Immunoglobulin M deficiency: Difference between revisions

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{{SI}}
{{SI}}


{{CMG}}; {{AE}}
{{CMG}}; {{AE}} {{JSS}}


{{SK}}  
{{SK}}IgM deficiency


==Overview==
==Overview==
Immunoglobulin M deficiency is said to be present when the levels of [[Immunoglobulin M|IgM]] in serum are <40mg/dl while the levels of all other [[Antibody|immunoglobulins]] are within normal range. IgM deficiency was first noted in 1969 by Roy- Chawdhary from a jejunal biopsy. It is of 2 types primary and secondary IgM deficiency. IgM deficiency can occur due to a defect in [[B cell|B cells]] or [[T cell|T cells]]. IgM deficiency can lead to chronic [[Infection|infections]], development of [[Autoimmunity|autoimmune]] disease and [[Cancer|neoplastic]] diseases. IgM deficiency cab be caused in [[autoimmune]] conditions and it can itself lead to the development of [[autoimmune]] conditions. IgM deficiency can be differentiated from other immunoglobuiln deficiencies by measuring the levels of all the immunoglobulins.The [[incidence]]/[[prevalence]] of IgM deficiency is approximately 100-2000 per 100,000 individuals worldwide. The patients usually present with chronic or recurrent infections. Signs and symptoms of [[Copd|chronic lung disease]], chronic [[Ear, nose, throat (ENT) deep-space infection|ear]] and chronic [[sinus]] infections may be present.


==Historical Perspective==
==Historical Perspective==
[Disease name] was first discovered by [name of scientist], a [nationality + occupation], in [year]/during/following [event].
* IgM was discovered by Waldenstrom, Pedersen and kunkel in 1944 by [[immunoelectrophoresis]] and [[Differential centrifugation|ultracentrifugation]]<ref name="pmid9046436">{{cite journal| author=Black CA| title=A brief history of the discovery of the immunoglobulins and the origin of the modern immunoglobulin nomenclature. | journal=Immunol Cell Biol | year= 1997 | volume= 75 | issue= 1 | pages= 65-8 | pmid=9046436 | doi=10.1038/icb.1997.10 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9046436  }} </ref>.
 
* IgM deficiency was first noted in 1969 by Roy- Chawdhary from a jejunal biopsy.
The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
 
In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].
 
In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].
 
There have been several outbreaks of [disease name], including -----.
 
In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].


==Classification==
==Classification==
There is no established system for the classification of IgM deficiency.
There is no established system for the classification of IgM deficiency<ref name="pmid15611856">{{cite journal| author=Manson JJ, Mauri C, Ehrenstein MR| title=Natural serum IgM maintains immunological homeostasis and prevents autoimmunity. | journal=Springer Semin Immunopathol | year= 2005 | volume= 26 | issue= 4 | pages= 425-32 | pmid=15611856 | doi=10.1007/s00281-004-0187-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15611856  }} </ref>.However, it may present as 2 types:
* However, it may present as 2 types:
*Primary IgM deficiency-
* Primary IgM deficiency- There ocuurs just IgM deficiency and no other associated condition.
** There occurs just [[Immunoglobulin M|IgM]] deficiency and no other associated condition.
* Secondary IgM- Alongwith IgM deficiency there occurs autoimmune disorders or some neoplasms.
** It is present in children as a congenital condition.
*Secondary IgM deficiency-
** Along with IgM deficiency there occurs [[Autoimmunity|autoimmune]] disorders or some [[Neoplasm|neoplasms]].
** It occurs in adults and is acquired.


==Pathophysiology==
==Pathophysiology==
* IgM is the first antibody that is present on the surface of B lymphocytes.
* IgM is the first antibody that is present on the surface of [[B cell|B lymphocytes]] when they come in contact with an [[Antigen|antigen.]]<ref name="pmid10748243">{{cite journal| author=Ehrenstein MR, Cook HT, Neuberger MS| title=Deficiency in serum immunoglobulin (Ig)M predisposes to development of IgG autoantibodies. | journal=J Exp Med | year= 2000 | volume= 191 | issue= 7 | pages= 1253-8 | pmid=10748243 | doi= | pmc=2193170 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10748243  }} </ref><ref name="pmid14215847">{{cite journal| author=GILBERT C, HONG R| title=QUALITATIVE AND QUANTITATIVE IMMUNOGLOBULIN DEFICIENCY. | journal=Am J Med | year= 1964 | volume= 37 | issue=  | pages= 602-9 | pmid=14215847 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14215847  }} </ref><ref name="pmid18005364">{{cite journal| author=Gonzalez-Quintela A, Alende R, Gude F, Campos J, Rey J, Meijide LM et al.| title=Serum levels of immunoglobulins (IgG, IgA, IgM) in a general adult population and their relationship with alcohol consumption, smoking and common metabolic abnormalities. | journal=Clin Exp Immunol | year= 2008 | volume= 151 | issue= 1 | pages= 42-50 | pmid=18005364 | doi=10.1111/j.1365-2249.2007.03545.x | pmc=2276914 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18005364  }} </ref><ref name="pmid50091">{{cite journal| author=Hobbs JR| title=IgM deficiency. | journal=Birth Defects Orig Artic Ser | year= 1975 | volume= 11 | issue= 1 | pages= 112-6 | pmid=50091 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=50091  }} </ref>
* It is the primary antibody against [[ABO blood group system|A]] and [[ABO blood group system|B]] [[Antigen|antigens]] on [[Red blood cell|red blood cells]].
* It is the primary antibody against [[ABO blood group system|A]] and [[ABO blood group system|B]] [[Antigen|antigens]] on [[Red blood cell|red blood cells]].
* Other immunoglublins such as IgG, IgA and IgE are produced after alteration in the structure of IgM heavy chains on the B lymphocytes.
* Other [[An Bord Altranais|immunoglublins]] such as [[Immunoglobulin G|IgG]], [[Immunoglobulin A|IgA]] and [[Immunoglobulin E|IgE]] are produced after alteration in the structure of [[Immunoglobulin M|IgM]] heavy chains on the [[B cell|B lymphocytes]]<ref name="pmid12471099">{{cite journal| author=Baker N, Ehrenstein MR| title=Cutting edge: selection of B lymphocyte subsets is regulated by natural IgM. | journal=J Immunol | year= 2002 | volume= 169 | issue= 12 | pages= 6686-90 | pmid=12471099 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12471099  }} </ref>.
* IgM is present in the circulation as a pentamer structure. and is the largest antibody in human circulatory system.
* IgM is present in the circulation as a pentamer structure. and is the largest antibody in human [[Circulatory system|circulatory system.]]
* Demonstrating IgM antibodies in a patient's serum indicates recent infection, or in a neonate's serum indicates intrauterine infection (e.g. [[congenital rubella]]).
* Presence of IgM antibodies in a patient's [[serum]] indicates recent [[infection]], or in a [[Infant|neonate]]'s serum indicates intrauterine infection (e.g. [[congenital rubella]]).
* IgM activates the classic pathway of complement system.
* IgM activates the classic pathway of [[complement]] system.
* In IgM deficiency there occurs deficiency of IgM but normal levels of IgG and IgA.
* In IgM deficiency there occurs deficiency of IgM but normal levels of [[Immunoglobulin G|IgG]] and [[Immunoglobulin A|IgA.]]
* The level of IgM in serum is less than 40mg/dl (normal value 45-150mg/dl).
* The level of IgM in serum is less than 40mg/dl (normal value 45-150mg/dl).
* The defeciency in IgM could be due to:
* The defeciency in IgM could be due to<ref name="pmid2872228">{{cite journal| author=Inoue T, Okumura Y, Shirama M, Ishibashi H, Kashiwagi S, Okubo H| title=Selective partial IgM deficiency: functional assessment of T and B lymphocytes in vitro. | journal=J Clin Immunol | year= 1986 | volume= 6 | issue= 2 | pages= 130-5 | pmid=2872228 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2872228  }} </ref>:
*# B cell defect- inability of B cell differentiation into IgM secreting cells.
*# [[B cell]] defect- inability of B cell differentiation into IgM secreting cells<ref name="pmid21516014">{{cite journal| author=Saini S, Dettore AJ, Bhambhani KJ, Buck S, Poulik J, Savaşan S| title=Selective IgM deficiency in CD30+ cutaneous lymphoproliferative disorder. | journal=J Pediatr Hematol Oncol | year= 2011 | volume= 33 | issue= 4 | pages= e156-9 | pmid=21516014 | doi=10.1097/MPH.0b013e31820150d5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21516014  }} </ref><ref name="pmid6983403">{{cite journal| author=De la Concha EG, Garcia-Rodriguez MC, Zabay JM, Laso MT, Alonso F, Bootello A et al.| title=Functional assessment of T and B lymphocytes in patients with selective IgM deficiency. | journal=Clin Exp Immunol | year= 1982 | volume= 49 | issue= 3 | pages= 670-6 | pmid=6983403 | doi= | pmc=1536738 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6983403  }} </ref>.
*# T cell defect- decreased helper T cell activity for IgM.
*# [[T cell]] defect- decreased [[T helper cell|helper T cell]] activity for IgM.
*# Genetics- chromosome 22q11.2 deletion syndrome.
* This results in decreased sysnthesis of IgM in the body.
* This results in decreased sysnthesis of IgM in the body.
* It can be primary or secondary.
* It can be primary or secondary.
* The primary IgM deficiency presents with chronic infections while secondary presents with associated autoimmune conditions or neoplasms.
* The primary IgM deficiency presents with chronic infections while secondary presents with associated autoimmune conditions or neoplasms.
* Due to absence of IgM infections like otitis media, chronic sinusitis, bronchitis, bronchiectasis, pneumonia, urinary tract infections, cellulitis, meningitis, sepsis, etc. are very common.
* Due to absence of IgM, infections like [[otitis media]], [[Rhinosinusitis|chronic sinusitis]], [[bronchitis]], [[bronchiectasis]], [[pneumonia]], [[Urinary tract infection|urinary tract infections]], [[cellulitis]], [[meningitis]], [[sepsis]], etc. are very common.
* Most common infections are staphylococcus aures, streptoccus pneumonia and haemophilus influenza.
* Most common infections are [[staphylococcus aureus]], [[streptococcus pneumonia]] and [[Haemophilus influenzae|haemophilus influenza]].
* IgM deficiency increases the risk of acquiring autoimmune and some malignant conditions.
* IgM deficiency increases the risk of acquiring [[autoimmune]] and some [[malignant]] conditions.
** Autoimmune glomerulonephritis
** [[Glomerular disease|Autoimmune glomerulonephritis]]
** Autoimmune hemolytic anemia
**<nowiki/>[[Autoimmune hemolytic anemia|Autoimmune hemolytic anemi]]<nowiki/>a
** Autoimmune thrombocytopenia
** [[Thrombocytopenia|Autoimmune thrombocytopeni]]<nowiki/>[[Thrombocytopenia|a]]
** Celiac disease
** [[Celiac disease]]
** Crohns disease
** [[Crohn's disease|Crohns disease]]
** Hashimotto thyroiditis
** [[Hashimoto's thyroiditis]]
** Myathenia gravis
** [[Rheumatoid arthritis]]
** Rheumatoid arthritis
** [[Sarcoma]]
** Sarcoma
** [[Lymphoma]]
** Lymphoma
** [[Myasthenia gravis]]
** Plasmacytoma
** [[Multiple myeloma|Plasmacytoma]]
 


==Causes==
==Causes==
The exact cause of IgM deficiency is not known.
The exact cause of IgM deficiency is not known.
* Primary IgM deficiency ouccrs in children and is congenital.
* Primary IgM deficiency ouccrs in children and is [[Congenital disorder|congenital]].
* Secondary igM deficiency occurs in adullts and is associated with autoimmune disorders and neoplasms.
* Secondary IgM deficiency occurs in adults and is associated with [[Autoimmunity|autoimmune disorders]] and [[Neoplasm|neoplasms]]<ref name="pmid6603786">{{cite journal| author=Takenaka T, Nakamine H, Nishihara T, Tsuda T, Tsujimoto M, Maeda J| title=Prolymphocytic leukemia with IgM hypogammaglobulinemia. | journal=Am J Clin Pathol | year= 1983 | volume= 80 | issue= 2 | pages= 237-42 | pmid=6603786 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6603786  }} </ref>.
* There is no identified cause but risk factors which are:
* There is no identified cause but risk factors which are:
** Autoimmune glomerulonephritis
**[[Clear cell tumor|Clear cell carcinoma]]
** Autoimmune hemolytic anemia
** [[Bloom syndrome]]
** Autoimmune thrombocytopenia
** [[Promyelocytic leukemia protein|Promyelocytic leukemia]]
** Celiac disease
** [[Brucella]]
** Crohns disease
** [[Crohn's disease]]
** Hashimotto thyroiditis
** [[Chronic diarrhea]]
** Myathenia gravis
** [[Whipple's disease|Whipple disease]]
** Rheumatoid arthritis
** Infants with [[hypothyroidism]]
** Systemic lupus erythematosus
** [[Multiple myeloma]]
** Sarcoma
** Chromosome 22q.11.2 deletion
** Lymphoma
** Plasmacytoma


==Differentiating ((Page name)) from Other Diseases==
==Differentiating IgM deficiency from Other Diseases==
*IgM deficiency can be differentiated from other diseases of the same kind by measuring the value of other immunoglobulins.
*IgM deficiency can be differentiated from other diseases of the same kind by measuring the value of other immunoglobulins<ref name="pmid367347">{{cite journal| author=Thong YH, Maxwell GM| title=Primary selective deficiency of immunoglobulin M. | journal=Aust N Z J Med | year= 1978 | volume= 8 | issue= 4 | pages= 436-8 | pmid=367347 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=367347  }} </ref><ref name="pmid23654075">{{cite journal| author=Cipe FE, Doğu F, Güloğlu D, Aytekin C, Polat M, Biyikli Z et al.| title=B-cell subsets in patients with transient hypogammaglobulinemia of infancy, partial IgA deficiency, and selective IgM deficiency. | journal=J Investig Allergol Clin Immunol | year= 2013 | volume= 23 | issue= 2 | pages= 94-100 | pmid=23654075 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23654075  }} </ref><ref name="pmid14699405">{{cite journal| author=Fischer A| title=Human primary immunodeficiency diseases: a perspective. | journal=Nat Immunol | year= 2004 | volume= 5 | issue= 1 | pages= 23-30 | pmid=14699405 | doi=10.1038/ni1023 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14699405  }} </ref><ref name="pmid20851686">{{cite journal| author=Ozen A, Baris S, Karakoc-Aydiner E, Ozdemir C, Bahceciler NN, Barlan IB| title=Outcome of hypogammaglobulinemia in children: immunoglobulin levels as predictors. | journal=Clin Immunol | year= 2010 | volume= 137 | issue= 3 | pages= 374-83 | pmid=20851686 | doi=10.1016/j.clim.2010.08.010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20851686  }} </ref><ref name="pmid15356576">{{cite journal| author=Notarangelo L, Casanova JL, Fischer A, Puck J, Rosen F, Seger R et al.| title=Primary immunodeficiency diseases: an update. | journal=J Allergy Clin Immunol | year= 2004 | volume= 114 | issue= 3 | pages= 677-87 | pmid=15356576 | doi=10.1016/j.jaci.2004.06.044 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15356576  }} </ref>.
*In Igm deficiency only IgM levels are defiicent while the rest are within normal limits.
*In IgM deficiency only IgM levels are deficIent while the rest are within normal limits.


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| ↓ || ↓ || ↑ || ↑ || - || +  
| ↓ || ↓ || ↑ || ↑ || - || +  
|-
|-
! Hyper IgE syndrome
| - || - || - || ↑ || - || +
|}
|}


==Epidemiology and Demographics==
==Epidemiology and Demographics==
The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
* The [[incidence]] of IgM deficiency is approximately 100-2000 per 100,000 individuals worldwide.
 
OR
 
In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.


OR
* Primary IgM defiicency is present congenitally in children which presents in age group 3-36 months.
* Secondary IgM deficiency occurs in adults in age group 30-40, no relation with sex or age.


In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.
* Complications such as development of [[autoimmune disease]] and [[Cancer|neoplastic]] conditions occur in adult age group.
 
 
 
Patients of all age groups may develop [disease name].
 
OR
 
The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
 
OR
 
[Disease name] commonly affects individuals younger than/older than [number of years] years of age.
 
OR
 
[Chronic disease name] is usually first diagnosed among [age group].
 
OR
 
[Acute disease name] commonly affects [age group].
 
 
 
There is no racial predilection to [disease name].
 
OR
 
[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
 
 
 
[Disease name] affects men and women equally.
 
OR
 
[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
 
 
 
The majority of [disease name] cases are reported in [geographical region].
 
OR
 
[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].


==Risk Factors==
==Risk Factors==
There are no established risk factors for [disease name].
There are no established risk factors for IgM deficiency but it occurs more commonly in<ref name="pmid7053825">{{cite journal| author=Vogelzang NJ, Corwin H, Finlay JL, Pellettiere EV, Luskin AT, Di Camelli RF et al.| title=Clear cell sarcoma and selective IgM deficiency: a case report. | journal=Cancer | year= 1982 | volume= 49 | issue= 2 | pages= 234-8 | pmid=7053825 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7053825  }} </ref><ref name="pmid17650836">{{cite journal| author=Kung SJ, Gripp KW, Stephan MJ, Fairchok MP, McGeady SJ| title=Selective IgM deficiency and 22q11.2 deletion syndrome. | journal=Ann Allergy Asthma Immunol | year= 2007 | volume= 99 | issue= 1 | pages= 87-92 | pmid=17650836 | doi=10.1016/S1081-1206(10)60627-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17650836  }} </ref>:
 
* [[Clear cell tumor|Clear cell carcinoma]]
OR
* [[Bloom syndrome]]
 
* [[Promyelocytic leukemia protein|Promyelocytic leukemia]]
The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].
* [[Brucella]]
 
* [[Crohn's disease]]
OR
* [[Chronic diarrhea]]
 
* [[Whipple's disease|Whipple disease]]
Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
* Infants with [[hypothyroidism]]
 
* [[Multiple myeloma]]
OR
* Chromosome 22q.11.2 deletion
 
Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.
 
==Screening==
There is insufficient evidence to recommend routine screening for [disease/malignancy].
 
OR
 
According to the [guideline name], screening for [disease name] is not recommended.


OR
== Screening ==
* There is insufficient data to recommend screening for IgM deficiency.


According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].
* However, in patients presenting with recurrent infections, all immunologlobulins (IgM, IgG, IgA, IgE and IgD) can be measured.


==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
* The patient may be asysmptomatic or present with signs of chronic infections.
* The patient may be asysmptomatic or present with signs of chronic infections<ref name="pmid1274982">{{cite journal| author=Yocum MW, Strong DM, Chusid MJ, Lakin JD| title=Selective immunoglobulin M (IgM) deficiency in two immunodeficient adults with recurrent staphylococcal pyoderma. | journal=Am J Med | year= 1976 | volume= 60 | issue= 4 | pages= 486-94 | pmid=1274982 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1274982  }} </ref>.
* Children born with IgM deficiency as in primary IgM deficiency present with chronic infections such as:
* Children born with IgM deficiency as in primary IgM deficiency present with chronic infections such as:
** Otitis media
** [[Otitis media]]
** Chronic sinusitis
** [[Rhinosinusitis|Chronic sinusitis]]
** Bronchitis
** [[Bronchitis]]
** Bronchiectasis
** [[Bronchiectasis]]
** Pneumonia
** [[Pneumonia]]
** Urinary tract infections  
** [[Urinary tract infection|Urinary tract infections]]
** Abscesses
** [[Abscess|Abscesses]]
** Meningitis
** [[Meningitis]]
** Gastroenteritis
** [[Gastroenteritis]]
* Adlults with IgM deficiency as in secondary IgM deficiency also present with chronic and multiple infections.
* Adlults with IgM deficiency as in secondary IgM deficiency also present with chronic and multiple infections<ref name="pmid23523054">{{cite journal| author=Phuphuakrat A, Ngamjanyaporn P, Nantiruj K, Luangwedchakarn V, Malathum K| title=Selective IgM deficiency in an adult presenting with Streptococcus pneumoniae septic arthritis. | journal=J Microbiol Immunol Infect | year= 2016 | volume= 49 | issue= 1 | pages= 150-3 | pmid=23523054 | doi=10.1016/j.jmii.2013.01.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23523054  }} </ref><ref name="pmid2962796">{{cite journal| author=Raziuddin S, Bilal N, Benjamin B| title=Transient T-cell abnormality in a selective IgM-immunodeficient patient with Brucella infection. | journal=Clin Immunol Immunopathol | year= 1988 | volume= 46 | issue= 3 | pages= 360-7 | pmid=2962796 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2962796  }} </ref><ref name="pmid4182649">{{cite journal| author=Chandra RK, Kaveramma B, Soothill JF| title=Generalised non-progressive vaccinia associated with IgM deficiency. | journal=Lancet | year= 1969 | volume= 1 | issue= 7597 | pages= 687-9 | pmid=4182649 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4182649  }} </ref><ref name="pmid11905841">{{cite journal| author=Takeuchi T, Nakagawa T, Maeda Y, Hirano S, Sasaki-Hayashi M, Makino S et al.| title=Functional defect of B lymphocytes in a patient with selective IgM deficiency associated with systemic lupus erythematosus. | journal=Autoimmunity | year= 2001 | volume= 34 | issue= 2 | pages= 115-22 | pmid=11905841 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11905841  }} </ref>.
* With time, autoimmune and neoplastic diseases may also occur such as:
* With time, autoimmune and neoplastic diseases may also occur such as:
** Autoimmune glomerulonephritis
** Autoimmune glomerulonephritis
** Autoimmune hemolytic anemia
** [[Autoimmune hemolytic anemia]]
** Autoimmune thrombocytopenia
** [[Thrombocytopenia|Autoimmune thrombocytopenia]]
** Celiac disease
** [[Celiac disease]]
** Crohns disease
** [[Crohn's disease|Crohns disease]]
** Hashimotto thyroiditis
** [[Hashimoto's thyroiditis]]
** Myathenia gravis
** [[Myasthenia gravis]]
** Rheumatoid arthritis
** [[Rheumatoid arthritis]]
** Systemic lupus erythematosus
** [[Systemic lupus erythematosus]]
** Sarcoma
** [[Sarcoma]]
** Lymphoma
** [[Lymphoma]]
** Plasmacytoma
** [[Multiple myeloma|Plasmacytoma]]
** [[Splenomegaly]]
** [[Atopy]] ([[asthma]], [[anaphylaxis]] and [[angioedema]])
* The prgnosis is good in primary IgM deficiency if diagnosed early as IgM supplements can be given.
* The prgnosis is good in primary IgM deficiency if diagnosed early as IgM supplements can be given.
* In secondary IgM, autoiimune and neoplastic complications occur and prognosis is poor.
* In secondary IgM, [[autoimmune]] and neoplastic complications occur and prognosis is poor.


==Diagnosis==
==Diagnosis==
===Diagnostic Study of Choice===
===Diagnostic Study of Choice===
The diagnosis of IgM deficiency is made by measuring serum IgM levels.
The diagnosis of IgM deficiency is made by measuring serum IgM levels<ref name="pmid10600329">{{cite journal| author=Conley ME, Notarangelo LD, Etzioni A| title=Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). | journal=Clin Immunol | year= 1999 | volume= 93 | issue= 3 | pages= 190-7 | pmid=10600329 | doi=10.1006/clim.1999.4799 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10600329  }} </ref>.
* Serum IgM levels of >40mg/dl is diagnostic of igM deficiency
* Serum IgM levels of <40mg/dl is diagnostic of IgM deficiency
* Serum sample is taken and then anti immunoglobulins are added.
* [[Serum]] sample is taken and then anti [[Antibodies|immunoglobulins]] are added.
* The amount of IgM produced is then measured.
* The amount of IgM produced is then measured.
* Other tests include
* Other tests include
** CBC
** [[Complete blood count|CBC]]
** Serum IgG, IgA, IgE and IgD levels
** Serum IgG, IgA, IgE and IgD levels
** Complement profile
** [[Complement]] profile
** Serum and urine electrophoresis
** Serum and urine [[electrophoresis]]
** Serum ANA levels
** Serum [[Antinuclear antibodies|ANA]] levels


===History and Symptoms===
===History and Symptoms===
* The majority of patients with IgM deficiency are asymptomatic.
* The majority of patients with IgM deficiency are asymptomatic<ref name="pmid19494527">{{cite journal| author=Yel L, Ramanuja S, Gupta S| title=Clinical and immunological features in IgM deficiency. | journal=Int Arch Allergy Immunol | year= 2009 | volume= 150 | issue= 3 | pages= 291-8 | pmid=19494527 | doi=10.1159/000222682 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19494527  }} </ref><ref name="pmid18564634">{{cite journal| author=Hong R, Gupta S| title=Selective immunoglobulin M deficiency in an adult with Streptococcus pneumoniae sepsis and invasive aspergillosis. | journal=J Investig Allergol Clin Immunol | year= 2008 | volume= 18 | issue= 3 | pages= 214-8 | pmid=18564634 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18564634  }} </ref><ref name="pmid23257944">{{cite journal| author=Arahata M, Tajiri K, Nomoto K, Tsuneyama K, Minami S, Shimizu Y| title=A novel type of selective immunoglobulin m deficiency in a patient with autoimmune liver cirrhosis with recurrent hepatocellular carcinoma: a case report and review of the literature. | journal=Int Arch Allergy Immunol | year= 2013 | volume= 161 | issue= 1 | pages= 91-6 | pmid=23257944 | doi=10.1159/000343583 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23257944  }} </ref>.
* They may present with history of :
* They may present with history of :
** Chronic infections
** Chronic infections
** Weight loss
** [[Weight loss]]
** Chronic lung disease
** [[Copd|Chronic lung disease]]
** Chronic diarrhea
** Chronic [[diarrhea]]
** Arthralgias
** [[Arthralgia|Arthralgias]]
** Abscesses
** [[Abscess|Abscesses]]
** Allergic disorders
** [[Allergy|Allergic disorders]]
** Liver disease
** [[Hepato-biliary diseases|Liver disease]]


=== Symptoms ===
=== Symptoms ===
* The patients may be asymptomatic.
* The patients may be asymptomatic<ref name="pmid6748317">{{cite journal| author=Morita M, Saibara T, Nakazawa Y, Miyao M, Okazaki K, Onishi S et al.| title=[A case of Crohn's disease associated with selective IgM deficiency]. | journal=Nihon Shokakibyo Gakkai Zasshi | year= 1984 | volume= 81 | issue= 4 | pages= 1070-5 | pmid=6748317 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6748317  }} </ref>.


* Symptoms of IgMdeficiency may include the symptoms of recurrent sinopulmonary infections include otitis media, rhinosinusitis, and pneumonia andmore serious infections that can occur include osteomyelitis, meningitis, septicemia, diarrhea, and various skin infections:
* Symptoms of IgM deficiency may include the symptoms of recurrent sinopulmonary infections include [[otitis media]], [[rhinosinusitis]], [[pneumonia]] and more serious infections that can occur, include [[osteomyelitis]], [[Meningococcemia|meningitis,]] [[Sepsis|septicemia]], [[diarrhea]], and various skin infections:


===Physical Examination===
===Physical Examination===
* Physical examination of patients with longstanding IgM deficiency may present with
* Physical examination of patients with longstanding IgM deficiency may present with<ref name="pmid12426258">{{cite journal| author=Popa V, Colby TV, Reich SB| title=Pulmonary interstitial disease in Ig deficiency. | journal=Chest | year= 2002 | volume= 122 | issue= 5 | pages= 1594-603 | pmid=12426258 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12426258  }} </ref><ref name="pmid977778">{{cite journal| author=Ross IN, Thompson RA| title=Severe selective IgM deficiency. | journal=J Clin Pathol | year= 1976 | volume= 29 | issue= 9 | pages= 773-7 | pmid=977778 | doi= | pmc=476177 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=977778  }} </ref>
* :Fever
***[[Fever]]
** Low body mass index
*** Low body mass index
** Swelling and tenderness over maxilla and frontal sinuses.
*** [[Edema|Swelling]] and [[tenderness]] over [[Maxillary bone|maxilla]] and [[Frontal sinus|frontal sinuse]]<nowiki/>s.
** Perforated tympanic membrane or scarring over tympanic membrane.
*** Perforated [[Eardrum|tympanic membrane]] or scarring over tympanic membrane.
** Chronic nasal discharge.
*** Chronic nasal discharge.
** Chronic cough, Crepitations, clubbing and wheezing.
*** Chronic [[cough]], [[Rales|Crepitations]], [[clubbing]] and [[Wheeze|wheezing]].
** Abdominal dystension, tenderness
*** Abdominal dystension, tenderness
** Abscesses  
*** [[Abscess|Abscesses]]
** Ulcers.
*** [[Ulcer|Ulcers]].


===Laboratory Findings===
===Laboratory Findings===
* Decreasedlevels of IgM(>40mg/dl) is considered diagnostic for IgM deficiency.
* Decreased levels of IgM(>40mg/dl) is considered diagnostic for IgM deficiency<ref name="pmid10600329">{{cite journal| author=Conley ME, Notarangelo LD, Etzioni A| title=Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). | journal=Clin Immunol | year= 1999 | volume= 93 | issue= 3 | pages= 190-7 | pmid=10600329 | doi=10.1006/clim.1999.4799 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10600329  }} </ref>.
* Levels of other immunoglobulins(IgA, IgG, IgE and IgD)are within normal range.
* Levels of other immunoglobulins(IgA, IgG, IgE and IgD)are within normal range.
* TLC may be raised.
* [[White blood cells|White blood cell]] count may be raised.


===Electrocardiogram===
===Electrocardiogram===
Line 279: Line 219:


===X-ray===
===X-ray===
There are no specific findings for IgM deficiency on x ray but signs of lung disease may be present.
There are no specific findings for IgM deficiency on x ray but signs of lung disease may be present<ref name="pmid15908625">{{cite journal| author=Rossi UG, Owens CM| title=The radiology of chronic lung disease in children. | journal=Arch Dis Child | year= 2005 | volume= 90 | issue= 6 | pages= 601-7 | pmid=15908625 | doi=10.1136/adc.2004.051383 | pmc=1720446 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15908625  }} </ref>.
* Increased bronchovascular markings in bronchitis.
* Increased bronchovascular markings in [[bronchitis]].
* '''lung''' hyperinflation with flattened hemidiaphragms in emphysema.
* '''Lung''' hyperinflation with flattened hemidiaphragms in [[emphysema]].
* Consolidation in pneumonia.
* [[Consolidation (medicine)|Consolidation]] in [[pneumonia]].
* Tram track opacities in bronchiectasis.
* Tram track opacities in [[bronchiectasis]].


===Echocardiography or Ultrasound===
===Echocardiography or Ultrasound===
Line 291: Line 231:
* There are no CT scan findings associated with IgM deficiency.
* There are no CT scan findings associated with IgM deficiency.


* Changes of chronic lung disease, if present will be visible on CT and can help differentiate the cause and extent of the disease.
* Changes of [[Copd|chronic lung disease]], if present will be visible on CT and can help differentiate the cause and extent of the disease.
* Chronic sinusitis- mucosal thickening, complete opacification, bone remodeling and thickening due to osteitis, and polyposis.  
* Chronic sinusitis- mucosal thickening, complete opacification, bone remodeling and thickening due to [[osteitis]], and [[Polyp|polyposis]].  


===MRI===
===MRI===
There are no MRI findings associated with IgM deficiency.
There are no MRI findings associated with IgM deficiency.


However, signs of chronic lung disease or chronic sinsuitis may be present.
However, signs of chronic lung disease or chronic [[Sinus|sinsuitis]] may be present.


===Other Imaging Findings===
===Other Imaging Findings===
Line 307: Line 247:
==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
* Asymptomatic individuals do not require any treatment.
* Asymptomatic individuals do not require any treatment<ref name="pmid10600329">{{cite journal| author=Conley ME, Notarangelo LD, Etzioni A| title=Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). | journal=Clin Immunol | year= 1999 | volume= 93 | issue= 3 | pages= 190-7 | pmid=10600329 | doi=10.1006/clim.1999.4799 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10600329  }} </ref><ref name="pmid1274982">{{cite journal| author=Yocum MW, Strong DM, Chusid MJ, Lakin JD| title=Selective immunoglobulin M (IgM) deficiency in two immunodeficient adults with recurrent staphylococcal pyoderma. | journal=Am J Med | year= 1976 | volume= 60 | issue= 4 | pages= 486-94 | pmid=1274982 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1274982  }} </ref><ref name="pmid19494527">{{cite journal| author=Yel L, Ramanuja S, Gupta S| title=Clinical and immunological features in IgM deficiency. | journal=Int Arch Allergy Immunol | year= 2009 | volume= 150 | issue= 3 | pages= 291-8 | pmid=19494527 | doi=10.1159/000222682 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19494527  }} </ref>.
* IVIG can be used in some patients but not effective in all the patients in a dose range of 400 to 600 mg/kg infused every three to four weeks intravenously or subcutaneosly.
* [[Intravenous immunoglobulin|IVIG]] can be used in some patients but not effective in all the patients in a dose range of 400 to 600 mg/kg infused every three to four weeks intravenously or subcutaneosly.
* Conjugated pneumococcal vaccine, conjugated haemophillus influenza B and conjugated meningococcal vaccine administered to prevent infections.
* Conjugated [[pneumococcal vaccine]], conjugated haemophilus [[influenza]] B and conjugated [[Neisseria meningitidis|meningococcal]] vaccine administered to prevent infections.
* Prohphylactic antibiotics can be used for recurrent sinopulmonary infections:
* Prohphylactic [[Antibiotic|antibiotics]] can be used for recurrent sinopulmonary infections:
** Amoxicillin
** [[Amoxicillin]]
** Cefuroxime
** [[Cefuroxime]]
** Trimethoprim/sulphamethoxazole
** [[Trimethoprim]]/sulphamethoxazole
** Clarithromycin/eryhtromycin.
** [[Clarithromycin]]/eryhtromycin.
* Treatment of the infections with the appropriate antibiotics.
* Treatment of the infections with the appropriate antibiotics.
* Treatment of atopic diseases such as allergic rhinits with antihistamines can help in reducing sinopulmonary infections.
* Treatment of [[Atopy|atopic]] diseases such as allergic [[rhinitis]] with [[H1 antihistamine|antihistamines]] can help in reducing sinopulmonary infections.


===Surgery===
===Surgery===
Line 326: Line 266:
===Secondary Prevention===
===Secondary Prevention===
* Secondary prevention includes prevention of infections by:
* Secondary prevention includes prevention of infections by:
** Avoidance- reduce exposure to others with potentially contagious illnesses, proper hand washing and immunization of family members and close contacts.
** Avoidance- reduce exposure to others with potentially contagious illnesses, proper hand washing and [[Vaccination|immunization]] of family members and close contacts.
** Vaccination with conjugate vaccines -conjugated pneumococcal vaccine, conjugated haemophillus influenza B and conjugated meningococcal vaccine administered to prevent infections.
** Vaccination with conjugate vaccines -conjugated pneumococcal vaccine, conjugated haemophillus influenza B and conjugated meningococcal vaccine administered to prevent infections.
** Use of prophylactic broad spectrum antibiotics.
** Use of prophylactic broad spectrum antibiotics.

Latest revision as of 20:28, 24 September 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]

Synonyms and keywords:IgM deficiency

Overview

Immunoglobulin M deficiency is said to be present when the levels of IgM in serum are <40mg/dl while the levels of all other immunoglobulins are within normal range. IgM deficiency was first noted in 1969 by Roy- Chawdhary from a jejunal biopsy. It is of 2 types primary and secondary IgM deficiency. IgM deficiency can occur due to a defect in B cells or T cells. IgM deficiency can lead to chronic infections, development of autoimmune disease and neoplastic diseases. IgM deficiency cab be caused in autoimmune conditions and it can itself lead to the development of autoimmune conditions. IgM deficiency can be differentiated from other immunoglobuiln deficiencies by measuring the levels of all the immunoglobulins.The incidence/prevalence of IgM deficiency is approximately 100-2000 per 100,000 individuals worldwide. The patients usually present with chronic or recurrent infections. Signs and symptoms of chronic lung disease, chronic ear and chronic sinus infections may be present.

Historical Perspective

Classification

There is no established system for the classification of IgM deficiency[2].However, it may present as 2 types:

  • Primary IgM deficiency-
    • There occurs just IgM deficiency and no other associated condition.
    • It is present in children as a congenital condition.
  • Secondary IgM deficiency-
    • Along with IgM deficiency there occurs autoimmune disorders or some neoplasms.
    • It occurs in adults and is acquired.

Pathophysiology

Causes

The exact cause of IgM deficiency is not known.

Differentiating IgM deficiency from Other Diseases

  • IgM deficiency can be differentiated from other diseases of the same kind by measuring the value of other immunoglobulins[12][13][14][15][16].
  • In IgM deficiency only IgM levels are deficIent while the rest are within normal limits.
Disease IgM levels IgG levels IgA levels IgE levels B cell defect T cell defect
IgM deficiency ↓ - - - - -
IgA deficiency - - ↓ - - -
IgG deficiency - ↓ - - - -
IgE deficiency - - - ↓ - -
Hypoproteinemia/Proteinuria ↓ ↓ ↓ ↓ - -
Comined Immunodeficiency ↓ ↓ ↓ ↓ + +
X linked agammaglobulinemia ↓ ↓ ↓ ↓ + -
Hyperimmunoglobulin M syndrome ↑ ↓ ↓ ↓ + -
Common variable immunodeficiency ↓ ↓ ↓ ↓ + -
Wiskott-Aldrich syndrome ↓ ↓ ↑ ↑ - +
Hyper IgE syndrome - - - ↑ - +

Epidemiology and Demographics

  • The incidence of IgM deficiency is approximately 100-2000 per 100,000 individuals worldwide.
  • Primary IgM defiicency is present congenitally in children which presents in age group 3-36 months.
  • Secondary IgM deficiency occurs in adults in age group 30-40, no relation with sex or age.

Risk Factors

There are no established risk factors for IgM deficiency but it occurs more commonly in[17][18]:

Screening

  • There is insufficient data to recommend screening for IgM deficiency.
  • However, in patients presenting with recurrent infections, all immunologlobulins (IgM, IgG, IgA, IgE and IgD) can be measured.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

The diagnosis of IgM deficiency is made by measuring serum IgM levels[24].

  • Serum IgM levels of <40mg/dl is diagnostic of IgM deficiency
  • Serum sample is taken and then anti immunoglobulins are added.
  • The amount of IgM produced is then measured.
  • Other tests include

History and Symptoms

Symptoms

  • The patients may be asymptomatic[28].

Physical Examination

Laboratory Findings

  • Decreased levels of IgM(>40mg/dl) is considered diagnostic for IgM deficiency[24].
  • Levels of other immunoglobulins(IgA, IgG, IgE and IgD)are within normal range.
  • White blood cell count may be raised.

Electrocardiogram

There are no ECG findings associated with IgM defiicency.

X-ray

There are no specific findings for IgM deficiency on x ray but signs of lung disease may be present[31].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with IgM deficiency

CT scan

  • There are no CT scan findings associated with IgM deficiency.
  • Changes of chronic lung disease, if present will be visible on CT and can help differentiate the cause and extent of the disease.
  • Chronic sinusitis- mucosal thickening, complete opacification, bone remodeling and thickening due to osteitis, and polyposis.

MRI

There are no MRI findings associated with IgM deficiency.

However, signs of chronic lung disease or chronic sinsuitis may be present.

Other Imaging Findings

There are no other imaging findings associated with IgM deficiency.

Other Diagnostic Studies

There are no other diagnostic studies associated with IgM deficiency.

Treatment

Medical Therapy

  • Asymptomatic individuals do not require any treatment[24][19][25].
  • IVIG can be used in some patients but not effective in all the patients in a dose range of 400 to 600 mg/kg infused every three to four weeks intravenously or subcutaneosly.
  • Conjugated pneumococcal vaccine, conjugated haemophilus influenza B and conjugated meningococcal vaccine administered to prevent infections.
  • Prohphylactic antibiotics can be used for recurrent sinopulmonary infections:
  • Treatment of the infections with the appropriate antibiotics.
  • Treatment of atopic diseases such as allergic rhinitis with antihistamines can help in reducing sinopulmonary infections.

Surgery

Surgical intervention is not recommended for the management of IgM deficiency.

Primary Prevention

There are no established measures for the primary prevention of IgM deficiency.

Secondary Prevention

  • Secondary prevention includes prevention of infections by:
    • Avoidance- reduce exposure to others with potentially contagious illnesses, proper hand washing and immunization of family members and close contacts.
    • Vaccination with conjugate vaccines -conjugated pneumococcal vaccine, conjugated haemophillus influenza B and conjugated meningococcal vaccine administered to prevent infections.
    • Use of prophylactic broad spectrum antibiotics.

References

  1. ↑ Black CA (1997). "A brief history of the discovery of the immunoglobulins and the origin of the modern immunoglobulin nomenclature". Immunol Cell Biol. 75 (1): 65–8. doi:10.1038/icb.1997.10. PMID 9046436.
  2. ↑ Manson JJ, Mauri C, Ehrenstein MR (2005). "Natural serum IgM maintains immunological homeostasis and prevents autoimmunity". Springer Semin Immunopathol. 26 (4): 425–32. doi:10.1007/s00281-004-0187-x. PMID 15611856.
  3. ↑ Ehrenstein MR, Cook HT, Neuberger MS (2000). "Deficiency in serum immunoglobulin (Ig)M predisposes to development of IgG autoantibodies". J Exp Med. 191 (7): 1253–8. PMC 2193170. PMID 10748243.
  4. ↑ GILBERT C, HONG R (1964). "QUALITATIVE AND QUANTITATIVE IMMUNOGLOBULIN DEFICIENCY". Am J Med. 37: 602–9. PMID 14215847.
  5. ↑ Gonzalez-Quintela A, Alende R, Gude F, Campos J, Rey J, Meijide LM; et al. (2008). "Serum levels of immunoglobulins (IgG, IgA, IgM) in a general adult population and their relationship with alcohol consumption, smoking and common metabolic abnormalities". Clin Exp Immunol. 151 (1): 42–50. doi:10.1111/j.1365-2249.2007.03545.x. PMC 2276914. PMID 18005364.
  6. ↑ Hobbs JR (1975). "IgM deficiency". Birth Defects Orig Artic Ser. 11 (1): 112–6. PMID 50091.
  7. ↑ Baker N, Ehrenstein MR (2002). "Cutting edge: selection of B lymphocyte subsets is regulated by natural IgM". J Immunol. 169 (12): 6686–90. PMID 12471099.
  8. ↑ Inoue T, Okumura Y, Shirama M, Ishibashi H, Kashiwagi S, Okubo H (1986). "Selective partial IgM deficiency: functional assessment of T and B lymphocytes in vitro". J Clin Immunol. 6 (2): 130–5. PMID 2872228.
  9. ↑ Saini S, Dettore AJ, Bhambhani KJ, Buck S, Poulik J, Savaşan S (2011). "Selective IgM deficiency in CD30+ cutaneous lymphoproliferative disorder". J Pediatr Hematol Oncol. 33 (4): e156–9. doi:10.1097/MPH.0b013e31820150d5. PMID 21516014.
  10. ↑ De la Concha EG, Garcia-Rodriguez MC, Zabay JM, Laso MT, Alonso F, Bootello A; et al. (1982). "Functional assessment of T and B lymphocytes in patients with selective IgM deficiency". Clin Exp Immunol. 49 (3): 670–6. PMC 1536738. PMID 6983403.
  11. ↑ Takenaka T, Nakamine H, Nishihara T, Tsuda T, Tsujimoto M, Maeda J (1983). "Prolymphocytic leukemia with IgM hypogammaglobulinemia". Am J Clin Pathol. 80 (2): 237–42. PMID 6603786.
  12. ↑ Thong YH, Maxwell GM (1978). "Primary selective deficiency of immunoglobulin M." Aust N Z J Med. 8 (4): 436–8. PMID 367347.
  13. ↑ Cipe FE, Doğu F, Güloğlu D, Aytekin C, Polat M, Biyikli Z; et al. (2013). "B-cell subsets in patients with transient hypogammaglobulinemia of infancy, partial IgA deficiency, and selective IgM deficiency". J Investig Allergol Clin Immunol. 23 (2): 94–100. PMID 23654075.
  14. ↑ Fischer A (2004). "Human primary immunodeficiency diseases: a perspective". Nat Immunol. 5 (1): 23–30. doi:10.1038/ni1023. PMID 14699405.
  15. ↑ Ozen A, Baris S, Karakoc-Aydiner E, Ozdemir C, Bahceciler NN, Barlan IB (2010). "Outcome of hypogammaglobulinemia in children: immunoglobulin levels as predictors". Clin Immunol. 137 (3): 374–83. doi:10.1016/j.clim.2010.08.010. PMID 20851686.
  16. ↑ Notarangelo L, Casanova JL, Fischer A, Puck J, Rosen F, Seger R; et al. (2004). "Primary immunodeficiency diseases: an update". J Allergy Clin Immunol. 114 (3): 677–87. doi:10.1016/j.jaci.2004.06.044. PMID 15356576.
  17. ↑ Vogelzang NJ, Corwin H, Finlay JL, Pellettiere EV, Luskin AT, Di Camelli RF; et al. (1982). "Clear cell sarcoma and selective IgM deficiency: a case report". Cancer. 49 (2): 234–8. PMID 7053825.
  18. ↑ Kung SJ, Gripp KW, Stephan MJ, Fairchok MP, McGeady SJ (2007). "Selective IgM deficiency and 22q11.2 deletion syndrome". Ann Allergy Asthma Immunol. 99 (1): 87–92. doi:10.1016/S1081-1206(10)60627-8. PMID 17650836.
  19. ↑ 19.0 19.1 Yocum MW, Strong DM, Chusid MJ, Lakin JD (1976). "Selective immunoglobulin M (IgM) deficiency in two immunodeficient adults with recurrent staphylococcal pyoderma". Am J Med. 60 (4): 486–94. PMID 1274982.
  20. ↑ Phuphuakrat A, Ngamjanyaporn P, Nantiruj K, Luangwedchakarn V, Malathum K (2016). "Selective IgM deficiency in an adult presenting with Streptococcus pneumoniae septic arthritis". J Microbiol Immunol Infect. 49 (1): 150–3. doi:10.1016/j.jmii.2013.01.009. PMID 23523054.
  21. ↑ Raziuddin S, Bilal N, Benjamin B (1988). "Transient T-cell abnormality in a selective IgM-immunodeficient patient with Brucella infection". Clin Immunol Immunopathol. 46 (3): 360–7. PMID 2962796.
  22. ↑ Chandra RK, Kaveramma B, Soothill JF (1969). "Generalised non-progressive vaccinia associated with IgM deficiency". Lancet. 1 (7597): 687–9. PMID 4182649.
  23. ↑ Takeuchi T, Nakagawa T, Maeda Y, Hirano S, Sasaki-Hayashi M, Makino S; et al. (2001). "Functional defect of B lymphocytes in a patient with selective IgM deficiency associated with systemic lupus erythematosus". Autoimmunity. 34 (2): 115–22. PMID 11905841.
  24. ↑ 24.0 24.1 24.2 Conley ME, Notarangelo LD, Etzioni A (1999). "Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies)". Clin Immunol. 93 (3): 190–7. doi:10.1006/clim.1999.4799. PMID 10600329.
  25. ↑ 25.0 25.1 Yel L, Ramanuja S, Gupta S (2009). "Clinical and immunological features in IgM deficiency". Int Arch Allergy Immunol. 150 (3): 291–8. doi:10.1159/000222682. PMID 19494527.
  26. ↑ Hong R, Gupta S (2008). "Selective immunoglobulin M deficiency in an adult with Streptococcus pneumoniae sepsis and invasive aspergillosis". J Investig Allergol Clin Immunol. 18 (3): 214–8. PMID 18564634.
  27. ↑ Arahata M, Tajiri K, Nomoto K, Tsuneyama K, Minami S, Shimizu Y (2013). "A novel type of selective immunoglobulin m deficiency in a patient with autoimmune liver cirrhosis with recurrent hepatocellular carcinoma: a case report and review of the literature". Int Arch Allergy Immunol. 161 (1): 91–6. doi:10.1159/000343583. PMID 23257944.
  28. ↑ Morita M, Saibara T, Nakazawa Y, Miyao M, Okazaki K, Onishi S; et al. (1984). "[A case of Crohn's disease associated with selective IgM deficiency]". Nihon Shokakibyo Gakkai Zasshi. 81 (4): 1070–5. PMID 6748317.
  29. ↑ Popa V, Colby TV, Reich SB (2002). "Pulmonary interstitial disease in Ig deficiency". Chest. 122 (5): 1594–603. PMID 12426258.
  30. ↑ Ross IN, Thompson RA (1976). "Severe selective IgM deficiency". J Clin Pathol. 29 (9): 773–7. PMC 476177. PMID 977778.
  31. ↑ Rossi UG, Owens CM (2005). "The radiology of chronic lung disease in children". Arch Dis Child. 90 (6): 601–7. doi:10.1136/adc.2004.051383. PMC 1720446. PMID 15908625.


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