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PubMed1978

Distribution of IgA 1 and IgA 2 plasma cells in various normal human tissues and in the jejunum of plasma IgA-deficient patients.

The distribution of IgA 1 and IgA 2 plasma cells was studied in normal human tissues. IgA 2 is a minor constituent in peripheral lymph nodes as well as in serum and in bone marrow plasma cells. An increased proportion of IgA 2 plasma cells was observed in gastric and intestinal mucosa, as well as in bronchial mucosa and salivary and mammary glands. Tonsils and mesenteric lymph nodes exhibit values intermediate between those of central and peripheral lymphoid systems. In patients with plasma IgA-deficiency, IgA 2 is the predominant intestinal IgA plasma cells. This may explain the frequent association of an asymptomatic condition and plasma IgA deficiency.

Adolescent↗
PubMed1985

The wasted mutant mouse. I. An animal model of secretory IgA deficiency with normal serum IgA.

The wasted (wst) mutation was recently described as a spontaneous, recessive mutation leading to pathologic changes affecting both the neurologic and the immune systems of wst/wst homozygotes, which presented symptoms analogous to those observed in patients with ataxia-telangiectasia (A.T.). We studied the IgA system of wst/wst mutants and their normal littermates to determine whether IgA deficiency commonly found in A.T. patients was also affecting these mutants. Interestingly, although IgA plasma cells were totally absent from their entire (small and large) intestine, their serum contained a normal level of IgA with a normal ratio of monomeric vs polymeric IgA. The absence of gut IgA plasma cells was not due to malnutrition and was not compensated by the appearance of cells secreting any other isotypes. Studies at the precursor cell level showed the absence of IgA-specific B cell precursors in the Peyer's patches, whereas sIgA B cells and IgA plasma cells were found in normal numbers in the spleen of wasted mice. These data suggest that secretory and serum IgA may comprise distinct systems and that the wasted mutant mouse is a potential model for the study of the physiology and regulation of IgA production.

Animals↗
PubMed1998

[A case report of selective IgA deficiency in rheumatoid arthritis and anti-IgA antibody induced anaphylactic transfusion].

We described a case of rheumatoid arthritis (RA) with selective IgA deficiency. A 69 year-old female with RA was admitted because of gall bladder cancer, and also had selective IgA deficiency which serum IgA level was less than 5.0 mg/dl, and IgA 1 and IgA 2 subclasses were not detected. Prior to the operation, she was given red cell compatible blood transfusion because of severe anemia. After 30 min of transfusion, she developed chill, nausea, vomiting and hypotension. These anaphylactic reactions might be induced by the presence of anti-IgA antibody, since the level of this antibody titers in her serum was elevated, assessed by the methods of ELISA and Western blotting. Although a case of RA associated with selective IgA deficiency, and also with elevated serum anti-IgA antibody level is extremely uncommon, attention should be paid to the presence of anti-IgA antibody in patients with selective IgA deficiency to avoid any unexpected anaphylactic reactions.

Aged↗
PubMed1989

Recurrent extended HLA haplotypes in children with selective IgA deficiency.

HLA supratypes as well as serum IgG, IgA and IgM levels were determined in 44 children and adolescents with severe IgA deficiency (serum IgA less than 5 mg/dl) and in first degree relatives. Frequencies of the HLA alleles B14, DR1, DQW1, C4A2 and C4B2 were significantly higher in the IgA-deficient patients than in the controls. The most recurrent haplotype among patients was B14, DR1 (p less than 10(-4) preferentially associated with A33, A28 or A blank. The supratype B14, Bfs, C4A2, C4B2, DR1, DQW1 was present in a 14-fold higher frequency than in the controls, and strongly suggests the presence of a gene in the HLA region involved in the deficiency of IgA. The fact that this supratype was not always associated with IgA deficiency in the parents and that not all IgA-deficient subjects had this supratype is discussed. Severe IgA deficiency was found in four mothers (two of the four mother-child pairs shared the B14, DR1 haplotype); three other relatives (one father and two brothers) had partial IgA deficiency and did not have the B14, DR1, DQW1 haplotype.

Adolescent↗
PubMed1985

In-vitro analysis of defective IgA production in selective IgA deficiency in childhood.

IgA production from B cells can be driven by three signals (anti-Ig antibody, B cell growth factor and helper T cell factor). It was thought that this three signal system might be a way other than the mitogen-stimulated system, to access B cell differentiation into Ig-producing cells. Seven children with IgA deficiency were investigated by this method. In all cases B cell differentiation was defective when using this three signal system. In one case, IgA producing cells were generated in normal number from B cells when stimulated with pokeweed mitogen in the presence of normal T cells. Definite abnormality of regulatory T cells in the IgA production was observed in only one case. These observations suggested that the main cause of IgA deficiency in most cases is the defect of B cells to differentiate into IgA secreting cells.

B-Lymphocytes↗
PubMed1991

IgG subclass deficiency with or without IgA deficiency.

IgG subclass deficiency (IgGSD) is difficult to define since reference materials vary between laboratories and the clinically relevant cut off levels for the various subclasses are not well known. The diagnosis of IgGSD should be based on more than one determination since the levels vary, due to various factors such as infections, operations, etc. There is a relation between IgGSD and occurrence of frequent infections, but at the same time even total lack of a subclass can be seen in healthy individuals. Infections in the upper and lower respiratory tract predominate. Gm allotypes influence IgG subclass levels. Most IgGSD patients are homozygous in the Gm system. Immunocytes producing IgG3 in the nasal mucosa correlate with serum IgG3 levels, whereas rectal IgG1 producing cells relate to serum IgG1 levels. This may reflect differences in antigen and/or mitogen exposure at the two mucosal sites. IgG2SD is the predominant form of IgGSD among children, whereas after puberty IgG3SD is most common. At the same age period there is a switch in sex distribution from three boys/one girl to one male/three females. Various abnormalities in B and T lymphocyte numbers and function are often found. In a double blind crossover study of Ig prophylaxis over 2 years in 43 IgGSD adult patients a significant decrease in the number of days with infections was seen both in the whole group and among the IgG1 deficient. Days with bronchial constriction were also significantly decreased among the 22 patients who had asthma as well. Acute bronchitis became significantly less frequent in the IgG3 deficient patients during Ig prophylaxis. This was seen in those under the lower range of 0.41 g/liter of Oxelius' early normal material, although many recent reference materials suggest 0.14-0.15 g/liter of IgG3 as the cut off. In a group of 25 consecutive patients with IgGSD + IgA deficiency recurrent respiratory infections were the major problem and lung function impairment was found in 12, and bronchiectasies in 5. Still many individuals with this combined deficiency have been reported to be healthy, again illustrating our limited understanding of these conditions.

Disease↗
PubMed1982

Frequent occurrence of anti-rabbit IgM in IgA deficiency.

Analysis of IgA deficient sera revealed impaired hemolysis of sensitized sheep erythrocytes when tested by a hemolysis in gel (HIG) assay developed for detection of complement deficiencies. All sera were normal in a test for the alternative pathway. The impaired hemolysis was not related to complement aberrations but was caused by antibodies to rabbit IgM, demonstrated in 14 of 21 IgA deficient sera, by use of HIG technique and by agglutination. The presence of these antibodies was not related to age, sex or disease. One serum was further examined and the antibodies were shown to be of IgG class.

Adult↗
PubMed1975

Selective IgA deficiency in blood donors.

The frequency of selective deficiency of serum IgA was determined in a population of 64,588 new Finnish blood donors by Ouchterlony's double diffusion with 10 mug/ml as the limit of detection. The incidence was 1:396. Those found IgA-deficient were retested by hemagglutination inhibition and by radioimmunoassay. The calculated incidences of IgA levels below 0.5 and 0.015 mug/ml were 1:500 and 1:800, respectively. Statistically significant compensatory elevation of serum IgG was observed in the IgA-deficient donors. The IgM levels were not changed. Among 9,920 hospital patients, the incidence of IgA deficiency was 1:660. The age structure of the IgA-deficient patients was similar to that of the IgA-deficient healthy blood donors but lower than that of hospital patients in general. No difference was observed between the clinical history of IgA-deficienct blood donors and of the controls.

Adolescent↗
PubMed1990

Preparation of IgA-deficient platelets.

The IgA-deficient patient presents a difficult transfusion problem, particularly if he or she has previously been immunized and has formed IgA antibodies. Blood components from IgA-deficient donors may be used safely but are often not accessible to transfusion facilities. A satisfactory red cell component may be obtained by standard freezing and washing methods. There is no comparable platelet concentrate that uniformly affords protection against anti-IgA-mediated reactions. A method has been developed for the preparation of an IgA-deficient platelet concentrate by washing with citrate-buffered saline. Platelets prepared in this manner were transfused successfully to an IgA-deficient patient with a history of anaphylaxis related to transfusion. It was further demonstrated that the removal of IgA is enhanced if platelets are washed within 48 hours of collection. When platelets were stored for more than 48 hours, similar washing techniques resulted in significantly higher levels of IgA in the platelets washed with either buffered (p = 0.004) or unbuffered (p = 0.0002) saline. Platelets washed with unbuffered saline (pH 4.5-5.5) contained substantially more IgA than did platelets washed in a similar manner with citrate-buffered saline (pH 6.5-6.8) (p = 0.001).

Adolescent↗
PubMed1992

Selective IgA deficiency and autoimmunity.

Selective IgA deficiency is the most common primary immunodeficiency. Selective IgA deficiency, as most other immunodeficiencies, is frequently associated with autoimmune phenomena. In this article the authors critically review the literature concerning this association, and discuss the possible mechanisms leading to the increased frequency of autoimmune disorders in subjects with selective IgA deficiency.

Autoantibodies↗
PubMed1995

[IgG subclasses in patients with symptomatic IgA deficiency].

BACKGROUND: Patients with IgA deficiency may be asymptomatic or may present recurrent infections mainly respiratory. A possible explanation for this variety of symptoms may be the association with a deficiency of IgG subclasses. METHODS: Twenty-five patients with a symptomatic IgA deficiency with a mean age of 18 +/- 12 years were studied. The quantification of the IgG subclasses was performed by ELISA test with antisubclass specific monoclonal murine antibodies. Serum from 100 healthy adults was collected to determine normal values of the IgG subclasses. RESULTS: Twenty-five (48%) had a deficiency of associated IgG subclasses (6 IgG2 deficiency, 1 IgG3, 3 IgG4 and 2 combined deficiency). The patient with a deficiency of associated IgG subclasses had more recurrent respiratory infections (chi 2, p < 0.03) and pneumonias (chi 2, p < 0.04). This group also had a greater FEV1 alteration (Student-t test, p < 0.04). CONCLUSIONS: Patients with symptomatic IgA deficiency frequently present IgG subclass deficiency and are more likely to have recurrent respiratory infections and greater changes in pulmonary function.

Adolescent↗
PubMed2005

IgA deficiency: clinical correlates with IgG subclass and mannan-binding lectin deficiencies.

OBJECTIVE: To characterize an IgA deficient population in terms of the incidence of IgG subclass and mannose-binding lectin (MBL) deficiencies and the type and severity of infections and other associated disorders. BACKGROUND: Selective IgA deficiency is probably the commonest of the primary immunodeficiency disorders and although it may lead to an increased risk for respiratory and gastrointestinal infections and associated to various autoimmune diseases, it may also be asymptomatic. Several studies have suggested the need of a concomitant defect in order for manifestation of its symptoms. METHODS: A total of 27 patients fulfilling the diagnostic criteria of selective IgA deficiency were evaluated for IgG subclass and MBL deficiencies after a thorough medical history, physical examination and pertinent evaluation for concomitant medical conditions. RESULTS: The overall incidence of IgG subclass deficiency found in the IgA deficient group was 18.5%. MBL deficiency was found to be 3.7%. These frequencies may have been influenced by the age group evaluated and the size of the population studied. Severe infections were more common in patients with combined deficiencies, either IgA and any of the IgG subclasses or IgA and MBL deficiency. Atopy was widely represented in the patients studied. CONCLUSIONS: The observed relationship between combined deficiencies Ig A, IgG subclasses and MBL and the increased representation of severe infections needs to be corroborated in a larger sample of patients with an inclusion of pediatric patients.

Adult↗
PubMed2002

Increased frequency of intestinal Escherichia coli carrying genes for S fimbriae and haemolysin in IgA-deficient individuals.

Persons with selective IgA deficiency carry an increased risk of coeliac disease, inflammatory bowel disease and perhaps also gastrointestinal malignancies. Inflammatory bowel disease is associated with an increased carriage of adherent and haemolytic Escherichia coli in the intestinal microflora. This study was designed to investigate whether IgA-deficient individuals carry E. coli with virulence-associated properties in their gut flora. The last free-lying colony of E. coli isolates obtained from rectal flora of 25 IgA-deficient and 20 age-matched control individuals was assayed by multiplex PCR for genes for the following adhesins or virulence determinants: P, type 1 and S fimbriae, Dr haemagglutinin, haemolysin, aerobactin and the capsular types K1 and K5. E. coli strains from the intestinal microflora of IgA-deficient individuals more often had the gene for S fimbriae (36% of the strains compared with 0% in control subjects, P=0.003) as well as for haemolysin (40 vs 10% of the strains, P=0.040). IgA-deficient individuals had instead lower frequencies of E. coli carrying genes for type 1 fimbriae in their microflora (68 vs 90%, P=0.14). The results suggest that IgA-deficient individuals carry an increased frequency of E. coli with potentially inflammatogenic properties in their microflora, which may contribute to the development of gastrointestinal disorders such as inflammatory bowel diseases.

Adolescent↗
PubMed1996

[Chronic meningococcemia and IgA deficiency in an adolescent].

BACKGROUND: Chronic meningococcemia, defined as a meningococcal septicemia without meningeal symptoms with persistence of fever for at least one week prior to any antibiotics, is uncommon. Its pathophysiology remains unclear and a defect in host immunity has been suggested. CASE REPORT: A 15 year-old adolescent was examined because he suffered from fever for 6 days, headache, arthralgias. A disseminated erythema led to consider the diagnosis of vascularitis that was confirmed by skin biopsy. At day 9, blood culture yielded Neisseria meningitis group B that was confirmed by a second blood culture; the CSF was normal and sterile. The patient was given ceftriaxone plus penicillin for 14 days and completely cured. A detailed analysis of the complement system was negative but the patient was found to be deficient in IgA. CONCLUSION: This is the first reported case in which chronic meningococcemia is associated with complete IgA deficiency.

Adolescent↗
PubMed1978

Immunoglobulin abnormalities in relatives of IgA deficient epileptics.

Serum levels of IgA were found to be reduced in some patients with epilepsy. Further studies revealed that only epileptics with constitutional factors for seizures showed, if ever, IgA deficiency, particularly those treated with hydantoins (up to 25%). In order further to substantiate the association of immunoglobulin alterations with epilepsy nine families in whom the disease was clustered were investigated. An IgA deficiency was detected in 16 of the 19 epileptics (three without hydantoin medication), but in none of their 45 non-epileptic relatives. However, four of the relatives had a low IgM. Seven other families were tested in each of which only one IgA deficient epileptic was known. No other family members were found with a low IgA, but 24 of 58 such relatives had increased IgM serum concentrations. The association of IgA deficiency and epilepsy with IgM imbalances in relatives of IgA deficient epileptics gives additional support for the hypothesis that immune imbalances and certain forms of epilepsy might be linked.

Epilepsy↗
PubMed1993

Laboratory tests to exclude IgA deficiency in the investigation of suspected anti-IgA transfusion reactions.

Four methods were compared as to their suitability for excluding IgA deficiency in the investigation of suspected anti-IgA transfusion reactions. The methods were radial immunodiffusion, passive hemagglutination inhibition, sandwich enzyme-linked immunosorbent assay, and membrane enzyme immunoassay. Parallel testing was performed on sera from 40 patients or blood donors previously found to have anti-IgA and low or undetectable levels of IgA. All test methods identified the 40 sera as having abnormally low IgA levels. The membrane enzyme immunoassay required 10 minutes or less for testing, as compared to 3 hours for passive hemagglutination inhibition, 4 hours for sandwich enzyme-linked immunosorbent assay, and 48 hours for radial immunodiffusion. The membrane enzyme immunoassay offers the potential for a rapid, instrument-free screen of IgA levels and therefore may be useful in identifying those patients with suspected anti-IgA anaphylactic transfusion reactions who are not IgA deficient and do not require IgA-deficient blood components for additional transfusions.

Anaphylaxis↗