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{{Infobox_Disease |
 
  Name          = {{PAGENAME}} |
  Image          = Immunglobulin A as Dimer.png  |
  Caption        = The dimeric IgA molecule. 1 H-chain, 2 L-chain, 3 J-chain, 4 secretory component |
  DiseasesDB    = 29569 |
  ICD10          = {{ICD10|D|80|2|d|80}} |
  ICD9          = {{ICD9|279.01}} |
  ICDO          = |
  OMIM          = 137100 |
  MedlinePlus    = 001476 |
  eMedicineSubj  = med |
  eMedicineTopic = 1159 |
}}
__NOTOC__
__NOTOC__
{{Selective immunoglobulin A deficiency}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}


==Overview==
'''For patient information, click [[Selective immunoglobulin A deficiency(patient information)|here]]'''


'''Selective immunoglobulin A (IgA) deficiency''' is a relatively mild genetic [[immunodeficiency]]. People with this deficiency lack immunoglobulin A (IgA), a type of antibody that protects against infections of the mucous membranes lining the mouth, airways, and digestive tract. It is defined as an undetectable serum IgA level in the presence of normal serum levels of IgG and IgM. It is the most common of the primary antibody deficiencies.
{{CMG}}; {{AE}}


==Historical Perspective==
{{SK}}


==Classification==
==[[Selective immunoglobulin A deficiency overview|Overview]]==


==Pathophysiology==
==[[Selective immunoglobulin A deficiency historical perspective|Historical Perspective]]==


==Causes==
==[[Selective immunoglobulin A deficiency classification|Classification]]==


==Differentiating {{PAGENAME}} from Other Diseases==
==[[Selective immunoglobulin A deficiency pathophysiology|Pathophysiology]]==


==Epidemiology and Demographics==
==[[Selective immunoglobulin A deficiency causes|Causes]]==
Prevalence varies by population, but is on the order of 1 in 500 to 1 in 800 people, making it relatively common for a genetic disease.


==Risk Factors==
==[[Selective immunoglobulin A deficiency differential diagnosis|Differentiating selective immunoglobulin A deficiency from other Diseases]]==


==Screening==
==[[Selective immunoglobulin A deficiency epidemiology and demographics|Epidemiology and Demographics]]==


==Natural History, Complications, and Prognosis==
==[[Selective immunoglobulin A deficiency risk factors|Risk Factors]]==
===Natural History===


===Complications===
==[[Selective immunoglobulin A deficiency screening|Screening]]==


===Prognosis===
==[[Selective immunoglobulin A deficiency natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
Prognosis is excellent, although there is an association with [[autoimmune disease]].  Of note, selective IgA deficiency can complicate the diagnosis of one such condition, [[celiac disease]], as the deficiency masks the [[celiac disease#Blood tests|high levels of certain IgA antibodies]] usually seen in celiac disease.


==Diagnosis==
==Diagnosis==
===Diagnostic Criteria===
[[Selective immunoglobulin A deficiency diagnostic study of choice|Diagnostic study of choice]] | [[Selective immunoglobulin A deficiency history and symptoms|History and Symptoms]] | [[Selective immunoglobulin A deficiency physical examination|Physical Examination]] | [[Selective immunoglobulin A deficiency laboratory findings|Laboratory Findings]] | [[Selective immunoglobulin A deficiency electrocardiogram|Electrocardiogram]] | [[Selective immunoglobulin A deficiency x ray|X-Ray Findings]] | [[Selective immunoglobulin A deficiency echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Selective immunoglobulin A deficiency CT scan|CT-Scan Findings]] | [[Selective immunoglobulin A deficiency MRI|MRI Findings]] | [[Selective immunoglobulin A deficiency other imaging findings|Other Imaging Findings]] | [[Selective immunoglobulin A deficiency other diagnostic studies|Other Diagnostic Studies]]
 
===History and Symptoms===
People with selective IgA deficiency are asymptomatic, or have increased frequency of infections such as [[sinusitis]].  These infections are generally mild and would not usually lead to an in-depth workup except when unusually frequent.  When suspected, the diagnosis can be confirmed by laboratory measurement of IgA level in the blood. Patients have an increased susceptibility to pneumonia and recurrent episodes of other respiratory infections and a higher risk of developing autoimmune diseases in middle age.<ref>{{cite journal | author=Koskinen S | title=Long-term follow-up of health in blood donors with primary selective IgA deficiency. | journal=J Clin Immunol | volume=16 | issue=3 | pages=165-70 | year=1996 | id=PMID 8734360}}</ref>
 
===Physical Examination===
 
===Laboratory Findings===
 
===Imaging Findings===
 
===Other Diagnostic Studies===


==Treatment==
==Treatment==
===Medical Therapy===
[[Selective immunoglobulin A deficiency medical therapy|Medical Therapy]] | [[Selective immunoglobulin A deficiency interventions|Interventions]] | [[Selective immunoglobulin A deficiency surgery|Surgery]] | [[Selective immunoglobulin A deficiency primary prevention|Primary Prevention]] | [[Selective immunoglobulin A deficiency secondary prevention|Secondary Prevention]] | [[Selective immunoglobulin A deficiency cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Selective immunoglobulin A deficiency future or investigational therapies|Future or Investigational Therapies]]
 
The treatment consists of identification of comorbid conditions, preventive measures to reduce the risk of infection, and prompt and effective treatment of infections. Infections in an IgA-deficient person are treated as usual (i.e., with antibiotics). There is no treatment for the underlying disorder (except for [[bone marrow transplant]], which is far too drastic to be used). In the absence of normal IgA, the body can develop an immune response against IgA itself (anti-IgA antibodies), which can lead to severe reactions including [[anaphylaxis]] to blood transfusions or [[intravenous immunoglobulin]]. Therefore, people known to have this condition must notify their healthcare providers. Since this is a genetic condition, careful discussion should be undertaken prior to having children.
 
===Surgery===
 
===Primary Prevention===
 
===Secondary Prevention===
 
==Treatment==
 
==References==
{{reflist|2}}


{{Immune disorders}}
==Case Studies==
[[Category:Immunology]]
[[Selective immunoglobulin A deficiency case study one|Case #1]]
[[Category:Hematology]]


{{WS}}
[[Category: (name of the system)]]
{{WH}}

Revision as of 22:56, 14 September 2018


Template:Selective immunoglobulin A deficiency Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

For patient information, click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]; Associate Editor(s)-in-Chief:

Synonyms and keywords:

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating selective immunoglobulin A deficiency from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Interventions | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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