Common variable immunodeficiency: Difference between revisions

From IDWiki
No edit summary
m (Text replacement - " species]]" to "]]")
 
(One intermediate revision by the same user not shown)
Line 4: Line 4:
 
*Must exclude other causes of hypogammaglobulinemia
  
*Must exclude other causes of hypogammaglobulinemia
   
−
=== Classification ===
 +
===Classification===
   
−
*
+
*
   
 
===Epidemiology===
  
===Epidemiology===
Line 12: Line 12:
 
*Affects up to 1 in 25,000 people
  
*Affects up to 1 in 25,000 people
   
−
== Clinical Manifestations ==
 +
==Clinical Manifestations==
   
−
* Wide spectrum of manifestations
 +
*Wide spectrum of manifestations
−
* Most common presentation is recurrent sinopulmonary infections
 +
*Most common presentation is recurrent sinopulmonary infections
−
** Especially [[Streptococcus pneumoniae]], [[Haemophilus influenzae]], and [[Mycoplasma species]]
 +
**Especially [[Streptococcus pneumoniae]] and [[Haemophilus influenzae]]
  +
**Severe or chronic [[Mycoplasma species|Mycoplasma]] infections
⚫
** [[Empyema]], [[bacteremia]], [[meningitis]], and [[osteomyelitis]]
  
  +
**Severe or chronic [[Enterovirus]] infections, including meningitis
⚫
* Can eventually develop chronic lung disease including obstruction, restriction, and bronchiectasis
  
  ⚫
**[[Empyema]], [[bacteremia]], [[meningitis]], and [[osteomyelitis]]
⚫
* Diarrhea is common (21 to 57%)
  
  ⚫
*Can eventually develop chronic lung disease including obstruction, restriction, and bronchiectasis
⚫
** [[Giardia lamblia]] is the most common cause of chronic diarrhea
  
  ⚫
*Diarrhea is common (21 to 57%)
⚫
** Others include [[Cryptosporidium parvum]], [[CMV]], [[Salmonella species]], [[Clostridium difficile]], and [[Campylobacter jejuni]]
  
  ⚫
**[[Giardia lamblia]] is the most common cause of chronic diarrhea
⚫
* Can also develop [[sarcoidosis]] (8 to 22%), with granulomatous changes occurring years before the hypogammaglobulinemia
  
  ⚫
**Others include [[Cryptosporidium parvum]], [[CMV]], [[Salmonella]], [[Clostridium difficile]], and [[Campylobacter jejuni]]
⚫
** Mainly in lung, lymph nodes, or liver
  
  ⚫
*Can also develop [[sarcoidosis]] (8 to 22%), with granulomatous changes occurring years before the hypogammaglobulinemia
⚫
** Portends a higher risk of [[immune-mediated thrombocytopenia]] and [[autoimmune hemolytic anemia]]
  
  ⚫
**Mainly in lung, lymph nodes, or liver
⚫
* Autoimmune phenomena include [[immune-mediated thrombocytopenia]], [[autoimmune hemolytic anemia]], [[Evans syndrome]], and [[autoimmune neutropenia]]
  
  ⚫
**Portends a higher risk of [[immune-mediated thrombocytopenia]] and [[autoimmune hemolytic anemia]]
⚫
* Higher rates of malignancies, primarily [[non-Hodgkin lymphoma]] and [[stomach cancer]]
  
  ⚫
*Autoimmune phenomena include [[immune-mediated thrombocytopenia]], [[autoimmune hemolytic anemia]], [[Evans syndrome]], and [[autoimmune neutropenia]]
⚫
** The latter may be related to infection with [[Helicobacter pylori]]
  
  ⚫
*Higher rates of malignancies, primarily [[non-Hodgkin lymphoma]] and [[stomach cancer]]
  ⚫
**The latter may be related to infection with [[Helicobacter pylori]]
   
−
=== Prognosis ===
 +
===Prognosis===
   
−
* Decreased life expectancy
 +
*Decreased life expectancy
−
* Median survival 13.7 years with granulomatous changes or 28.8 years without
 +
*Median survival 13.7 years with granulomatous changes or 28.8 years without
   
−
== Investigations ==
 +
==Investigations==
 
{| class="wikitable"
  
{| class="wikitable"
 
!Serum IgG (mg/dL)
  
!Serum IgG (mg/dL)
Line 61: Line 63:
 
|}
  
|}
   
−
== Management ==
 +
==Management==
   
−
=== Immune Globulin Replacement ===
 +
===Immune Globulin Replacement===
   
−
* The mainstay of therapy if IVIg 400 to 600 mg/kg IV monthly, usually given every 3 to 4 weeks
 +
*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
 +
**Can be given subcutaneously, divided every 1 to 2 weeks, if IV access is difficult
−
* Target trough depends on baseline IgG level
 +
*Target trough depends on baseline IgG level
−
** <100 mg/dL: target trough 600 mg/dL
 +
**<100 mg/dL: target trough 600 mg/dL
−
** 300 mg/dl: target trough 900 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
 +
*Although many are IgA deficient, there are low rates of anti-IgA antibodies in CVID, so IVIg is generally safe
   
−
=== Follow-Up ===
 +
===Follow-Up===
 
{| class="wikitable"
  
{| class="wikitable"
 
!Patients
  
!Patients
Line 110: Line 112:
 
|}
  
|}
   
−
== Further Reading ==
 +
==Further Reading==
   
−
* 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]
 +
*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]

Latest revision as of 21:04, 26 January 2022

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

Retrieved from "https://idwiki.org/index.php?title=Common_variable_immunodeficiency&oldid=8485"
Powered by MediaWiki
Powered by Semantic MediaWiki