Common variable immunodeficiency - Revision history

Aidan at 20:31, 6 October 2025

2025-10-06T20:31:45Z

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 *Characterized by decreased IgG immunoglobulins, with or without decreases in IgA and IgM, and decreased response to vaccination
-*Must exclude other causes of hypogammaglobulinemia
+*Must exclude other causes of [[hypogammaglobulinemia]]
 ===Epidemiology===
@@ Line 63: / Line 59: @@
 |}
 ==Management==

Aidan: Text replacement - " species]]" to "]]"

2022-01-27T01:04:43Z

Text replacement - " species]]" to "]]"

← Older revision		Revision as of 01:04, 27 January 2022
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	*Diarrhea is common (21 to 57%)		*Diarrhea is common (21 to 57%)
	**[[Giardia lamblia]] is the most common cause of chronic diarrhea		**[[Giardia lamblia]] is the most common cause of chronic diarrhea
	**Others include [[Cryptosporidium parvum]], [[CMV]], [[Salmonella ~~species~~]], [[Clostridium difficile]], and [[Campylobacter jejuni]]		**Others include [[Cryptosporidium parvum]], [[CMV]], [[Salmonella]], [[Clostridium difficile]], and [[Campylobacter jejuni]]
	*Can also develop [[sarcoidosis]] (8 to 22%), with granulomatous changes occurring years before the hypogammaglobulinemia		*Can also develop [[sarcoidosis]] (8 to 22%), with granulomatous changes occurring years before the hypogammaglobulinemia
	**Mainly in lung, lymph nodes, or liver		**Mainly in lung, lymph nodes, or liver

Aidan: /* Clinical Manifestations */

2020-09-17T13:15:34Z

Clinical Manifestations

← Older revision		Revision as of 13:15, 17 September 2020
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	*Must exclude other causes of hypogammaglobulinemia		*Must exclude other causes of hypogammaglobulinemia

	=== Classification ===		===Classification===

	*		*

	===Epidemiology===		===Epidemiology===
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	*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 species]], [[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)
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	\|}		\|}

	== 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
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	\|}		\|}

	== 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]

Aidan at 01:16, 14 September 2020

2020-09-14T01:16:34Z

@@ Line 1: / Line 1: @@
-== Background ==
+==Background==
-* Characterized by decreased IgG immunoglobulins, with or without decreases in IgA and IgM, and decreased response to vaccination
+*Characterized by decreased IgG immunoglobulins, with or without decreases in IgA and IgM, and decreased response to vaccination
-=== Epidemiology ===
+=== Classification ===

Aidan: 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..."

2020-09-14T00:48:47Z

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..."

New page

== Background ==

* Characterized by decreased IgG immunoglobulins, with or without decreases in IgA and IgM, and decreased response to vaccination
** Diagnosis of exclusion

=== Epidemiology ===

* Affects up to 1 in 25,000 people