
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|-
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|450-600
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|repeat immunoglobulins to confirm
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check tetanus, diphtheria, MMR, and VZV titres
  +
  +
check pneumococcal vaccine titres pre- and post-vaccination
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|}
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== Management ==
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=== Immune Globulin Replacement ===
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* The mainstay of therapy if IVIg 400 to 600 mg/kg IV monthly, usually given every 3 to 4 weeks
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** Can be given subcutaneously, divided every 1 to 2 weeks, if IV access is difficult
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* Target trough depends on baseline IgG level
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** <100 mg/dL: target trough 600 mg/dL
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** 300 mg/dl: target trough 900 mg/dL
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* Although many are IgA deficient, there are low rates of anti-IgA antibodies in CVID, so IVIg is generally safe
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=== Follow-Up ===
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{| class="wikitable"
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!Patients
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!Assessment
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!Interval
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|-
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| rowspan="5" |all
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|History and physical
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|annually
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|-
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|CBC, liver and renal panel, albumin
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|annually
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|-
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|spirometry
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|annually
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|-
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|serum trough IgG ± IgA and IgM
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|6-12 months
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|-
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|chest x-ray
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|on referral
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|-
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| rowspan="2" |lung disease
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|high-resolution CT chest
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|3-4 years or after change of therapy
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|-
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|lung function with DLCO
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|annually
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|-
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|GI complications
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|upper and/or lower endoscopy
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|as required
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|-
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|malabsorption or loss of height
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|bone density and micronutrient assessment
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|as required
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|}
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== Further Reading ==
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* How I treat common variable immune deficiency. ''Blood''. 2010;116(1):7-15. doi: [https://doi.org/10.1182/blood-2010-01-254417 10.1182/blood-2010-01-254417]

Revision as of 21:16, 13 September 2020

Background

  • Characterized by decreased IgG immunoglobulins, with or without decreases in IgA and IgM, and decreased response to vaccination
  • Must exclude other causes of hypogammaglobulinemia

Classification

Epidemiology

  • Affects up to 1 in 25,000 people

Clinical Manifestations

Prognosis

  • Decreased life expectancy
  • Median survival 13.7 years with granulomatous changes or 28.8 years without

Investigations

Serum IgG (mg/dL) Recommendation
<150 repeat immunoglobulins to confirm
150-250 repeat immunoglobulins to confirm

consider tetanus and diphtheria titres

consider pneumococcal vaccine titres pre- and post-vaccination

250-450 repeat immunoglobulins to confirm

check tetanus and diphtheria titres

check pneumococcal vaccine titres pre- and post-vaccination

450-600 repeat immunoglobulins to confirm

check tetanus, diphtheria, MMR, and VZV titres

check pneumococcal vaccine titres pre- and post-vaccination

Management

Immune Globulin Replacement

  • The mainstay of therapy if IVIg 400 to 600 mg/kg IV monthly, usually given every 3 to 4 weeks
    • Can be given subcutaneously, divided every 1 to 2 weeks, if IV access is difficult
  • Target trough depends on baseline IgG level
    • <100 mg/dL: target trough 600 mg/dL
    • 300 mg/dl: target trough 900 mg/dL
  • Although many are IgA deficient, there are low rates of anti-IgA antibodies in CVID, so IVIg is generally safe

Follow-Up

Patients Assessment Interval
all History and physical annually
CBC, liver and renal panel, albumin annually
spirometry annually
serum trough IgG ± IgA and IgM 6-12 months
chest x-ray on referral
lung disease high-resolution CT chest 3-4 years or after change of therapy
lung function with DLCO annually
GI complications upper and/or lower endoscopy as required
malabsorption or loss of height bone density and micronutrient assessment as required

Further Reading

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