Common variable immunodeficiency - Revision history

Michael: /* General */

2016-03-24T22:14:51Z

General

← Older revision		Revision as of 22:14, 24 March 2016
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	==General==		==General==
	Features:<ref name=pmid20629103>{{cite journal \|author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L \|title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease \|journal=Inflamm Bowel Dis \|volume= \|issue= \|pages= \|year=2010 \|month=July \|pmid=20629103 \|doi=10.1002/ibd.21376 \|url=}}</ref>
	*Recurrent sinus and pulmonary infections.		*Recurrent sinus and pulmonary infections.
	**Pneumonia seen in ~1/3 of ~2200 patients.<ref name=pmid24582312>{{Cite journal \| last1 = Gathmann \| first1 = B. \| last2 = Mahlaoui \| first2 = N. \| last3 = Gérard \| first3 = L. \| last4 = Oksenhendler \| first4 = E. \| last5 = Warnatz \| first5 = K. \| last6 = Schulze \| first6 = I. \| last7 = Kindle \| first7 = G. \| last8 = Kuijpers \| first8 = TW. \| last9 = van Beem \| first9 = RT. \| title = Clinical picture and treatment of 2212 patients with common variable immunodeficiency. \| journal = J Allergy Clin Immunol \| volume = 134 \| issue = 1 \| pages = 116-26 \| month = Jul \| year = 2014 \| doi = 10.1016/j.jaci.2013.12.1077 \| PMID = 24582312 }}</ref>		**Pneumonia seen in ~1/3 of ~2200 patients.<ref name=pmid24582312>{{Cite journal \| last1 = Gathmann \| first1 = B. \| last2 = Mahlaoui \| first2 = N. \| last3 = Gérard \| first3 = L. \| last4 = Oksenhendler \| first4 = E. \| last5 = Warnatz \| first5 = K. \| last6 = Schulze \| first6 = I. \| last7 = Kindle \| first7 = G. \| last8 = Kuijpers \| first8 = TW. \| last9 = van Beem \| first9 = RT. \| title = Clinical picture and treatment of 2212 patients with common variable immunodeficiency. \| journal = J Allergy Clin Immunol \| volume = 134 \| issue = 1 \| pages = 116-26 \| month = Jul \| year = 2014 \| doi = 10.1016/j.jaci.2013.12.1077 \| PMID = 24582312 }}</ref>
	*[[Splenomegaly]] seen in ~25% of ~2200 patients.<ref name=pmid24582312/>		*[[Splenomegaly]] seen in ~25% of ~2200 patients.<ref name=pmid24582312/>
	*[[Inflammatory bowel disease]]-like disease.		*[[Inflammatory bowel disease]]-like disease.<ref name=pmid20629103>{{cite journal \|author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L \|title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease \|journal=Inflamm Bowel Dis \|volume= \|issue= \|pages= \|year=2010 \|month=July \|pmid=20629103 \|doi=10.1002/ibd.21376 \|url=}}</ref>
	*Often diagnosed in adulthood ~ mean age 43+/-14 years.<ref name=pmid26992251/>		*Often diagnosed in adulthood ~ mean age 43+/-14 years.<ref name=pmid26992251/>

Michael: /* General */

2016-03-24T22:14:13Z

General

← Older revision		Revision as of 22:14, 24 March 2016
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	*Recurrent sinus and pulmonary infections.		*Recurrent sinus and pulmonary infections.
	**Pneumonia seen in ~1/3 of ~2200 patients.<ref name=pmid24582312>{{Cite journal \| last1 = Gathmann \| first1 = B. \| last2 = Mahlaoui \| first2 = N. \| last3 = Gérard \| first3 = L. \| last4 = Oksenhendler \| first4 = E. \| last5 = Warnatz \| first5 = K. \| last6 = Schulze \| first6 = I. \| last7 = Kindle \| first7 = G. \| last8 = Kuijpers \| first8 = TW. \| last9 = van Beem \| first9 = RT. \| title = Clinical picture and treatment of 2212 patients with common variable immunodeficiency. \| journal = J Allergy Clin Immunol \| volume = 134 \| issue = 1 \| pages = 116-26 \| month = Jul \| year = 2014 \| doi = 10.1016/j.jaci.2013.12.1077 \| PMID = 24582312 }}</ref>		**Pneumonia seen in ~1/3 of ~2200 patients.<ref name=pmid24582312>{{Cite journal \| last1 = Gathmann \| first1 = B. \| last2 = Mahlaoui \| first2 = N. \| last3 = Gérard \| first3 = L. \| last4 = Oksenhendler \| first4 = E. \| last5 = Warnatz \| first5 = K. \| last6 = Schulze \| first6 = I. \| last7 = Kindle \| first7 = G. \| last8 = Kuijpers \| first8 = TW. \| last9 = van Beem \| first9 = RT. \| title = Clinical picture and treatment of 2212 patients with common variable immunodeficiency. \| journal = J Allergy Clin Immunol \| volume = 134 \| issue = 1 \| pages = 116-26 \| month = Jul \| year = 2014 \| doi = 10.1016/j.jaci.2013.12.1077 \| PMID = 24582312 }}</ref>
	*[[Splenomegaly]] - seen in ~ 25% of ~2200 patients.<ref name=pmid24582312/>		*[[Splenomegaly]] seen in ~25% of ~2200 patients.<ref name=pmid24582312/>
	*[[Inflammatory bowel disease]]-like disease.		*[[Inflammatory bowel disease]]-like disease.
	*Often diagnosed in adulthood ~ mean age 43+/-14 years.<ref name=pmid26992251/>		*Often diagnosed in adulthood ~ mean age 43+/-14 years.<ref name=pmid26992251/>

Michael: /* General */

2016-03-24T22:13:55Z

General

← Older revision		Revision as of 22:13, 24 March 2016
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	Features:<ref name=pmid20629103>{{cite journal \|author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L \|title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease \|journal=Inflamm Bowel Dis \|volume= \|issue= \|pages= \|year=2010 \|month=July \|pmid=20629103 \|doi=10.1002/ibd.21376 \|url=}}</ref>		Features:<ref name=pmid20629103>{{cite journal \|author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L \|title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease \|journal=Inflamm Bowel Dis \|volume= \|issue= \|pages= \|year=2010 \|month=July \|pmid=20629103 \|doi=10.1002/ibd.21376 \|url=}}</ref>
	*Recurrent sinus and pulmonary infections.		*Recurrent sinus and pulmonary infections.
			**Pneumonia seen in ~1/3 of ~2200 patients.<ref name=pmid24582312>{{Cite journal \| last1 = Gathmann \| first1 = B. \| last2 = Mahlaoui \| first2 = N. \| last3 = Gérard \| first3 = L. \| last4 = Oksenhendler \| first4 = E. \| last5 = Warnatz \| first5 = K. \| last6 = Schulze \| first6 = I. \| last7 = Kindle \| first7 = G. \| last8 = Kuijpers \| first8 = TW. \| last9 = van Beem \| first9 = RT. \| title = Clinical picture and treatment of 2212 patients with common variable immunodeficiency. \| journal = J Allergy Clin Immunol \| volume = 134 \| issue = 1 \| pages = 116-26 \| month = Jul \| year = 2014 \| doi = 10.1016/j.jaci.2013.12.1077 \| PMID = 24582312 }}</ref>
			*[[Splenomegaly]] - seen in ~ 25% of ~2200 patients.<ref name=pmid24582312/>
	*[[Inflammatory bowel disease]]-like disease.		*[[Inflammatory bowel disease]]-like disease.
	*Often diagnosed in adulthood ~ mean age 43+/-14 years.<ref name=pmid26992251/>		*Often diagnosed in adulthood ~ mean age 43+/-14 years.<ref name=pmid26992251/>


	Labs in a series of 8 patients:<ref name=pmid26992251>{{Cite journal \| last1 = Dong \| first1 = J. \| last2 = Liang \| first2 = H. \| last3 = Wen \| first3 = D. \| last4 = Wang \| first4 = J. \| title = Adult Common Variable Immunodeficiency. \| journal = Am J Med Sci \| volume = 351 \| issue = 3 \| pages = 239-43 \| month = Mar \| year = 2016 \| doi = 10.1016/j.amjms.2015.12.010 \| PMID = 26992251 }}</ref>		Labs in a series of 8 patients:<ref name=pmid26992251>{{Cite journal \| last1 = Dong \| first1 = J. \| last2 = Liang \| first2 = H. \| last3 = Wen \| first3 = D. \| last4 = Wang \| first4 = J. \| title = Adult Common Variable Immunodeficiency. \| journal = Am J Med Sci \| volume = 351 \| issue = 3 \| pages = 239-43 \| month = Mar \| year = 2016 \| doi = 10.1016/j.amjms.2015.12.010 \| PMID = 26992251 }}</ref>

Michael: /* General */

2016-03-24T21:13:01Z

General

← Older revision		Revision as of 21:13, 24 March 2016
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	*Often diagnosed in adulthood ~ mean age 43+/-14 years.<ref name=pmid26992251/>		*Often diagnosed in adulthood ~ mean age 43+/-14 years.<ref name=pmid26992251/>

	Labs in series of 8 patients:<ref name=pmid26992251>{{Cite journal \| last1 = Dong \| first1 = J. \| last2 = Liang \| first2 = H. \| last3 = Wen \| first3 = D. \| last4 = Wang \| first4 = J. \| title = Adult Common Variable Immunodeficiency. \| journal = Am J Med Sci \| volume = 351 \| issue = 3 \| pages = 239-43 \| month = Mar \| year = 2016 \| doi = 10.1016/j.amjms.2015.12.010 \| PMID = 26992251 }}</ref>		Labs in a series of 8 patients:<ref name=pmid26992251>{{Cite journal \| last1 = Dong \| first1 = J. \| last2 = Liang \| first2 = H. \| last3 = Wen \| first3 = D. \| last4 = Wang \| first4 = J. \| title = Adult Common Variable Immunodeficiency. \| journal = Am J Med Sci \| volume = 351 \| issue = 3 \| pages = 239-43 \| month = Mar \| year = 2016 \| doi = 10.1016/j.amjms.2015.12.010 \| PMID = 26992251 }}</ref>
	*IgG 2.5 +/-0.6 g/L.		*IgG 2.5 +/-0.6 g/L.
	*IgA 0.23 +/-0.1 g/L.		*IgA 0.23 +/-0.1 g/L.

Michael: /* General */

2016-03-24T21:09:46Z

General

← Older revision		Revision as of 21:09, 24 March 2016
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	*Recurrent sinus and pulmonary infections.		*Recurrent sinus and pulmonary infections.
	*[[Inflammatory bowel disease]]-like disease.		*[[Inflammatory bowel disease]]-like disease.
			*Often diagnosed in adulthood ~ mean age 43+/-14 years.<ref name=pmid26992251/>

			Labs in series of 8 patients:<ref name=pmid26992251>{{Cite journal \| last1 = Dong \| first1 = J. \| last2 = Liang \| first2 = H. \| last3 = Wen \| first3 = D. \| last4 = Wang \| first4 = J. \| title = Adult Common Variable Immunodeficiency. \| journal = Am J Med Sci \| volume = 351 \| issue = 3 \| pages = 239-43 \| month = Mar \| year = 2016 \| doi = 10.1016/j.amjms.2015.12.010 \| PMID = 26992251 }}</ref>
			*IgG 2.5 +/-0.6 g/L.
			*IgA 0.23 +/-0.1 g/L.
			*IgM 0.17 +/-0.1 g/L.

	==Gross==		==Gross==

Michael: /* Microscopic */

2016-03-18T17:48:06Z

Microscopic

← Older revision		Revision as of 17:48, 18 March 2016
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	*[[Plasma cells]] absent (or reduced) - '''key feature'''.<ref name=pmid20629103>{{cite journal \|author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L \|title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease \|journal=Inflamm Bowel Dis \|volume= \|issue= \|pages= \|year=2010 \|month=July \|pmid=20629103 \|doi=10.1002/ibd.21376 \|url=}}</ref>		*[[Plasma cells]] absent (or reduced) - '''key feature'''.<ref name=pmid20629103>{{cite journal \|author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L \|title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease \|journal=Inflamm Bowel Dis \|volume= \|issue= \|pages= \|year=2010 \|month=July \|pmid=20629103 \|doi=10.1002/ibd.21376 \|url=}}</ref>
	*+/-Lymphoid aggregates.		*+/-Lymphoid aggregates.
			*+/-Granulomatous disease ~8-22%.<ref name=pmid19716342>{{Cite journal \| last1 = Ardeniz \| first1 = O. \| last2 = Cunningham-Rundles \| first2 = C. \| title = Granulomatous disease in common variable immunodeficiency. \| journal = Clin Immunol \| volume = 133 \| issue = 2 \| pages = 198-207 \| month = Nov \| year = 2009 \| doi = 10.1016/j.clim.2009.05.001 \| PMID = 19716342 }}</ref>

	DDx:		DDx:

Michael: /* See also */

2014-09-04T18:07:36Z

Michael: /* See also */

2013-11-26T03:45:02Z

Michael: /* Microscopic */ make table

2013-03-07T04:50:46Z

Microscopic: make table

← Older revision		Revision as of 04:50, 7 March 2013
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	*[[Graft versus host disease]].		*[[Graft versus host disease]].

	~~===Esophagus===~~		Features by site in the GI tract:<ref name=pmid18043034>{{Cite journal \| last1 = Daniels \| first1 = JA. \| last2 = Lederman \| first2 = HM. \| last3 = Maitra \| first3 = A. \| last4 = Montgomery \| first4 = EA. \| title = Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review. \| journal = Am J Surg Pathol \| volume = 31 \| issue = 12 \| pages = 1800-12 \| month = Dec \| year = 2007 \| doi = 10.1097/PAS.0b013e3180cab60c \| PMID = 18043034 }}</ref>
	Features ~~- esophagus~~:<ref name=pmid18043034>{{Cite journal \| last1 = Daniels \| first1 = JA. \| last2 = Lederman \| first2 = HM. \| last3 = Maitra \| first3 = A. \| last4 = Montgomery \| first4 = EA. \| title = Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review. \| journal = Am J Surg Pathol \| volume = 31 \| issue = 12 \| pages = 1800-12 \| month = Dec \| year = 2007 \| doi = 10.1097/PAS.0b013e3180cab60c \| PMID = 18043034 }}</ref>		{\| class="wikitable sortable"
	*Intraepithelial PMNs & candida.		!Site
	*+/-~~Abundant~~ intraepithelial lymphocytes.		!Features
			\|-
	~~===~~Stomach~~===~~		\| Esophagus
	~~Features - stomach:<ref name=pmid18043034/>~~		\| intraepithelial PMNs & candida +/-abundant intraepithelial lymphocytes
	*Lack of [[plasma cell]]s ~ 2/3 of individuals ~~- '''key feature'''~~		\|-
	*Lymphoid aggregates - common.		\| Stomach
	*+/-[[~~Lymphocytic~~ gastritis]].		\| lack of [[plasma cell]]s (~2/3 of individuals), lymphoid aggregates (common), +/-[[lymphocytic gastritis]], +/-intraepithelial neutrophils, +/-infections ([[CMV]], [[H. pylori]], [[cryptosporidium]])
	*+/-~~Intraepithelial~~ neutrophils.		\|-
	*+/-~~Infections~~ ([[CMV]], [[H. pylori]], [[cryptosporidium]]).		\| Small bowel
			\| villous blunting (>80% of individuals), decreased plasma cells (~2/3 of individuals), lymphoid aggregates (~1/2 of individuals), +/-neutrophils + infections (CMV, cryptosporidium)
	~~===~~Small bowel~~===~~		\|-
	~~Features - small bowel:<ref name=pmid18043034/>~~		\| Large bowel
	*Villous blunting > 80% of individuals.		\| decreased plasma cells, lymphoid aggregates, +/-apparent apoptosis
	*Decreased plasma cells ~ 2/3 of individuals ~~- '''key feature'''.~~		\|}
	*Lymphoid aggregates ~ 1/2 of individuals.
	*+/-~~Neutrophils~~ + infections (CMV, cryptosporidium).

	~~===~~Large bowel~~===~~
	~~Features:<ref name=pmid18043034/>~~
	*Decreased plasma cells ~~- '''key feature'''.~~
	*Lymphoid aggregates.
	*+/-~~Apparent~~ apoptosis.

	==See also==		==See also==

Michael: /* Microscopic */

2013-03-07T04:43:13Z

Microscopic

← Older revision		Revision as of 04:43, 7 March 2013
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	*[[Plasma cells]] absent (or reduced) - '''key feature'''.<ref name=pmid20629103>{{cite journal \|author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L \|title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease \|journal=Inflamm Bowel Dis \|volume= \|issue= \|pages= \|year=2010 \|month=July \|pmid=20629103 \|doi=10.1002/ibd.21376 \|url=}}</ref>		*[[Plasma cells]] absent (or reduced) - '''key feature'''.<ref name=pmid20629103>{{cite journal \|author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L \|title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease \|journal=Inflamm Bowel Dis \|volume= \|issue= \|pages= \|year=2010 \|month=July \|pmid=20629103 \|doi=10.1002/ibd.21376 \|url=}}</ref>
	*+/-Lymphoid aggregates.		*+/-Lymphoid aggregates.

			DDx:
			*[[Inflammatory bowel disease]].
			*[[Celiac disease]].
			*[[Graft versus host disease]].

	===Esophagus===		===Esophagus===

← Older revision		Revision as of 18:07, 4 September 2014
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	*[[Plasma cell]].		*[[Plasma cell]].
	*[[Sarcoidosis]].		*[[Sarcoidosis]].
			*[[Autoimmune enteropathy]].

	==References==		==References==

← Older revision		Revision as of 03:45, 26 November 2013
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	==See also==		==See also==
	*[[Plasma cell]].		*[[Plasma cell]].
			*[[Sarcoidosis]].

	==References==		==References==