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Gamma 3 gene-disrupted mice selectively deficient in the dominant IgG subclass made to bacterial polysaccharides undergo normal isotype switching after immunization with polysaccharide-protein conjugate vaccines.

Bacterial polysaccharides (PS) are T-independent type 2 Ags that elicit restricted Ab responses of IgM and IgG3 in mice and IgM and predominantly IgG2 in humans. Immunodeficiency in the dominant IgG subclass made to PS is associated with chronic sinus and pulmonary infections with PS-encapsulated bacteria. To elucidate the biologic role of the dominant IgG subclass in the immune response to PS and to make an animal model of human IgG subclass deficiency, we generated mice with a targeted disruption of the exon encoding the CH1 domain of the gamma 3 heavy-chain constant region gene. Homozygotes had no detectable serum IgG3, and their splenocytes did not produce IgG3 after LPS stimulation. IgG3(-/-) mice immunized with PS from Pseudomonas aeruginosa LPS O-side chain or Streptococcus pneumoniae type 19F capsule did not produce any IgG3 anti-PS Abs, in contrast to wild-type mice in which IgG3 was the major IgG subclass. Immunizing both wild-type and IgG3(-/-) mice with 19F PS-protein conjugate elicited IgG1 Abs. We conclude that IgG3(-/-) mice have a selective deficiency in the dominant murine IgG subclass made to T-independent type 2 Ags and may be a useful animal model of IgG subclass deficiency. In addition, we show that the anti-PS Ab class switching to IgG1 that occurs when mice are immunized with a PS-protein conjugate vaccine does not require sequential Ig expression or an intact, upstream gamma 3 heavy-chain gene.

Animals↗

Familial hypercatabolic hypoproteinemia caused by deficiency of the neonatal Fc receptor, FcRn, due to a mutant beta2-microglobulin gene.

Two siblings, products of a consanguineous marriage, were markedly deficient in both albumin and IgG because of rapid degradation of these proteins, suggesting a lack of the neonatal Fc receptor, FcRn. FcRn is a heterodimeric receptor composed of a nonclassical MHC class I alpha-chain and beta(2)-microglobulin (beta(2)m) that binds two ligands, IgG and albumin, and extends the catabolic half-lives of both. Eight relatives of the siblings were moderately IgG-deficient. From sera archived for 35 years, we sequenced the two siblings' genes for the heterodimeric FcRn. We found that, although the alpha-chain gene sequences of the siblings were normal, the beta(2)m genes contained a single nucleotide transversion that would mutate a conserved alanine to proline at the midpoint of the signal sequence. Concentrations of soluble beta(2)m and HLA in the siblings' sera were <1% of normal. Transfection assays of beta(2)m-deficient cultured cells with beta(2)m cDNA indicated that the mutant beta(2)m supported <20% of normal expression of beta(2)m, MHC class I, and FcRn proteins. We concluded that a beta(2)m gene mutation underlies the hypercatabolism and reduced serum levels of albumin and IgG in the two siblings with familial hypercatabolic hypoproteinemia. This experiment of nature affirms our hypothesis that FcRn binds IgG and albumin, salvages both from a degradative fate, and maintains their physiologic concentrations.

Amino Acid Sequence↗

IgG3 deficiency: common in obstructive lung disease. Hereditary in families with immunodeficiency and autoimmune disease.

Among 313 patients with serum IgG deficiency, selective IgG3 deficiency was found in 59.5%, combined IgG3 deficiency together with IgG1 deficiency in 36% and combined IgG3-IgG2 deficiency in only 4.5%. Most of the patients with IgG3 deficiency suffered from upper respiratory tract infections, but many also from recurrent bronchitis, bronchopneumonias and asthma bronchiale. Those with combined IgG3-IgG1 deficiency often suffered from obstructive lung disease and chronic lower respiratory tract infections. Other diagnoses found in patients with IgG3 deficiency were diabetes mellitus, Henoch-Schönlein, recurrent herpes simplex infections and recurrent erysipelas. IgG3 deficiency was also found in relatives of patients with common variable immunodeficiency and IgA deficiency. In one family both parents and all four children showed IgG3 deficiency, some of them also C2 deficiency. In another family 2 siblings with diabetes mellitus showed IgG3 deficiency. In still another family the mother and her daughter both with asthma bronchiale showed IgG3 deficiency. Patients with IgG3 deficiency could respond to pneumococcal vaccine and seemed to respond to immunoglobulin substitution given every or every other week.

Autoimmune Diseases↗

Antiendomysium versus antigliadin antibodies in screening the general population for coeliac disease.

BACKGROUND: It has recently been shown that mass screening for coeliac disease, using either the serum antigliadin (AGA) or antiendomysium antibodies (EMA) as screening test, can detect large numbers of cases that had escaped clinical diagnosis. The influence of the diagnostic algorithm on the results of the coeliac screening has not yet been evaluated. Our aim was to compare the validity of the AGA and the EMA protocols in 2096 students living in northwest Sardinia, who took part in a serologic screening for coeliac disease. METHODS: The sample included 2096 of 2345 eligible students (89%) aged 11-15 years who underwent serum IgG AGA, IgA AGA, and IgA EMA determinations. Total serum IgA level was measured in sera showing isolated IgG AGA positivity. Subjects showing at least one of the following: a) EMA positivity, b) IgA AGA positivity, or c) IgG AGA positivity and IgA deficiency (<5 mg/dl) were asked to submit to a small-intestinal biopsy. RESULTS: The prevalence of coeliac disease was 19 (16 showing typical enteropathy, 1 potential case, and 2 known cases) of 2096 (0.91%; 95% confidence interval = 0.50-1.31). Seventeen small-intestinal biopsy specimens were needed to confirm 16 cases of manifest coeliac disease (positive predictive value (PPV) = 94%) by the EMA protocol, whereas the AGA protocol required 21 biopsy specimens for 12 cases of coeliac disease (PPV = 57%). None of six IgA-deficient, IgG AGA-positive cases detected by the AGA protocol also had coeliac disease. CONCLUSIONS: The EMA protocol is superior to the AGA protocol for mass screening of coeliac disease because of higher sensitivity, decreased need for intestinal biopsy, and possibility to detect potential cases of coeliac disease.

Adolescent↗

Desquamative interstitial pneumonia and antigen-antibody complexes in two infants with congenital rubella.

Desquamative interstitial pneumonia was observed in two infants with the late-onset congenital rubella syndrome. In both infants this unusual lung disease was associated with circulating immunoglobulin M complexes and interstitial pulmonary deposits of IgM by immunofluorescence. Both infants had IgG deficiency. The first child recovered with a reduction in IgM complex levels and synthesis of rubella-specific IgG. The second infant died during the acute phase of his illness at which time there were high serum concentrations of IgM complexes and slightly increased levels of IgG complexes. Delayed maturation of the immune response in congenital rubella may predispose to persistent antigenemia and pulmonary deposition of rubella antigen-containing IgM complexes resulting in an acute form of interstitial pneumonia.

Antigen-Antibody Complex↗

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↗

Retinoid repletion of vitamin A-deficient mice restores IgG responses.

Vitamin A-deficient (A-) mice produce poor IgG antibody responses due to a helper T cell dysfunction. We performed retinoid repletion studies to determine the minimum dietary retinyl acetate dose and the most active retinoid for supporting immune function. Dietary retinyl acetate repletion at 2 (R2 group) or 4 (R4 group) microgram/g diet restored serum retinol in A- mice to vitamin A-sufficient (A+) control levels within 24 h. However, in R4 mice, liver retinyl palmitate was restored about twofold faster than in R2 mice; liver retinyl palmitate reached A+ control levels by d 30 in R4 mice but not in R2 mice. We challenged the mice with antigen 24 h post repletion; the R4 mice gave an IgG1 response equal to that of A+ controls, but the R2 mice were comparable with the A- controls. We also compared four retinoids for IgG1 response restoration in vitro; 1 nmol/L retinoic acid fully repleted A- cell IgG1 responses and helper T cell frequencies to the unsupplemented A+ control levels. Retinoic acid was at least 10-fold more active than retinyl acetate or retinaldehyde, and 100-fold more active than retinol. Collectively, our results suggest that retinoic acid is probably the physiologically important metabolite for sustaining IgG immune responses in vivo. We discuss the possible relationship between liver retinyl palmitate levels and availability of retinoic acid to support immune function.

Animals↗

Acquired total immunoglobulin G deficiency in a patient with primary biliary cirrhosis.

A 38-year-old Japanese woman was hospitalized for susceptibility to respiratory tract infections. Clinical examinations revealed asymptomatic primary cholestasis, abnormally elevated immunoglobulin M (IgM) and antimitochondrial antibody, being consistent with asymptomatic primary biliary cirrhosis. Three years later her serum immunoglobulin G (IgG) decreased remarkably, whereas other immunoglobulins were unchanged. Immunological examinations on the peripheral blood lymphocytes demonstrated spontaneous over-synthesis of serum IgM and decreased synthesis of IgG due to abnormal function of both T and B cells. Our case suggests a new possible association between primary biliary cirrhosis and IgG deficiency.

Adult↗

IgG subclasses in symptomatic IgA deficiency.

We evaluated IgG subclass levels in 11 symptomatic patients (ages between 3 and 22; mean 7.5 years) with IgA deficiency, seven with selective IgA deficiency, and four with low IgA levels. All patients had experienced three or more episodes of sinopulmonary infections a year. Combined IgG2-IgG4 deficiency was detected in two patients, IgG2 deficiency in one patient and IgG4 deficiency in two patients. Elevated IgG1 and IgG3 levels were detected in most of the IgG subclass deficient and sufficient patients. It is known that gammaglobulin replacement therapy reduces the frequency of infections significantly in IgG subclass deficiency. Although immunization against IgA is a risk in IgA deficiency, these patients can be treated with gammaglobulins containing low IgA.

Adolescent↗

Progressive hypogammaglobulinaemia in a child born to a mother with Hodgkin's disease.

A young woman developed Hodgkin's disease (nodular sclerosis) in pregnancy and gave birth to a boy who developed common variable immunodeficiency. Initially there was normal IgG with low IgA and IgM, and antibody deficiency. IgG levels fell progressively over 4 years. Cellular immunity was normal. We suggest that this is a further family with immune deficiency presenting with common variable immunodeficiency and lymphoid malignancy.

Adult↗

Juvenile onset pernicious anemia, partial intestinal villous atrophy, ulcerative colitis, and squamous metaplasia of the stomach.

We report a case of a 13-yr-old white boy with juvenile onset pernicious anemia in association with IgG deficiency. He had marked gastric atrophy, intestinal metaplasia of the stomach, and an intractable antral ulcer that required surgery. In addition, his gastric mucosa showed evidence of a progressive squamous metaplasia. Diffuse squamous metaplasia of the stomach, a very rare gastric lesion, has not previously been described either in association with pernicious anemia, atrophic gastritis, or hypogammaglobulinemia. This patient also has ulcerative colitis involving the entire colon and partial villous atrophy noted on small intestinal biopsy.

Anemia, Pernicious↗

[Mechanisms of action of intravenous immunoglobulins].

Intravenous immunoglobulins (IVIG) are now used to treat various diseases, including autoimmune diseases, systemic inflammatory diseases, allografts and for replacement therapy in the case of IgG deficiency. Only in some of the indications has the efficacy of this treatment been confirmed in large-scale studies. Also, in many cases the modes of action remain unclear. Principally, the following therapeutic strategies can be differentiated: Replacement treatment, blocking of the effector molecules, influencing of the cellular and humoral limbs of the immune defence system and interaction with cytokines. In certain CNS diseases, displacement of pathological immunoglobulins may be involved. It would be desirable to acquire more detailed knowledge about modes of action with the aim of using IVIG with greater specificity in the future.

Acute-Phase Reaction↗

Periodontal disease and dental caries in relation to primary IgG subclass and other humoral immunodeficiencies.

22 females and 3 males with primary hypogammaglobulinaemia (n = 6) or IgG subclass deficiencies with (n = 3) or without (n = 16) concomitant IgA deficiency were examined for periodontal disease and dental caries. Only 1 patient showed more tooth loss than that found in the normal Swedish population. 1 patient demonstrated advanced periodontal disease. No patient exhibited more severe dental caries than that of comparable normal Swedes. Microbiological samples from periodontal pockets and saliva showed recovery of potential periodontopathic and cariogenic bacteria within normal ranges. This study could not support the notion that immunodeficient subjects exhibit an increased risk of developing periodontal disease or dental caries.

Adult↗

Quantitative immunoglobulins and IgG subclasses in patients with corticosteroid-dependent reversible airway obstruction.

Six of 12 patients with corticosteroid-dependent bronchial asthma and recurrent sinopulmonary infections were found to have significant abnormalities in quantitative immunoglobulins and/or IgG subclasses. Five patients had a combined quantitative immunoglobulin and IgG subclass deficiency and one patient had an isolated IgG deficiency. Combined IgG subclass deficiencies were observed in two patients, both with deficiencies of IgG2 and IgG3. The most common IgG subclass deficiencies were of IgG2 and IgG3, which were found in three patients each.

Adrenal Cortex Hormones↗

IgG subclass levels in patients with B cell chronic lymphocytic leukaemia.

Patients with B-cell chronic lymphocytic leukaemia (BCLL) have low levels of serum IgG. In order to determine if this is a pan IgG deficiency or a selective suppression of one or more IgG subclasses, levels of IgG 1, 2, 3 and 4 in nine BCLL patients were determined and compared to those of nine age and sex matched controls. No significant differences were found in the levels of IgG1 and IgG2, but the patients were found to have significantly lower levels of IgG3 (p < 0.05) and IgG4 (p < 0.05). Selective deficiencies of these isotypes may explain the particular pattern of infection seen in BCLL patients.

Aged↗

[Antibody deficiency in obstructive lung diseases].

Severe chronic chest disease is the most serious complication in patients with antibody deficiency syndromes. IgG-subclass-deficiencies were a frequent finding in patients with obstructive lung disease and in patients with sinopulmonary infections. In the patient population referred to us for immunological investigation recurrent infection of the upper and lower respiratory tract was the most common reason to suspect undue susceptibility to infection. In 1034 pediatric patients analyzed 299 were found to be deficient in one of several IgG subclasses. In a group of 30 children, all of whom had severe lung disease and normal concentrations of serum IgG, IgA and IgM, airway-obstruction has been diagnosed in 19. 20 of the 30 patients had IgG-subclass-deficiency. The large percentage of IgG-subclass-deficiencies in this group of patients indicates that immunological disregulation is likely to contribute to the pathogenesis of chronic lung disease.

Adolescent↗

Immunoglobulin levels in bronchoalveolar lavage fluid of children with chronic chest disease.

The concentration and distribution of immunoglobulin isotypes (IgG, IgM, sIgA) and IgG-subclass levels (IgG-1-4) were measured in bronchoalveolar lavage fluid (BALF) in 47 children with chronic chest disease (age range 1.0-9.9 years) and 18 healthy controls (age range 1.0-6.25 years). Of these patients, 19 had nonallergic asthma (Group A), 19 suffered from recurrent pneumonia or chronic bronchitis (Group B), and 9 patients had IgG-2 deficiency (Group C). In all individuals, IgG was the predominant immunoglobulin in the lower respiratory tract, followed by IgA and IgM. In patients of Group A and B, IgG, IgM and IgA levels in BALF were significantly elevated when compared to controls. Assessment of IgG-subclass concentrations in BALF revealed that IgG-1 levels were increased in Group A and B when compared to controls (P < 0.05). Since this difference could not be explained by difference in age, it is possibly due to the inflammatory process at the mucosal level. IgG-2 levels were elevated in all patients except those with IgG-2 deficiency. IgG-2 concentration in the IgG-2 deficent group was lower compared to controls (P < 0.005) and patients in Group A (P < 0.0005) and B (P < 0.005). IgG-3 levels were elevated in asthmatics in group A compared to healthy controls (P < 0.005). IgG-4 concentrations were the same in all study groups. Since IgG-subclasses in percentage of total IgG were similar in BALF and serum, our results do not indicate a local production of any of the IgG-subclasses in the respiratory tract.

Asthma↗