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Latest revision as of 19:30, 10 December 2018

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

Synonyms and keywords:: Selective IgA deficiency, SIgAD

Overview

Selective Immunoglobulin A deficiency (SIgAD) is the most common primary immunodeficiency and is defined as "serum level of IgA equal or below 7mg/dl in the presence of normal level of other immunoglobulins in individuals older than four years of age and in which other causes of hypogammaglobulinemia have been excluded". The first cases with selective IgA deficiency were diagnosed in 1963-64, 10 years after immunoglobulin A was described in the serum by Graber and Williams. SIgAD is more prevalent in caucasians. It is classified based on either the laboratory values of B-cells subsets or the clinical phenotype of individuals with the condition. SIgAD has been attributed to an intrinsic B cell lymphocyte defect, T cell lymphocyte abnormalities and most recently an impairment in cytokine regulation indicating that it is a heterogenous dysfunction but the exact mechanism is still not clear. SIgAD may be genetically transferred but the inheritance pattern is variable. Several studies have reported SIgAD linkage with MHC and non MHC susceptibility genes that are also found in many autoimmune conditions which somewhat explains their association. Majority of patients with SIgAD are asymptomatic. Symptomatic patients may present with Infections such otitis media, sinopulmonary infections, gastrointestinal infections, allergies or autoimmune conditions. Diagnosis is usually based on serum level of immunoglobulin A. There is no specific treatment for selective IgA deficiency but there are several components of its management to prevent the progression and complications such as patient education, vaccination, use of antibiotics and immunoglobulins. The prognosis is generally very good but few cases may progress to common variable immunodeficiency that doesn't predict a favorable outcome.

Historical Perspective

Immunoglobulin A was first discovered in the serum by Graber and Williams in 1953.^[1]
Within 10 years, the first cases with selective IgA deficiency were identified in healthy as well as in patients with ataxia telangiectasia.^[2]^[3]

Classification

Selective IgA deficiency may be classified based on either the laboratory values of B-cells subsets or the clinical phenotype of individuals with the condition.

Classification Based on Memory B cell Population

SIgAD can be classified based on the laboratory values of the B-cell subsets such as naive, IgM memory, switched memory or IgM+CD21- B cells in patients as compared to healthy individuals.^[4]

Percentage of switched Memory B cells (CD 19+, CD 21+, IgD-)

SIgAD1
Less than 0.4% of switched memory B cells (CD19+, CD21+, IgD-)

SIgAD2
Greater than 0.4% of switched memory B cells (CD19+, CD21+, IgD-)

Classification Based on the Clinical Presentation

Health related quality of life(HRQL) can be compromised by the severity of symptoms in patients with selective IgA deficiency. It is classified based on the clinical presentation of the patients suffering from it.^[5]^[6]^[7]^[8]^[9]^[10]^[11]^[12]^[13]^[14]^[15]

Clinical Phenotypes	Description
Asymptomatic	About 60% of the patients are asymptomatic Care should be taken while transfusing blood or blood products in these patients. Regular monitoring i.e every 4-6 months is required as the disease can progress. Patient should be educated but no therapeutic intervention is required.
Minor Infections	About 12% of the patients have minor infections. Infections are usually caused by viral or bacterial agents that normally resolves without any sequel but sometimes it severe infections can occur Investigations for secondary immune defects such as IgG2 subclass deficiency or mannan-binding lectin should be done to find out predisposing factors. A notable decrease in both physical and mental component affecting quality of life ( QoL) is observed in patients with this phenotype.
Allergy	About 15% of the patients with SIgAD have allergic manifestations predominantly. Mental component is affected compromising the quality of life (QoL)in patients of this group. Allergic manifestations are more severe in patients with SIgAD as compared to patients with normal IgA levels so special consideration should be given to the prevention and treatment of these patients. Evaluations of serum IgE level, skin prick test and lung function test are suggested in these patients.
Autoimmune	Evidence of reduced proper mental and physical function affecting the QoL in patients with SIgAD. Idiopathic thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) can be seen in patients with SIgAD requiring blood or blood product administration frequently which should be performed with caution. Other autoantibodies could be found in the serum of some patients with SIgAD without any clinical autoimmune manifestations such as antibodies against thyroglobulin, thyroid microsomal antigens, red blood cells, basement membrane, smooth muscle cells, pancreatic cells, cardiolipin, human collagen, and adrenal cells. Although the presence and certain level of these antibodies do not predict the development of disorders but could be suggestive of future disease.
Severe symptoms	A few percent of the patient with SIgAD develop both recurrent or severe infections and autoimmune disorders fall into severe symptoms phenotype. Associaton with HLA haplotype( A1, B8, DR1) increases the risk to develop severe symptoms and progression to common variable Immunodeficiency (CVID). Mortality rate for this group is twice as compared to the general population. It is proposed that the patient with this phenotype and the family history of both SIgAD and CVID perform the basic clinical and immunologic evaluation and regular follow-up examinations.

Pathophysiology

Pathogenesis

Several studies were carried out to establish the mechanism involved in selective IgA immunodeficiency but the exact pathogensis is still not clear.
SIgAD has been attributed to an intrinsic B cell lymphocyte defect, T cell lymphocyte abnormalities and most recently an impairment in cytokines regulation indicating that it is a heterogenous dysfunction.^[8]^[16]^[17]
The most common pathological process involved in patients with selective immunoglobulin A deficiency is a maturation defect in B cells to produce IgA.^[18]
Normally, the surface immunoglobulins are acquired in a sequential manner in B- cell differentiation. The first surface immunoglobulin to appear on B cells is IgM, as the cells mature they acquire surface IgD and sometimes IgA or IgG. A fully differentiated B cell performs a specfic function which means it would bear a specfic surface immunoglobulin. It is found that Patients with sIgAD have B cells arrested at a stage where they co-express surface IgM, IgD as well as IgA and donot develop into IgA secreting plasma cells.^[19].
The abnormality appears to involve stem cells as it can be passed on by bone marrow transplantation.^[20]
Cytokine dysregulation such as lack of IL-4, IL-6, IL-7, IL-10, TGF-b and most recently IL-21 is suggested to play a role in SIgAD.^[17]^[21]

Genetics

Several genetic mutations are associated with SIgAD which suggest its polygenic nature but whether and how they imply causation is yet to be established.

MHC Susceptibility genes	Non MHC Susceptibility genes
SIgAD association with MHC and Non MHC Genes
HLA class I: HLA-A1, HLA-A28, HLA-B8 and HLA-B14^[22]^[23]	IFIH1 and CLEC16A^[24]
HLA class II: HLA-DR1, HLA-DR3, HLA-DR7^[25]^[26]^[27]^[28]
MHC class III: C2, C4A, C4B and MSH5^[29]

Mutation in tumor necrosis factor receptor superfamily member 13B (TNFRSF13B) gene that encodes for tumor necrosis factor receptor superfamily member 13B (TNFRSF13B) protein also known as "transmembrane activator and calcium-modulator and cyclophilin ligand interactor"(TACI), a molecule responsible for isotype switching in B-cells is also found in this condition.^[30]^[31]

Associated Conditions

Diseases	Description
Common Variable Immunodeficiency	Common genetic basis has been proposed for IgA deficiency and common variable immunodeficiency by their existence in the members of the same family and the underlying B cell defect.^[32] IgA deficiency may progress into combined variable immundeficinecy.^[33]
Autoimmune Conditions	Several autoimmune diseases have been associated with SIgAD.^[34]^[35]^[36]^[37] The most common of them are: Celiac disease Diabetes mellitus type 1 Inflammatory bowel disease Other less common conditions associated are: Graves Disease Rheumatoid arthritis Systemic lupus erythematosus Myasthenia gravis Ulcerative colitis Juvenile idiopathic arthritis Hyperthyreosis Hypothyreosis Idiopathic Pulmonary Hemosiderosis
Ataxia Telangectasia ^[38]^[39]
Risk for Cancer	There is an increased risk of cancer specifically of the gastrointestinal tract in adults. Though children with IgA deficiency are not at increased risk for cancers.^[40]^[41]

Causes

The cause of selective IgA deficiency has not been identified. To review risk factors for the development of this disease, click risk factors.

Differentiating IgA Deficiency from Other Diseases

IgA defieciency should be differentiated from other disorders leading to hypogammaglobulinemia and defects of humoral immunity. The following conditions may be considered as differentials:^[42]^[43]^[44]^[45]^[46]^[47]^[48]^[49]^[50]^[8]^[51]^[52]^[53]^[54]^[55]^[35]^[56]^[57]^[58]^[59]^[60]^[61]^[62]^[63]^[64]^[65]^[66]^[67]^[63]^[68]^[69]^[70]^[71]^[72]^[73]^[74]^[75]^[76]^[77]^[78]^[31]^[79]^[80]^[81]^[82]^[83]^[84]^[85]^[86]^[87]^[88]


Disorder	Defect (Mechanism of Development)	Characteristic Features	Clinical Presentation	Laboratory Findings
X-Linked (Bruton) Agammaglobulinemia	Defect in tyrosine kinase gene (BTK) B cells fail to mature	X-linked recessive pattern of inheritance Increased prevalence in males	Recurrent bacterial and enteroviral infections after 6 months of age Pre-disposition to development of infections by encapsulated organisms Pre-disposition to development of Giardia infections Absent lymph nodes and tonsils	Normal CD19+ B cell count Decreased pro-B cells Increased pre-B cells Decreased immunoglobulins of all classes
Selective IgA Deficiency	Stem cell defect (Transferrable with marrow transplant) Lack of IL-4, IL-6, IL-7, IL-10, TGF-β, and IL-21 Mutations in transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI, TNFRSF13B)	Most common primary immune deficiency	Majority of the cases are asymptomatic Respiratory and gastrointestinal infections (mucosal infections) Associated with autoimmune diseases Atopy Anaphylaxis to IgA containing products	Serum IgA < 7 mg/dl Normal IgG and IgM levels
Common Variable Immunodeficiency	Defective B cell differentiation	May be acquired in 20-30 years of age	May present with other autoimmune diseases Associated with bronchiectasis Associated with lymphoma Associated with sinopulmonary infections (Bacterial, enteroviral and parasitic such as Giardia)	Decreased plasma cells Decreased immunoglobulins
Autosomal dominant hype IgE syndrome (Job's Syndrome)	Defieciency of Th17 cells due to STAT3 mutation Impaired neutrophils to sites of infection	Distinctive coarse facies Cold (non-inflammatory) Staphylococcal abscesses Retained primary teeth Eczema		Increased levels of IgE Decreased levels of interferon gamma (IFN-gamma)
Severe combined immunodeficiency (SCID)	Defective interleukin-2 receptor gamma chain Adenosine deaminase deficiency Reg 1 and Reg 2 nonsense mutations	IL-2R disease is X-linked ADA deficiency and reg mutations are typically autosomal recessive	Failure to thrive Chronic diarrhea Thrush Recurrent bacterial, viral and protozoal infections Treatment is bone marrow transplant	Decreased T cell receptor excision circles (TRECs) Abscence of thymic shadow on chest X-Ray Absent germinal centers of lymph node biopsy Absent T cells on flow cytometry
Ataxia Telangiectasia	Defect in ATM gene DNA double stranded breaks leading to cell cycle arrest	Hypersensitivity to X-Rays	Triad of: Ataxia Spider angiomas (Nests of distended capillaries) IgA deficiency	Increased alpha fetoprotein (AFP) Decreased IgA, IgG and IgE Lymphopenia Cerebellar atrophy
Hyper IgM Syndrome	Defective CD40L (CD40 ligand) on Th cells leading to class switching defect	X-linked recessive pattern of inheritance	Severe pyogenic infections in early life Opportunistic infection with: Pneumocystis jiroveci Cryptosporidium Cytomegalovirus (CMV)	Increased IgM Decreased IgG, IgA and IgE No germinal centers
Wiskott-Aldrich Syndrome	Mutation in WAS gene T cells unable to reorganize actin microfilaments (microfilament defect)	X-linked recessive pattern of inheritance Increased risk of autoimmune disease and malignancy	Thrombocytopenic purpura Eczema Recurrent infections	Decreased to normal IgG and IgM Increased IgE and IgA Fewer and smaller platelets

Malignancy: can cause the reduction in the immunoglobulin production.^[89]
Viral infections: such as Epstein-Barr virus, HIV, cytomegalovirus are other causes of hypogammaglobulinemia.
Side effect of certain medications: Some drugs include systemic glucocorticoids, phenytoin, and carbamazepine, have been associated with IgG deficiency.^[90]
Other causes of primary humoral immunodeficiencies.
Smoking: may cause IgG2 subclass deficiency.^[91]
Protein-losing conditions: enteropathies, nephrotic syndrome, burns, and other traumas may cause abnormal loss of immunoglobulins.

Epidemiology and Demographics

Selective IgA deficency is the most common primary immunodeficiency. It is more common in caucasians with the prevalence rate of 167/100,000^[92]
The incidence of selective IgA deficiency differ based on the ethnic background.

Incidence of selective IgA deficiency in blood donors among different countries
Country	Incidence per 100,000 person years
Czech Republic^[93]	244
Australia^[94]	226
Finland^[95]	200
Iceland^[96]	157
England^[97]	114
Brazil^[98]	104
Iran^[99]	102
Japan^[100]	5

Incidence of selective IgA deficiency in children and young age group among different countries
Country	Incidence per 100,000 person years
Canada^[101]	746
Spain^[102]	613
Turkey^[103]	578
Finland^[104]	531
Nigeria^[105]	520
China^[106]	398
Sweden^[8]	52

High prevalence rate of SIgAD was observed in first degree relatives of symptomatic SIgAD patient with consanguineous marriages.^[107]

Risk Factors

Positive family history of IgA deficiency or common variable immunodeficiency.^[108]
The familial inheritance pattern is variable.^[109]
Moreover penetrance of IgAD in the offspring varies with the gender of the transmitting parent with affected mother being more likely to transfer the disease to her offspring.^[110]

Screening

As high rate of familial inheritance is in families with SIgAD, screening in first-degree relatives of such patients may be performed.^[111]^[112]

Natural History, Complications, and Prognosis

Natural History

Children ≤ 4 years of age may have transient IgA defiecncy and have a full recovery.^[113]
Majority of the patients > 4 years of age with SIgAD remain asymptomatic. Some of them will develop minor infections, allergies, autoimmune conditions and very few cases will have severe symptoms or progress to CVID.^[114]

Complications

SIgAD may progress to common variable immunodeficiency.
Another potential complication of SIgAD is transfusion reaction to blood/blood products or intravenous immunoglobulin therapy.

Prognosis

Prognosis in patients with selective IgA deficiency depends on the clinical phenotype and is generally good as most of the patient are asymptomatic.
Sponatanous recovery has been seen in cases with partial IgA deficiency.^[115]
In rare occasions, the disease may progress to common variable immunodeficiency which doesn't predict a favourable outcome.^[11]^[116]

Diagnostic Criteria

Selective IgA deficiency is a laboratory finding that may not be associated with significant clinical presentaion.
Mainly based on direct measurement of serum IgA levels.
Serum IgA levels ≤ 7 mg/dl in the presence of normal IgG and IgM in patients older than 4 years of age is diagnostic.^[56]
It can be transient finding in children ≤4 years of age.^[117]
There should be high suspicion of SIgAD in patients having blood transfusion reaction.
In addition SIgAD should always be considered as one of the differentials in patients suffering from recurrent infections.

Symptoms

The majority of patients with SIgAD are asymptomatic. Symptomatic patients may present with:^[92]^[114]
- Infections
  - Otitis media
  - Sinopulmonary infections
  - Gastrointestinal infections
- Allergies
- Autoimmune conditions

Physical Examination

Patients with SIgAD usually appear normal but may have physical findings due to associated conditions.

Laboratory Findings

serum IgA ≤ 0.7 mg/dl with normal levels of IgM and IgG.

Electrocardiogram

There are no ECG findings associated with SIgAD.

X-ray

There are no x-ray findings associated with SIgAD.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with SIgAD.

CT scan

There are no CT scan findings associated with SIgAD.

MRI

There are no MRI findings associated with SIgAD.

Other Imaging Findings

No other imaging studies are used to diagnose SIgAD.

Treatment

There is no specific treatment for selective IgA deficiency. Individuals can be managed based on their symptoms as the presentation varies.

Medical Therapy

Antibiotics are used to treat bacterial infections in patients with SIgAD. Prophylactic antibiotics may be used for recurrent infections^[118]
If prophylactic antibiotics fail, rarely, a trial of immunoglobulin replacement therapy with minimal component of IgA may be tried especially in patients with associated antibody or subclass deficiency, though its use in SIgAD is controversial as it can lead to anaphylactic reactions and serum immunoglobulins should always be checked before its administration.^[119].^[120]^[121]^[122].
If blood transfusion is required, IgA deficient or washed blood components should be used.^[123]^[124]
Other treatment options depends on the associated conditions.

Primary Prevention

Selective IgA deficiency is inherited with a variable inheritance pattern. There are no established measures for the primary prevention.

Secondary and Tertiary Prevention

Effective measures for secondary and tertiary prevention of selective IgA deficiency include:
- Patient education
- Vaccination
- Use of prophylactic antibiotics
Patient Education
- Patient with severe IgA deficiency may have anaphylactic reaction secondary to blood transfusion or its products. It is specifically seen in patients with undetectable serum IgA levels. These patients develop anti IgA antibodies so they should be advised to wear medical alert bracelet.^[125]^[121]^[49]
- IgA levels should be periodically monitored in asymptomatic patients.
Vaccination:
- Pneumococcal vaccine is recommended in patients with SIgAD to reduce the risk of sinopulmonary infections^[51].
Use of antibiotics:
- Prophylactic antibiotics can be given to patients with SIgAD to prevent infections and other complications.^[126]

References

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[pmid23886747-87] Lin DD, Barker PB, Lederman HM, Crawford TO (January 2014). "Cerebral abnormalities in adults with ataxia-telangiectasia". AJNR Am J Neuroradiol. 35 (1): 119–23. doi:10.3174/ajnr.A3646. PMC 4106125. PMID 23886747.

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[90] W. B. Klaustermeyer, M. E. Gianos, M. L. Kurohara, H. T. Dao & D. C. Heiner (1992). "IgG subclass deficiency associated with corticosteroids in obstructive lung disease". Chest. 102 (4): 1137–1142. PMID 1343817. Unknown parameter |month= ignored (help)

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[pmid23389234-92] 92.0 ^92.1 Jorgensen GH, Gardulf A, Sigurdsson MI, Sigurdardottir ST, Thorsteinsdottir I, Gudmundsson S, Hammarström L, Ludviksson BR (May 2013). "Clinical symptoms in adults with selective IgA deficiency: a case-control study". J. Clin. Immunol. 33 (4): 742–7. doi:10.1007/s10875-012-9858-x. PMID 23389234.

[pmid11060491-93] Litzman J, Sevcíková I, Stikarovská D, Pikulová Z, Pazdírková A, Lokaj J (October 2000). "IgA deficiency in Czech healthy individuals and selected patient groups". Int. Arch. Allergy Immunol. 123 (2): 177–80. doi:10.1159/000024438. PMID 11060491.

[pmid6968556-94] Wells JV, McNally MP, King MA (August 1980). "Selective IgA deficiency in Australian blood donors". Aust N Z J Med. 10 (4): 410–3. PMID 6968556.

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[pmid304071-97] Holt PD, Tandy NP, Anstee DJ (November 1977). "Screening of blood donors for IgA deficiency: a study of the donor population of south-west England". J. Clin. Pathol. 30 (11): 1007–10. PMC 476624. PMID 304071.

[pmid2816663-98] Carneiro-Sampaio MM, Carbonare SB, Rozentraub RB, de Araújo MN, Riberiro MA, Porto MH (1989). "Frequency of selective IgA deficiency among Brazilian blood donors and healthy pregnant women". Allergol Immunopathol (Madr). 17 (4): 213–6. PMID 2816663.

[pmid19503637-99] Rezvan H, Ahmadi D, Esmailzadeh S, Dayhimi I (April 2009). "Selective deficiency of immunoglobulin A among healthy voluntary blood donors in Iran". Blood Transfus. 7 (2): 152–4. doi:10.2450/2008.0047-08. PMC 2689070. PMID 19503637.

[pmid3485858-100] Kanoh T, Mizumoto T, Yasuda N, Koya M, Ohno Y, Uchino H, Yoshimura K, Ohkubo Y, Yamaguchi H (1986). "Selective IgA deficiency in Japanese blood donors: frequency and statistical analysis". Vox Sang. 50 (2): 81–6. PMID 3485858.

[pmid18487281-101] McGowan KE, Lyon ME, Butzner JD (July 2008). "Celiac disease and IgA deficiency: complications of serological testing approaches encountered in the clinic". Clin. Chem. 54 (7): 1203–9. doi:10.1373/clinchem.2008.103606. PMID 18487281.

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[pmid5118054-104] Savilahti E, Pelkonen P, Visakorpi JK (October 1971). "IgA deficiency in children. A clinical study with special reference to intestinal findings". Arch. Dis. Child. 46 (249): 665–70. PMC 1647824. PMID 5118054.

[pmid2834928-105] Ezeoke AC (March 1988). "Selective IgA deficiency (SIgAD) in Eastern Nigeria". Afr J Med Med Sci. 17 (1): 17–21. PMID 2834928.

[pmid1327440-106] Feng L (February 1992). "[Epidemiological study of selective IgA deficiency among 6 nationalities in China]". Zhonghua Yi Xue Za Zhi (in Chinese). 72 (2): 88–90, 128. PMID 1327440.

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[pmid7586726-108] Vorechovský I, Zetterquist H, Paganelli R, Koskinen S, Webster AD, Björkander J, Smith CI, Hammarström L (November 1995). "Family and linkage study of selective IgA deficiency and common variable immunodeficiency". Clin. Immunol. Immunopathol. 77 (2): 185–92. PMID 7586726.

[pmid29226302-109] Picard C, Bobby Gaspar H, Al-Herz W, Bousfiha A, Casanova JL, Chatila T, Crow YJ, Cunningham-Rundles C, Etzioni A, Franco JL, Holland SM, Klein C, Morio T, Ochs HD, Oksenhendler E, Puck J, Tang M, Tangye SG, Torgerson TR, Sullivan KE (January 2018). "International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity". J. Clin. Immunol. 38 (1): 96–128. doi:10.1007/s10875-017-0464-9. PMC 5742601. PMID 29226302. Vancouver style error: initials (help)

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@@ Line 2: / Line 2: @@
 {{SI}}
-{{CMG}}; {{AE}}
+{{CMG}}; {{AE}} {{Affan}}
 {{SK}}: Selective IgA deficiency, SIgAD
@@ Line 10: / Line 10: @@
 ==Historical Perspective==
-*[[Immunoglobulin A]] was first discovered in the serum by Graber and Williams in 1953.
+*[[Immunoglobulin A]] was first discovered in the serum by Graber and Williams in 1953.<ref name="pmid13041735">{{cite journal |vauthors=GRABAR P, WILLIAMS CA |title=[Method permitting the combined study of the electrophoretic and the immunochemical properties of protein mixtures; application to blood serum] |language=Undetermined |journal=Biochim. Biophys. Acta |volume=10 |issue=1 |pages=193–4 |date=January 1953 |pmid=13041735 |doi= |url=}}</ref>
 * Within 10 years, the first cases with selective IgA deficiency were identified in healthy as well as in patients with [[ataxia telangiectasia]].<ref name="pmid14229760">{{cite journal |vauthors=DUNN HG, MEUWISSEN H, LIVINGSTONE CS, PUMP KK |title=ATAXIA-TELANGIECTASIA |journal=Can Med Assoc J |volume=91 |issue= |pages=1106–18 |date=November 1964 |pmid=14229760 |pmc=1928365 |doi= |url=}}</ref><ref name="pmid4160439">{{cite journal |vauthors=Crabbé PA, Heremans JF |title=Lack of gamma A-immunoglobulin in serum of patients with steatorrhoea |journal=Gut |volume=7 |issue=2 |pages=119–27 |date=April 1966 |pmid=4160439 |pmc=1552348 |doi= |url=}}</ref>
@@ Line 43: / Line 43: @@
 * About 12% of the patients have minor infections.
 * Infections are usually caused by [[viral]] or [[bacterial]] agents that normally resolves without any sequel but sometimes it severe infections can occur Investigations for secondary immune defects such as [[IgG2 subclass deficiency]] or [[mannan-binding lectin]] should be done to find out predisposing factors.
-* A notable decrease in both physical and mental component affecting Quality of life ( QoL) is observed in patients with this phenotype.
+* A notable decrease in both physical and mental component affecting quality of life ( QoL) is observed in patients with this phenotype.
 |-
 | style="background: #DCDCDC; padding: 5px; text-align: center;" | '''Allergy '''
 | style="background: #F5F5F5; padding: 5px;" |
 * About 15% of the patients with SIgAD have allergic manifestations predominantly.
-* Mental component is affected compromising the Quality of life (QoL)in patients of this group.
+* Mental component is affected compromising the quality of life (QoL)in patients of this group.
 * Allergic manifestations are more severe in patients with SIgAD as compared to patients with normal IgA levels  so special consideration should be given to the prevention and treatment of these patients. Evaluations of [[serum IgE level]], [[skin prick test]] and [[lung function test]] are suggested in these patients.
 |-
@@ Line 65: / Line 65: @@
 |}
 __NOTOC__
 ==Pathophysiology==
 ===Pathogenesis===
@@ Line 72: / Line 73: @@
 *Normally, the surface immunoglobulins are acquired in a sequential manner in B- cell differentiation. The first surface immunoglobulin to appear on B cells is [[IgM]], as the cells mature they acquire surface [[IgD]] and sometimes [[IgA]] or [[IgG]]. A fully differentiated B cell performs a specfic function which means it would bear a specfic surface immunoglobulin. It is found that Patients with sIgAD have B cells arrested at a stage where they co-express surface IgM, IgD as well as IgA and donot develop into IgA secreting [[plasma cells]].<ref name="pmid6973088">{{cite journal |vauthors=Conley ME, Cooper MD |title=Immature IgA B cells in IgA-deficient patients |journal=N. Engl. J. Med. |volume=305 |issue=9 |pages=495–7 |date=August 1981 |pmid=6973088 |doi=10.1056/NEJM198108273050905 |url=}}</ref>.
 *The abnormality appears to involve stem cells as it can be passed on by bone marrow transplantation.<ref name="pmid2858666">{{cite journal |vauthors=Hammarström L, Lönnqvist B, Ringdén O, Smith CI, Wiebe T |title=Transfer of IgA deficiency to a bone-marrow-grafted patient with aplastic anaemia |journal=Lancet |volume=1 |issue=8432 |pages=778–81 |date=April 1985 |pmid=2858666 |doi= |url=}}</ref>
-*[[Cytokine]] dysregulation such as lack of [[IL-4]], [[IL-6]], [[IL-7]], [[IL-10]], [[transforming growth factor beta|TGF-b]] and most recently [[IL-21]] is suggested to play a role in SIgAD<ref name="pmid8160012">{{cite journal |vauthors=Ramsay AJ, Husband AJ, Ramshaw IA, Bao S, Matthaei KI, Koehler G, Kopf M |title=The role of interleukin-6 in mucosal IgA antibody responses in vivo |journal=Science |volume=264 |issue=5158 |pages=561–3 |date=April 1994 |pmid=8160012 |doi= |url=}}</ref><ref name="pmid19738033">{{cite journal |vauthors=Borte S, Pan-Hammarström Q, Liu C, Sack U, Borte M, Wagner U, Graf D, Hammarström L |title=Interleukin-21 restores immunoglobulin production ex vivo in patients with common variable immunodeficiency and selective IgA deficiency |journal=Blood |volume=114 |issue=19 |pages=4089–98 |date=November 2009 |pmid=19738033 |doi=10.1182/blood-2009-02-207423 |url=}}</ref>
+*[[Cytokine]] dysregulation such as lack of [[IL-4]], [[IL-6]], [[IL-7]], [[IL-10]], [[transforming growth factor beta|TGF-b]] and most recently [[IL-21]] is suggested to play a role in SIgAD.<ref name="pmid8160012">{{cite journal |vauthors=Ramsay AJ, Husband AJ, Ramshaw IA, Bao S, Matthaei KI, Koehler G, Kopf M |title=The role of interleukin-6 in mucosal IgA antibody responses in vivo |journal=Science |volume=264 |issue=5158 |pages=561–3 |date=April 1994 |pmid=8160012 |doi= |url=}}</ref><ref name="pmid19738033">{{cite journal |vauthors=Borte S, Pan-Hammarström Q, Liu C, Sack U, Borte M, Wagner U, Graf D, Hammarström L |title=Interleukin-21 restores immunoglobulin production ex vivo in patients with common variable immunodeficiency and selective IgA deficiency |journal=Blood |volume=114 |issue=19 |pages=4089–98 |date=November 2009 |pmid=19738033 |doi=10.1182/blood-2009-02-207423 |url=}}</ref>
 ===Genetics===
 * Several genetic mutations are associated with SIgAD which suggest its [[polygenic]] nature but whether and how they imply causation is yet to be established.
@@ Line 90: / Line 91: @@
 |-
 | style="padding: 5px 5px; background: #F5F5F5;" |
-* HLA class II: [[HLA-DR1]], [[HLA-DR3]], [[HLA-DR7]]<ref name="pmid10931389">{{cite journal |vauthors=MacHulla HK, Schönermarck U, Schaaf A, Müller LP, Kloss C, Krüger J, Kunze G, Schönermarck G, Langner J |title=HLA-A, B, Cw and DRB1, DRB3/4/5, DQB1, DPB1 frequencies in German immunoglobulin A-deficient individuals |journal=Scand. J. Immunol. |volume=52 |issue=2 |pages=207–11 |date=August 2000 |pmid=10931389 |doi= |url=}}</ref><ref name="pmid1976229">{{cite journal |vauthors=Olerup O, Smith CI, Hammarström L |title=Different amino acids at position 57 of the HLA-DQ beta chain associated with susceptibility and resistance to IgA deficiency |journal=Nature |volume=347 |issue=6290 |pages=289–90 |date=September 1990 |pmid=1976229 |doi=10.1038/347289a0 |url=}}</ref><ref name="pmid9508785">{{cite journal |vauthors=Schroeder HW, Zhu ZB, March RE, Campbell RD, Berney SM, Nedospasov SA, Turetskaya RL, Atkinson TP, Go RC, Cooper MD, Volanakis JE |title=Susceptibility locus for IgA deficiency and common variable immunodeficiency in the HLA-DR3, -B8, -A1 haplotypes |journal=Mol. Med. |volume=4 |issue=2 |pages=72–86 |date=February 1998 |pmid=9508785 |pmc=2230309 |doi= |url=}}</ref><ref name="pmid9389325">{{cite journal |vauthors=Reil A, Bein G, Machulla HK, Sternberg B, Seyfarth M |title=High-resolution DNA typing in immunoglobulin A deficiency confirms a positive association with DRB1*0301, DQB1*02 haplotypes |journal=Tissue Antigens |volume=50 |issue=5 |pages=501–6 |date=November 1997 |pmid=9389325 |doi= |url=}}</ref>.
+* HLA class II: [[HLA-DR1]], [[HLA-DR3]], [[HLA-DR7]]<ref name="pmid10931389">{{cite journal |vauthors=MacHulla HK, Schönermarck U, Schaaf A, Müller LP, Kloss C, Krüger J, Kunze G, Schönermarck G, Langner J |title=HLA-A, B, Cw and DRB1, DRB3/4/5, DQB1, DPB1 frequencies in German immunoglobulin A-deficient individuals |journal=Scand. J. Immunol. |volume=52 |issue=2 |pages=207–11 |date=August 2000 |pmid=10931389 |doi= |url=}}</ref><ref name="pmid1976229">{{cite journal |vauthors=Olerup O, Smith CI, Hammarström L |title=Different amino acids at position 57 of the HLA-DQ beta chain associated with susceptibility and resistance to IgA deficiency |journal=Nature |volume=347 |issue=6290 |pages=289–90 |date=September 1990 |pmid=1976229 |doi=10.1038/347289a0 |url=}}</ref><ref name="pmid9508785">{{cite journal |vauthors=Schroeder HW, Zhu ZB, March RE, Campbell RD, Berney SM, Nedospasov SA, Turetskaya RL, Atkinson TP, Go RC, Cooper MD, Volanakis JE |title=Susceptibility locus for IgA deficiency and common variable immunodeficiency in the HLA-DR3, -B8, -A1 haplotypes |journal=Mol. Med. |volume=4 |issue=2 |pages=72–86 |date=February 1998 |pmid=9508785 |pmc=2230309 |doi= |url=}}</ref><ref name="pmid9389325">{{cite journal |vauthors=Reil A, Bein G, Machulla HK, Sternberg B, Seyfarth M |title=High-resolution DNA typing in immunoglobulin A deficiency confirms a positive association with DRB1*0301, DQB1*02 haplotypes |journal=Tissue Antigens |volume=50 |issue=5 |pages=501–6 |date=November 1997 |pmid=9389325 |doi= |url=}}</ref>
 | style="padding: 5px 5px; background: #F5F5F5;" |
 |-
@@ Line 139: / Line 140: @@
 ==Causes==
-The cause of selective IgA deficiency has not been identified. To review risk factors for the development of this disease, click [[Selective immunoglobulin A deficiency#Risk Factors| risk factors]].
+* The cause of selective IgA deficiency has not been identified. To review risk factors for the development of this disease, click [[Selective immunoglobulin A deficiency#Risk Factors| risk factors]].
 ==Differentiating IgA Deficiency from Other Diseases==
-IgA defieciency should be differentiated from other disorders leading to [[hypogammaglobulinemia]] and defects of [[humoral immunity]]. The following conditions may be considered as differentials:<ref name="pmid17910333">{{cite journal |vauthors=Agarwal S, Cunningham-Rundles C |title=Assessment and clinical interpretation of reduced IgG values |journal=Ann. Allergy Asthma Immunol. |volume=99 |issue=3 |pages=281–3 |date=September 2007 |pmid=17910333 |pmc=3099256 |doi=10.1016/S1081-1206(10)60665-5 |url=}}</ref><ref name="pmid7679206">{{cite journal |vauthors=Korthäuer U, Graf D, Mages HW, Brière F, Padayachee M, Malcolm S, Ugazio AG, Notarangelo LD, Levinsky RJ, Kroczek RA |title=Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM |journal=Nature |volume=361 |issue=6412 |pages=539–41 |date=February 1993 |pmid=7679206 |doi=10.1038/361539a0 |url=}}</ref><ref name="pmid9255191">{{cite journal |vauthors=Levy J, Espanol-Boren T, Thomas C, Fischer A, Tovo P, Bordigoni P, Resnick I, Fasth A, Baer M, Gomez L, Sanders EA, Tabone MD, Plantaz D, Etzioni A, Monafo V, Abinun M, Hammarstrom L, Abrahamsen T, Jones A, Finn A, Klemola T, DeVries E, Sanal O, Peitsch MC, Notarangelo LD |title=Clinical spectrum of X-linked hyper-IgM syndrome |journal=J. Pediatr. |volume=131 |issue=1 Pt 1 |pages=47–54 |date=July 1997 |pmid=9255191 |doi= |url=}}</ref><ref name="pmid14663287">{{cite journal |vauthors=Winkelstein JA, Marino MC, Ochs H, Fuleihan R, Scholl PR, Geha R, Stiehm ER, Conley ME |title=The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients |journal=Medicine (Baltimore) |volume=82 |issue=6 |pages=373–84 |date=November 2003 |pmid=14663287 |doi=10.1097/01.md.0000100046.06009.b0 |url=}}</ref><ref name="pmid10352287">{{cite journal |vauthors=Subauste CS, Wessendarp M, Sorensen RU, Leiva LE |title=CD40-CD40 ligand interaction is central to cell-mediated immunity against Toxoplasma gondii: patients with hyper IgM syndrome have a defective type 1 immune response that can be restored by soluble CD40 ligand trimer |journal=J. 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+* IgA defieciency should be differentiated from other disorders leading to [[hypogammaglobulinemia]] and defects of [[humoral immunity]]. The following conditions may be considered as differentials:<ref name="pmid17910333">{{cite journal |vauthors=Agarwal S, Cunningham-Rundles C |title=Assessment and clinical interpretation of reduced IgG values |journal=Ann. Allergy Asthma Immunol. |volume=99 |issue=3 |pages=281–3 |date=September 2007 |pmid=17910333 |pmc=3099256 |doi=10.1016/S1081-1206(10)60665-5 |url=}}</ref><ref name="pmid7679206">{{cite journal |vauthors=Korthäuer U, Graf D, Mages HW, Brière F, Padayachee M, Malcolm S, Ugazio AG, Notarangelo LD, Levinsky RJ, Kroczek RA |title=Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM |journal=Nature |volume=361 |issue=6412 |pages=539–41 |date=February 1993 |pmid=7679206 |doi=10.1038/361539a0 |url=}}</ref><ref name="pmid9255191">{{cite journal |vauthors=Levy J, Espanol-Boren T, Thomas C, Fischer A, Tovo P, Bordigoni P, Resnick I, Fasth A, Baer M, Gomez L, Sanders EA, Tabone MD, Plantaz D, Etzioni A, Monafo V, Abinun M, Hammarstrom L, Abrahamsen T, Jones A, Finn A, Klemola T, DeVries E, Sanal O, Peitsch MC, Notarangelo LD |title=Clinical spectrum of X-linked hyper-IgM syndrome |journal=J. Pediatr. |volume=131 |issue=1 Pt 1 |pages=47–54 |date=July 1997 |pmid=9255191 |doi= |url=}}</ref><ref name="pmid14663287">{{cite journal |vauthors=Winkelstein JA, Marino MC, Ochs H, Fuleihan R, Scholl PR, Geha R, Stiehm ER, Conley ME |title=The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients |journal=Medicine (Baltimore) |volume=82 |issue=6 |pages=373–84 |date=November 2003 |pmid=14663287 |doi=10.1097/01.md.0000100046.06009.b0 |url=}}</ref><ref name="pmid10352287">{{cite journal |vauthors=Subauste CS, Wessendarp M, Sorensen RU, Leiva LE |title=CD40-CD40 ligand interaction is central to cell-mediated immunity against Toxoplasma gondii: patients with hyper IgM syndrome have a defective type 1 immune response that can be restored by soluble CD40 ligand trimer |journal=J. Immunol. |volume=162 |issue=11 |pages=6690–700 |date=June 1999 |pmid=10352287 |doi= |url=}}</ref><ref name="pmid8993019">{{cite journal |vauthors=Hayward AR, Levy J, Facchetti F, Notarangelo L, Ochs HD, Etzioni A, Bonnefoy JY, Cosyns M, Weinberg A |title=Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM |journal=J. Immunol. |volume=158 |issue=2 |pages=977–83 |date=January 1997 |pmid=8993019 |doi= |url=}}</ref><ref name="pmid20180797">{{cite journal |vauthors=Davies EG, Thrasher AJ |title=Update on the hyper immunoglobulin M syndromes |journal=Br. J. Haematol. |volume=149 |issue=2 |pages=167–80 |date=April 2010 |pmid=20180797 |pmc=2855828 |doi=10.1111/j.1365-2141.2010.08077.x |url=}}</ref><ref name="pmid20101521">{{cite journal |vauthors=Yel L |title=Selective IgA deficiency |journal=J. Clin. Immunol. |volume=30 |issue=1 |pages=10–6 |date=January 2010 |pmid=20101521 |pmc=2821513 |doi=10.1007/s10875-009-9357-x |url=}}</ref><ref name="pmid19153537">{{cite journal |vauthors=Suzuki H, Kaneko H, Fukao T, Jin R, Kawamoto N, Asano T, Matsui E, Kasahara K, Kondo N |title=Various expression patterns of alpha1 and alpha2 genes in IgA deficiency |journal=Allergol Int |volume=58 |issue=1 |pages=111–7 |date=March 2009 |pmid=19153537 |doi=10.2332/allergolint.O-08-549 |url=}}</ref><ref name="pmid11720003">{{cite journal |vauthors=Cunningham-Rundles C |title=Physiology of IgA and IgA deficiency |journal=J. Clin. Immunol. |volume=21 |issue=5 |pages=303–9 |date=September 2001 |pmid=11720003 |doi= |url=}}</ref><ref name="pmid15093556">{{cite journal |vauthors=Edwards E, Razvi S, Cunningham-Rundles C |title=IgA deficiency: clinical correlates and responses to pneumococcal vaccine |journal=Clin. Immunol. |volume=111 |issue=1 |pages=93–7 |date=April 2004 |pmid=15093556 |doi=10.1016/j.clim.2003.12.005 |url=}}</ref><ref name="pmid305332">{{cite journal |vauthors=Chipps BE, Talamo RC, Winkelstein JA |title=IgA deficiency, recurrent pneumonias, and bronchiectasis |journal=Chest |volume=73 |issue=4 |pages=519–26 |date=April 1978 |pmid=305332 |doi= |url=}}</ref><ref name="pmid5056860">{{cite journal |vauthors=Zinneman HH, Kaplan AP |title=The association of giardiasis with reduced intestinal secretory immunoglobulin A |journal=Am J Dig Dis |volume=17 |issue=9 |pages=793–7 |date=September 1972 |pmid=5056860 |doi= |url=}}</ref><ref name="pmid18683032">{{cite journal |vauthors=Aghamohammadi A, Cheraghi T, Gharagozlou M, Movahedi M, Rezaei N, Yeganeh M, Parvaneh N, Abolhassani H, Pourpak Z, Moin M |title=IgA deficiency: correlation between clinical and immunological phenotypes |journal=J. Clin. 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Immunol. |volume=28 Suppl 1 |issue= |pages=S56–61 |date=May 2008 |pmid=18202833 |doi=10.1007/s10875-007-9163-2 |url=}}</ref><ref name="pmid10600329">{{cite journal |vauthors=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. |volume=93 |issue=3 |pages=190–7 |date=December 1999 |pmid=10600329 |doi=10.1006/clim.1999.4799 |url=}}</ref><ref name="pmid4012343">{{cite journal |vauthors=Mayer RJ, Schiffer CA, Peterson BA, Silver RT, Cornwell GG, McIntyre OR, Rai KR, Budman DR, Ellison RR, Maguire M |title=Intensive postremission therapy in adults with acute nonlymphocytic leukemia with ara-C by continuous infusion or bolus administration: preliminary results of a CALGB phase I study |journal=Semin. 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 {| class="wikitable"
 |+
@@ Line 319: / Line 320: @@
 }}</ref>
 *Protein-losing conditions: enteropathies, nephrotic syndrome, burns, and other traumas may cause abnormal loss of immunoglobulins.
 ==Epidemiology and Demographics==
 * Selective IgA deficency is the most common [[primary immunodeficiency]]. It is more common in [[caucasians]] with the [[prevalence]] rate of 167/100,000<ref name="pmid23389234">{{cite journal |vauthors=Jorgensen GH, Gardulf A, Sigurdsson MI, Sigurdardottir ST, Thorsteinsdottir I, Gudmundsson S, Hammarström L, Ludviksson BR |title=Clinical symptoms in adults with selective IgA deficiency: a case-control study |journal=J. Clin. Immunol. |volume=33 |issue=4 |pages=742–7 |date=May 2013 |pmid=23389234 |doi=10.1007/s10875-012-9858-x |url=}}</ref>
@@ Line 390: / Line 392: @@
 ==Screening==
-* As high rate of familial inheritance is in families with SIgAD, so It is recommended to do the screening in first-degree relatives of such patients.<ref name="pmid29978731">{{cite journal |vauthors=Karaca NE, Severcan EU, Bilgin BG, Azarsiz E, Akarcan S, Gunaydın NC, Gulez N, Genel F, Aksu G, Kutukculer N |title=Familial inheritance and screening of first-degree relatives in common variable immunodeficiency and immunoglobulin A deficiency patients |journal=Int J Immunopathol Pharmacol |volume=32 |issue= |pages=2058738418779458 |date=2018 |pmid=29978731 |pmc=6073834 |doi=10.1177/2058738418779458 |url=}}</ref>
+* As high rate of familial inheritance is in families with SIgAD, screening in first-degree relatives of such patients may be performed.<ref name="pmid29978731">{{cite journal |vauthors=Karaca NE, Severcan EU, Bilgin BG, Azarsiz E, Akarcan S, Gunaydın NC, Gulez N, Genel F, Aksu G, Kutukculer N |title=Familial inheritance and screening of first-degree relatives in common variable immunodeficiency and immunoglobulin A deficiency patients |journal=Int J Immunopathol Pharmacol |volume=32 |issue= |pages=2058738418779458 |date=2018 |pmid=29978731 |pmc=6073834 |doi=10.1177/2058738418779458 |url=}}</ref><ref name="pmid26033741">{{cite journal |vauthors=Soler-Palacín P, Cobos-Carrascosa E, Martín-Nalda A, Caracseghi F, Hernández M, Figueras-Nadal C |title=[Is familial screening useful in selective immunoglobulin A deficiency?] |language=Spanish; Castilian |journal=An Pediatr (Barc) |volume=84 |issue=2 |pages=70–8 |date=February 2016 |pmid=26033741 |doi=10.1016/j.anpedi.2015.04.017 |url=}}</ref>
 ==Natural History, Complications, and Prognosis==
 '''Natural History'''
-* Children less than 4 years of age may have transient IgA defiecncy and have a full recovery.<ref name="pmid25648982">{{cite journal |vauthors=Nurkic J, Numanovic F, Arnautalic L, Tihic N, Halilovic D, Jahic M |title=Diagnostic significance of reduced IgA in children |journal=Med Arch |volume=68 |issue=6 |pages=381–3 |date=December 2014 |pmid=25648982 |pmc=4314178 |doi=10.5455/medarh.2014.68.381-383 |url=}}</ref>
+* Children ≤ 4 years of age may have transient IgA defiecncy and have a full recovery.<ref name="pmid25648982">{{cite journal |vauthors=Nurkic J, Numanovic F, Arnautalic L, Tihic N, Halilovic D, Jahic M |title=Diagnostic significance of reduced IgA in children |journal=Med Arch |volume=68 |issue=6 |pages=381–3 |date=December 2014 |pmid=25648982 |pmc=4314178 |doi=10.5455/medarh.2014.68.381-383 |url=}}</ref>
-* Majority of the patients older than 4 years of age with SIgAD remain [[asymptomatic]]. Some of them will develop minor [[infections]], [[allergies]], [[autoimmune]] conditions and very few cases will have severe symptoms or progress to [[CVID]].<ref name="pmid22547079">{{cite journal |vauthors=Aytekin C, Tuygun N, Gokce S, Dogu F, Ikinciogullari A |title=Selective IgA deficiency: clinical and laboratory features of 118 children in Turkey |journal=J. Clin. Immunol. |volume=32 |issue=5 |pages=961–6 |date=October 2012 |pmid=22547079 |doi=10.1007/s10875-012-9702-3 |url=}}</ref>
+* Majority of the patients > 4 years of age with SIgAD remain [[asymptomatic]]. Some of them will develop minor [[infections]], [[allergies]], [[autoimmune]] conditions and very few cases will have severe symptoms or progress to [[CVID]].<ref name="pmid22547079">{{cite journal |vauthors=Aytekin C, Tuygun N, Gokce S, Dogu F, Ikinciogullari A |title=Selective IgA deficiency: clinical and laboratory features of 118 children in Turkey |journal=J. Clin. Immunol. |volume=32 |issue=5 |pages=961–6 |date=October 2012 |pmid=22547079 |doi=10.1007/s10875-012-9702-3 |url=}}</ref>
 '''Complications'''
 * SIgAD may progress to [[common variable immunodeficiency]].
@@ Line 401: / Line 403: @@
 '''Prognosis'''
 * Prognosis in patients with selective IgA deficiency depends on the clinical [[phenotype]] and is generally good as most of the patient are [[asymptomatic]].
-* Sponatanous recovery has been seen in rare cases with partial IgA deficiency.<ref name="pmid2870316">{{cite journal |vauthors=Plebani A, Ugazio AG, Monafo V, Burgio GR |title=Clinical heterogeneity and reversibility of selective immunoglobulin A deficiency in 80 children |journal=Lancet |volume=1 |issue=8485 |pages=829–31 |date=April 1986 |pmid=2870316 |doi= |url=}}</ref>
+* Sponatanous recovery has been seen in cases with partial IgA deficiency.<ref name="pmid2870316">{{cite journal |vauthors=Plebani A, Ugazio AG, Monafo V, Burgio GR |title=Clinical heterogeneity and reversibility of selective immunoglobulin A deficiency in 80 children |journal=Lancet |volume=1 |issue=8485 |pages=829–31 |date=April 1986 |pmid=2870316 |doi= |url=}}</ref>
-* In rare occasions, the disease may progress to [[common variable immunodeficiency]] which doesn't predict a favourable outcome..<ref name="pmid18520152">{{cite journal |vauthors=Aghamohammadi A, Mohammadi J, Parvaneh N, Rezaei N, Moin M, Espanol T, Hammarstrom L |title=Progression of selective IgA deficiency to common variable immunodeficiency |journal=Int. Arch. Allergy Immunol. |volume=147 |issue=2 |pages=87–92 |date=2008 |pmid=18520152 |doi=10.1159/000135694 |url=}}</ref><ref name="pmid8939274">{{cite journal |vauthors=Litzman J, Burianova M, Thon V, Lokaj J |title=Progression of selective IgA deficiency to common variable immunodeficiency in a 16 year old boy |journal=Allergol Immunopathol (Madr) |volume=24 |issue=4 |pages=174–6 |date=1996 |pmid=8939274 |doi= |url=}}</ref>
+* In rare occasions, the disease may progress to [[common variable immunodeficiency]] which doesn't predict a favourable outcome.<ref name="pmid18520152">{{cite journal |vauthors=Aghamohammadi A, Mohammadi J, Parvaneh N, Rezaei N, Moin M, Espanol T, Hammarstrom L |title=Progression of selective IgA deficiency to common variable immunodeficiency |journal=Int. Arch. Allergy Immunol. |volume=147 |issue=2 |pages=87–92 |date=2008 |pmid=18520152 |doi=10.1159/000135694 |url=}}</ref><ref name="pmid8939274">{{cite journal |vauthors=Litzman J, Burianova M, Thon V, Lokaj J |title=Progression of selective IgA deficiency to common variable immunodeficiency in a 16 year old boy |journal=Allergol Immunopathol (Madr) |volume=24 |issue=4 |pages=174–6 |date=1996 |pmid=8939274 |doi= |url=}}</ref>
 __NOTOC__
@@ Line 408: / Line 410: @@
 * Selective IgA deficiency is a laboratory finding that may not be associated with significant clinical presentaion.
 * Mainly based on direct measurement of serum IgA levels.
-* Serum IgA levels less than 0.7 mg/dl in the presence of normal [[IgG]] and [[IgM]] is diagnostic<ref name="pmid10600329">{{cite journal |vauthors=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. |volume=93 |issue=3 |pages=190–7 |date=December 1999 |pmid=10600329 |doi=10.1006/clim.1999.4799 |url=}}</ref>.
+* Serum IgA levels ≤ 7 mg/dl in the presence of normal [[IgG]] and [[IgM]] in patients older than 4 years of age is diagnostic.<ref name="pmid10600329">{{cite journal |vauthors=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. |volume=93 |issue=3 |pages=190–7 |date=December 1999 |pmid=10600329 |doi=10.1006/clim.1999.4799 |url=}}</ref>
+* It can be transient finding in children ≤4 years of age.<ref name="pmid7418266">{{cite journal |vauthors=Ostergaard PA |title=Clinical and immunological features of transient IgA deficiency in children |journal=Clin. Exp. Immunol. |volume=40 |issue=3 |pages=561–5 |date=June 1980 |pmid=7418266 |pmc=1538944 |doi= |url=}}</ref>
 * There should be high suspicion of SIgAD in patients having [[blood transfusion]] reaction.
 * In addition SIgAD should always be considered as one of the differentials in patients suffering from recurrent infections.
 ===Symptoms===
-*The majority of patients with SIgAD are [[asymptomatic]]. [[Symptomatic]] patients may present with<ref name="pmid23389234">{{cite journal |vauthors=Jorgensen GH, Gardulf A, Sigurdsson MI, Sigurdardottir ST, Thorsteinsdottir I, Gudmundsson S, Hammarström L, Ludviksson BR |title=Clinical symptoms in adults with selective IgA deficiency: a case-control study |journal=J. Clin. Immunol. |volume=33 |issue=4 |pages=742–7 |date=May 2013 |pmid=23389234 |doi=10.1007/s10875-012-9858-x |url=}}</ref><ref name="pmid22547079">{{cite journal |vauthors=Aytekin C, Tuygun N, Gokce S, Dogu F, Ikinciogullari A |title=Selective IgA deficiency: clinical and laboratory features of 118 children in Turkey |journal=J. Clin. Immunol. |volume=32 |issue=5 |pages=961–6 |date=October 2012 |pmid=22547079 |doi=10.1007/s10875-012-9702-3 |url=}}</ref>:
+*The majority of patients with SIgAD are [[asymptomatic]]. [[Symptomatic]] patients may present with:<ref name="pmid23389234">{{cite journal |vauthors=Jorgensen GH, Gardulf A, Sigurdsson MI, Sigurdardottir ST, Thorsteinsdottir I, Gudmundsson S, Hammarström L, Ludviksson BR |title=Clinical symptoms in adults with selective IgA deficiency: a case-control study |journal=J. Clin. Immunol. |volume=33 |issue=4 |pages=742–7 |date=May 2013 |pmid=23389234 |doi=10.1007/s10875-012-9858-x |url=}}</ref><ref name="pmid22547079">{{cite journal |vauthors=Aytekin C, Tuygun N, Gokce S, Dogu F, Ikinciogullari A |title=Selective IgA deficiency: clinical and laboratory features of 118 children in Turkey |journal=J. Clin. Immunol. |volume=32 |issue=5 |pages=961–6 |date=October 2012 |pmid=22547079 |doi=10.1007/s10875-012-9702-3 |url=}}</ref>
 ** [[Infections]]
 *** [[Otitis media]]
@@ Line 422: / Line 425: @@
 ===Physical Examination===
-Patients with SIgAD usually appear normal but may have physical findings due to associated conditions.
+* Patients with SIgAD usually appear normal but may have physical findings due to associated conditions.
 ===Laboratory Findings===
-* serum IgA less than 0.7 mg/dl with normal levels of [[IgM]] and [[IgG]].
+* serum IgA ≤ 0.7 mg/dl with normal levels of [[IgM]] and [[IgG]].
 ===Electrocardiogram===
 There are no [[ECG]] findings associated with SIgAD.
 ===X-ray===
-There are no [[x-ray]] findings associated with SIgAD.
+* There are no [[x-ray]] findings associated with SIgAD.
 ===Echocardiography or Ultrasound===
-There are no [[echocardiography]]/[[ultrasound]] findings associated with SIgAD.
+* There are no [[echocardiography]]/[[ultrasound]] findings associated with SIgAD.
 ===CT scan===
-There are no [[CT scan]] findings associated with SIgAD.
+* There are no [[CT scan]] findings associated with SIgAD.
 ===MRI===
-There are no [[MRI findings]] associated with SIgAD.
+* There are no [[MRI findings]] associated with SIgAD.
 ===Other Imaging Findings===
 * No other imaging studies are used to diagnose SIgAD.
@@ Line 445: / Line 448: @@
 * [[Antibiotics]] are used to treat [[bacterial infections]] in patients with SIgAD. Prophylactic antibiotics may be used for [[recurrent infections]]<ref name="pmid9310611">{{cite journal |vauthors=Gustafson R, Gardulf A, Granert C, Hansen S, Hammarström L |title=Prophylactic therapy for selective IgA deficiency |journal=Lancet |volume=350 |issue=9081 |pages=865 |date=September 1997 |pmid=9310611 |doi=10.1016/S0140-6736(05)62034-X |url=}}</ref>
 * If prophylactic antibiotics fail, rarely, a trial of [[immunoglobulin]] replacement therapy with minimal component of IgA may be tried especially in patients with associated antibody or subclass deficiency, though its use in SIgAD is controversial as it can lead to anaphylactic reactions and serum immunoglobulins should always be checked before its administration.<ref name="pmid15945566">{{cite journal |vauthors=Bonilla FA, Bernstein IL, Khan DA, Ballas ZK, Chinen J, Frank MM, Kobrynski LJ, Levinson AI, Mazer B, Nelson RP, Orange JS, Routes JM, Shearer WT, Sorensen RU |title=Practice parameter for the diagnosis and management of primary immunodeficiency |journal=Ann. Allergy Asthma Immunol. |volume=94 |issue=5 Suppl 1 |pages=S1–63 |date=May 2005 |pmid=15945566 |doi= |url=}}</ref>.<ref name="pmid25428649">{{cite journal |vauthors=Albin S, Cunningham-Rundles C |title=An update on the use of immunoglobulin for the treatment of immunodeficiency disorders |journal=Immunotherapy |volume=6 |issue=10 |pages=1113–26 |date=2014 |pmid=25428649 |pmc=4324501 |doi=10.2217/imt.14.67 |url=}}</ref><ref name="pmid3945295">{{cite journal |vauthors=Burks AW, Sampson HA, Buckley RH |title=Anaphylactic reactions after gamma globulin administration in patients with hypogammaglobulinemia. Detection of IgE antibodies to IgA |journal=N. Engl. J. Med. |volume=314 |issue=9 |pages=560–4 |date=February 1986 |pmid=3945295 |doi=10.1056/NEJM198602273140907 |url=}}</ref><ref name="pmid17761041">{{cite journal |vauthors=Hobbs JR |title=Further aspects of human immunoglobulin A deficiency |journal=Ann. Clin. Biochem. |volume=44 |issue=Pt 5 |pages=496–7 |date=September 2007 |pmid=17761041 |doi=10.1258/000456307781645950 |url=}}</ref>.
-* If [[blood transfusion]] is required, IgA deficient or washed blood components should be used.<ref name="pmid9544978">{{cite journal |vauthors=Rogers RL, Javed TA, Ross RE, Virella G, Stuart RK, Frei-Lahr D |title=Transfusion management of an IgA deficient patient with anti-IgA and incidental correction of IgA deficiency after allogeneic bone marrow transplantation |journal=Am. J. Hematol. |volume=57 |issue=4 |pages=326–30 |date=April 1998 |pmid=9544978 |doi= |url=}}</ref>
+* If [[blood transfusion]] is required, IgA deficient or washed blood components should be used.<ref name="pmid9544978">{{cite journal |vauthors=Rogers RL, Javed TA, Ross RE, Virella G, Stuart RK, Frei-Lahr D |title=Transfusion management of an IgA deficient patient with anti-IgA and incidental correction of IgA deficiency after allogeneic bone marrow transplantation |journal=Am. J. Hematol. |volume=57 |issue=4 |pages=326–30 |date=April 1998 |pmid=9544978 |doi= |url=}}</ref><ref name="pmid15679454">{{cite journal |vauthors=Vassallo RR |title=Review: IgA anaphylactic transfusion reactions. Part I. Laboratory diagnosis, incidence, and supply of IgA-deficient products |journal=Immunohematology |volume=20 |issue=4 |pages=226–33 |date=2004 |pmid=15679454 |doi= |url=}}</ref>
 * Other treatment options depends on the associated conditions.