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PubMed1991

Autoimmune chronic active hepatitis in a family.

A positive family history of autoimmune disease is common among patients with autoimmune chronic active hepatitis, but usually autoimmunity is directed at organs other than the liver. We document for the first time the multiple occurrence of autoimmune chronic active hepatitis in a family. Out of a sibship of seven, three sisters developed this, one sister developed coeliac and autoimmune thyroid disease, one sister showed serological signs of autoimmunity, while the two brothers were well with no signs of autoimmunity. HLA typing showed that in association with the female sex DR3 seems to be more important than B8 in conferring susceptibility to autoimmune chronic active hepatitis, at least in this family.

Adolescent↗

PubMed2001

Autoimmune pancreatitis: possibilities of CT characterization.

BACKGROUND: Pancreatitis is defined as an inflammatory disease of the pancreas, leading to morphological and pathological changes. Recently, an autoimmune pathogenesis of this disease has been proposed. This type of pancreatitis should be differentiated from other pancreatic diseases, since appropriate therapy is effective and morphological changes and pancreatic function can recover to normal levels. AIM OF THE STUDY: To assess the possibility of distinguishing autoimmune pancreatitis from other pancreatic diseases with an analogous clinical presentation on the basis of CT findings alone. METHODS: The CT images of 7 patients with proven autoimmune pancreatitis, along with those of 20 patients with other pancreatic diseases, but with an analogous clinical presentation, were retrospectively evaluated in a blinded fashion by 2 radiologists. In particular, the radiologists had to search for the typical signs of autoimmune pancreatitis. Discordant cases were further analyzed in the presence of a 3rd radiologist. The final diagnosis was acquired by means of a majority or overall consensus. The sensitivity, specificity, positive and negative predictive values of CT were calculated against each of the diseases (autoimmune pancreatitis; other pancreatic diseases), in order to evaluate the diagnostic value of the scan. RESULTS: After the consensus evaluation, the correct diagnosis was reached in 25/27 (92.5%) cases, with only 2/27 wrong diagnoses (autoimmune pancreatitis diagnosed as another pancreatic disease and vice versa). The sensitivity and specificity of CT against autoimmune pancreatitis were 86 and 95%, respectively. The positive and negative predictive values were 89 and 93%, respectively. CONCLUSION: Patients with autoimmune pancreatitis demonstrate imaging findings that enable the correct diagnosis by dynamic CT, even in the presence of nonspecific clinical symptoms. The precise classification of the disease is extremely important, since simple steroid therapy then represents the correct treatment, and leads to complete recovery.

Adult↗

PubMed2003

Asthma as a paradigm for autoimmune disease.

Allergy and autoimmunity result from dysregulation of the immune system. Until recently, it was generally accepted that the mechanisms that govern these disease processes are quite disparate; however, new discoveries suggest possible common pathogenetic effector pathways. This review illustrates the concomitant presentation of these conditions and the potential relationship or common mechanisms in some cases, by looking at the key elements that regulate the immune response in both asthma and autoimmune conditions: mast cells, antibodies, T cells, cytokines, and genetic determinants. The parallel appearance of asthma and autoimmune conditions in the same patients may reveal that such aberrations of the immune system have a common pathophysiologic mechanism. Mast cells, which play a key role in asthma, and the wealth of inflammatory mediators they express, make it likely that they have profound effects on many autoimmune processes. Activation of protein kinases by inflammatory cytokines and environmental stresses may contribute to both allergic and autoimmune diseases. The presence of autoantibodies in some allergic diseases suggests an autoimmune basis for these conditions. Because of the central role T cells play in immune reactivity, the T cell receptor loci have long been considered important candidates for a common disease susceptibility within the immune system such as asthma, atopy, and autoimmunity. Immunomodulation is the key to successful treatment of asthma and autoimmune conditions.

Asthma↗

PubMed1999

Familial clustering of autoimmune disorders and evaluation of medical risk factors in autism.

Autism is an age-dependent neurologic disorder that is often associated with autoimmune disorders in the patients' relatives. To evaluate the frequency of autoimmune disorders, as well as various prenatal and postnatal events in autism, we surveyed the families of 61 autistic patients and 46 healthy controls using questionnaires. The mean number of autoimmune disorders was greater in families with autism; 46% had two or more members with autoimmune disorders. As the number of family members with autoimmune disorders increased from one to three, the risk of autism was greater, with an odds ratio that increased from 1.9 to 5.5, respectively. In mothers and first-degree relatives of autistic children, there were more autoimmune disorders (16% and 21%) as compared to controls (2% and 4%), with odds ratios of 8.8 and 6.0, respectively. The most common autoimmune disorders in both groups were type 1 diabetes, adult rheumatoid arthritis, hypothyroidism, and systemic lupus erythematosus. Forty-six percent of the autism group reported having relatives with rheumatoid diseases, as compared to 26% of the controls. Prenatal maternal urinary tract, upper respiratory, and vaginal infections; asphyxia; prematurity, and seizures were more common in the autistic group, although the differences were not significant. Thirty-nine percent of the controls, but only 11% of the autistic, group, reported allergies. An increased number of autoimmune disorders suggests that in some families with autism, immune dysfunction could interact with various environmental factors to play a role in autism pathogenesis.

Adolescent↗

PubMed2000

Cytokine gene polymorphisms in autoimmune thyroid disease.

Susceptibility to the autoimmune thyroid diseases, Graves' disease (GD) and autoimmune hypothyroidism (AIH), depends on a complex interaction between environmental and genetic factors. The human leukocyte antigen and cytotoxic T lymphocyte-associated-4 regions appear to influence susceptibility to disease, but the effect is not major, and the other genes remain unknown. Cytokines are crucial in the regulation of immune and inflammatory responses and therefore are potential candidate genes for autoimmune thyroid disease. In a case-control study, using a unified method of genotyping, we have examined 15 polymorphisms in 9 cytokine genes in 215 patients with autoimmune thyroid disease (GD, 138; AIH, 77) and 101 normal controls. Polymorphisms in the genes for interleukin-1alpha (IL-1alpha), IL-1beta, IL-1 receptor antagonist, IL-1 receptor 1, IL-4, IL-4 receptor, IL-6, IL-10, and transforming growth factor-beta were investigated. Genotyping was performed using the PCR and sequence-specific primers. Analysis showed a reduced frequency of the variant t allele in the IL-4 promoter polymorphism (position 590) in patients with GD and in the entire patient group (GD and AIH) compared with the control group [corrected P (Pc) = 0.00004 and Pc < 0.00001 for GD and all patients, respectively]. This was reflected in a reduction in the heterozygote genotype in the patient groups compared to the controls [c/t heterozygotes GD, 12%; Pc = 0.06, odds ratio, 0.4 (95% confidence interval, 0.2-0.7); all patients, 11%; Pc = 0.008; odds ratio, 0.4 (95% confidence interval, 0.2-0.7); control subjects, 23%]. There were no significant differences between the study groups for the other polymorphisms examined, and subgroup analysis revealed no association with clinical parameters of disease. These results suggest that an IL-4 variant or a closely linked gene has a modest protective effect against the development of autoimmune thyroid disease, particularly GD. This variation in the IL-4 gene may provide further clues to the pathogenesis of autoimmune thyroid disease and other organ-specific autoimmune diseases. Furthermore, these results suggest that subtle variation in immunoregulatory genes may be associated with autoimmune disease states.

Cohort Studies↗

PubMed2001

Xenobiotic acceleration of idiopathic systemic autoimmunity in lupus-prone bxsb mice.

The diverse genetic backgrounds of lupus-prone murine models, which produce both quantitative and qualitative differences in disease expression, may be a valuable resource for studying the influence of environmental exposure on autoimmune disease in sensitive populations. We tested this premise by exposing autoimmune-prone BXSB and the nonautoimmune C57BL/6 mice to the heavy metal mercury. Although both strains express a nonsusceptible H-2 haplotype, exposure to mercury accelerated systemic autoimmunity in both male and female BXSB mice, whereas the C57BL/6 mice were resistant. The subclasses of antichromatin antibodies elicited in BXSB mice by mercury exposure were more consistent with the predominant Th1-type response of idiopathic disease than with the Th2-type response found in mercury-induced autoimmunity (HgIA). The appearance and magnitude of both humoral and cellular features of systemic autoimmunity correlated with the mercury dose. Furthermore, environmentally relevant tissue levels of mercury were associated with exacerbated systemic autoimmunity. These studies demonstrate that xenobiotic exposure can accelerate spontaneous systemic autoimmunity, and they support the possibility that low-level xenobiotic exposure enhances susceptibility to systemic autoimmunity in genetically susceptible individuals.

Animals↗

PubMed2006

Incidence of iodine deficiency in patients presenting with goitre--discrepancy between clinical and ultrasonographic evaluation of the thyroid: comparison of patients with and without autoimmune thyroiditis--clinical, hormonal and urinary iodine excretion studies.

In endemic areas iodine deficiency, and in iodine sufficient regions autoimmune thyroiditis, is the first aetiological factor for goitre. The aims of this study were to determine the incidence of iodine deficiency and autoimmune thyroiditis in patients presenting with goitre, to compare clinical and ultrasonographic assessment of thyroid size and to investigate the relationship between iodine and autoimmune thyroiditis. Patients diagnosed with goitre clinically (n = 204) were evaluated by their anthropometric measurements, ultrasonographic examination of the thyroid gland, thyroid function and TRH stimulation tests, thyroid autoantibodies and morning urinary iodine measurements. Thyroid volumes were evaluated according to three different reference criteria. Incidences of iodine deficiency and autoimmune thyroiditis were 54% and 17%. The incidences of iodine deficiency and excess were not significantly different in the autoimmune group (n = 35) compared to the non-autoimmune group (n = 169). In the autoimmune group, urinary iodine concentration correlated positively with serum thyroid hormones (FT3 r = 0.42, TT3 r = 0.38, TT4 r = 0.34) and negatively with serum TSH levels (r = 0.45). There were discrepancies between clinical and ultrasonographic evaluation of goitre, and between different reference criteria. This study revealed that iodine deficiency is still the first aetiological factor for goitre in our region and failed to show a relationship between iodine intake and autoimmune thyroid disease.

Adolescent↗

PubMed2006

Thyroid autoimmunity in patients with malignant and benign breast diseases before surgery.

BACKGROUND: Previous studies have demonstrated a high prevalence of thyroperoxidase antibodies (TPOAb) and autoimmune hypothyroidism in breast cancer (BC). These studies have been performed in BC patients generally 20-30 days after mastectomy. It is known that stress may have an influence on the immune system and a relation between stressful events and the onset or worsening of autoimmune thyroid disorders has been reported by several authors. The aim of the study was to evaluate the prevalence of autoimmune thyroid disease in patients with nodular breast disease selected for surgery before any treatment. Our hypothesis was that the high prevalence of thyroid autoimmune disorders in BC is independent of stressful events represented by surgery and/or anaesthetic procedures. METHODS: Our series included 61 consecutive women aged 52.8 +/- 10.2 yrs (mean age +/- s.d.) with nodular breast disease selected for breast surgery: 36 out of 61 of them (59%) had BC and 25 out of 61 had benign breast disease (BBD). Controls included 100 healthy age-matched women. All patients and control subjects were submitted to clinical, ultrasound thyroid evaluation and serum-free thyroxine (FT4), serum-free tri-iodothyronine (FT3), TSH, TPOAb and thyroglobulin antibodies (TgAb) determination. RESULTS: Mean FT3, FT4 and TSH concentration showed no differences between BC patients, BBD patients and controls. The prevalence of TPOAb in BC patients (12/36: 33.33%) was significantly higher than in BBD patients (5/25: 20%) (P < 0.01) and in controls (8/100: 8%) (P < 0.01). Similarly, the prevalence of TgAb in BC patients was 12 out of 36 (33.33%) significantly higher than that detected in BBD patients (4/25: 16%) (P < 0.01) and in controls (12/100: 12%) (P < 0.01). Of the 36 BC patients, 20 showed a diffuse hypoechogenicity of the thyroid gland to ultrasound evaluation, significantly higher than in BBD (7/25: 28%) (P = 0.03). Of the 20 BC patients who showed a hypoechogenic pattern of thyroid gland, 10 (50%) were associated with antithyroid antibodies positivity (TAb). This finding was present in two of seven BBD (28.57%) (P < 0.0001). Only two controls showed focal hypoechogenicity of the thyroid gland. Generally, 24 out of 36 (66.7%) of BC and 9 out of 25 (36%) of BBD (P = 0.02) had signs of thyroid autoimmunity consistent with the hypoechogenic pattern of thyroid gland associated or not with TAb; 2 out of 36 (5.55%) of BC and 1 out of 25 (4%) of BBD patients had autoimmune hypothyroidism and no hypothyroidism was found in controls. CONCLUSIONS: The results of this study confirm the strong relation between thyroid autoimmunity and BC. This finding is independent of stressful events represented by surgery or anaesthetic procedures. The present data call attention to the usefulness of screening for autoimmune thyroid disorders in patients with nodular breast disease selected for surgery.

Adult↗

PubMed2003

Gene expression profiles in human autoimmune disease.

To acquire a functional view of human autoimmunity, we compared differences in gene expression (>4000 genes) in the peripheral blood mononuclear cells of normal individuals following immunization to those in individuals with four different autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus, insulin dependent diabetes mellitus, and multiple sclerosis). Each individual from all disease groups displayed a similar pattern of gene expression that was highly distinct from the gene expression pattern of the immunized group. These findings indicate that the expression pattern accompanying autoimmunity is not simply a recapitulation of the immune response to non-self. Of note, expression levels of genes that encode key proteins in several distinct apoptosis pathways were markedly reduced in all autoimmune disease groups. Taken together, these data indicate that the pattern of gene expression describes a molecular portrait of autoimmunity that is constant among individuals with autoimmune disease but is independent of the specific autoimmune disease and the clinical parameters associated with any individual autoimmune disease.

Animals↗

PubMed2005

[Autoimmune thyroid disease and associated diseases].

Autoimmune thyroid disease (ATD) is a multifactorial, genetic disease. It is the sequelae of the impaired immunoregulation, tolerance and poor recognition of one's own proteins, oligopolysaccharides and polypeptides, due to development of somatic lymphocyte mutations. It is manifested by different clinical and morphological entities, inter-related by etiopathogenetic association, i.e., all of them are caused by disorder of immune system regulation. Chronic autoimmune thyroidism (Thyreoiditis lymphocytaria Hashimoto, HT), as well as immunogenic hyperthyroidism (Morbus Graves Basedow, MGB) are frequently associated with autoimmune diseases of other organs, such as: chronic insufficiency of salivary glands (Sy Sjögren), autoimmune hemolytic anemia, megalocytic pernicious anemia, thrombocytopenia, Rheumatoid arthritis, Diabetes mellitus (more often type 2, but also type 1), Morbus Addison, Coeliakia, and other autoimmune diseases such as systemic diseases of connecting tissue (Lupus erythematosus-SLE, Sclerodermia, Vasculitis superficialis). The incidence of autoimmune diseases has been at increase in all age groups of our population. The prevalence of organ-specific and organ-nonspecific antibodies increases with the age. Antigenicity of thyroid epithelial cell may be triggered by different chemical and biological agents (repeated viral infections), repeated stress, and in individuals with genetic propensity. Unrecognized ATD progressively leads to hypothyroidism with hyperlipidemia, blood vessel changes, osteoporosis, deformities, invalidity which substantially reduces the quality of life of patient and requires medical attention and expensive treatment on what account it is medically and socio-economically significant. Multiple diagnostic procedures contribute to faster recognition of this condition. The goal of the primary health care physician (given that preclinical phase of ATD and other associated diseases have different duration) and other specialists is to recognize ATD and, by early diagnosis and multidisciplinary treatment, to take secondary preventive measures of manifestation of above-mentioned associated autoimmune diseases, and in that way, to avoid the development of comorbidity and complications. It is particularly supported by medical doctrine based on evidence of application of corticosteroids, cytostatics, thyro-suppressive and substitution therapy, antilipemics, bisphosphonates and other drugs, significant for autoimmune diseases.

Autoimmune Diseases↗

PubMed1998

Autoimmune diabetes: the role of T cells, MHC molecules and autoantigens.

Type 1 diabetes (IDDM) is a T cell mediated autoimmune disease which in part is determined genetically by its association with major histocompatibility complex (MHC) class II alleles. The major role of MHC molecules is the regulation of immune responses through the presentation of peptide epitopes of processed protein antigens to the immune system. Recently it has been demonstrated that MHC molecules associated with autoimmune diseases preferentially present peptides of other endogenous MHC proteins, that often mimic autoantigen-derived peptides. Hence, these MHC-derived peptides might represent potential targets for autoreactive T cells. It has consistently been shown that humoral autoimmunity to insulin predominantly occurs in early childhood. The cellular immune response to insulin is relatively low in the peripheral blood of patients with IDDM. Studies in NOD mice however have shown, that lymphocytes isolated from pancreatic islet infiltrates display a high reactivity to insulin and in particular to an insulin peptide B 9-23. Furthermore we have evidence that cellular autoimmunity to insulin is higher in young pre-diabetic individuals, whereas cellular reactivity to other autoantigens is equally distributed in younger and older subjects. This implicates that insulin, in human childhood IDDM and animal autoimmune diabetes, acts as an important early antigen which may target the autoimmune response to pancreatic beta cells. Moreover, we observed that in the vast majority of newly diagnosed diabetic patients or individuals at risk for IDDM, T cell reactivity to various autoantigens occurs simultaneously. In contrast, cellular reactivity to a single autoantigen is found with equal frequency in (pre)-type 1 diabetic individuals as well as in control subjects. Therefore the autoimmune response in the inductive phase of IDDM may be targeted to pancreatic islets by the cellular and humoral reactivity to one beta-cell specific autoantigen, but spreading to a set of different antigens may be a prerequisite for progression to destructive insulitis and clinical disease. Due to mimic epitopes shared by autoantigen(s), autologous MHC molecules and environmental antigens autoimmunity may spread, intramolecularly and intermolecularly and amplify upon repeated reexposure to mimic epitopes of environmental triggers.

Alleles↗

PubMed2004

Molecular and cellular mechanisms associated with autoimmune diseases.

Evidence points to increases in the incidence and prevalence of several autoimmune diseases in the United States. As a result, the cost to public health from clinical management of autoimmune conditions is on the rise. The initiation and progression of autoimmune disturbances involves both genetic and environmental factors. Deficiencies in important proteins that normally participate in maintaining checks and balances within the internal milieu may render an individual prone to developing autoantibodies. Structural abnormalities or decline in normal levels of the pentraxins (serum amylase-P protein, the acute phase proteins, complement, and C-reactive proteins) have been shown to induce autoimmunity. Irregular transmission of information arising from multiple signal transduction pathways typically associated with the serine/threonine cascade routes of mitogen activating phosphorylation kinases, has also been found to induce autoimmunity. The kind of ligand/receptor interactions drives physical recruitment of different signals within the lymphocyte; these links define the quality and quantity of subsequent immune responses. CD95 or the Fas/Apo-1 and its ligand CD95L participate in regulating lymphocyte populations and therefore influence various aspects of immune responses. Mutational abnormalities resulting from synthesis of proteins by the CD95 and/or its ligand CD95L may result in alterations in the apoptotic pathways. Apoptosis may be completely inhibited, activated or partially stimulated. Modulation of apoptosis may lead to accumulation of self-antigens. Subsequently the immune system may be stimulated to react against self-molecules through lymphatic hyperplasia. This process may end up in proliferative disorders and enhanced susceptibility to autoimmune syndromes. This paper deals with mechanisms of autoimmunopathogenesis at the cellular and molecular levels. Emphasis is laid on the role of T and B cell receptor/ligand interactions, functions and malfunctions due to structural and quantitative alterations in T- B- cell clusterof antigen determinants. Genetically susceptible patients who develop spontaneous autoimmune diseases are examined and the etiological factors implicated in the initiation and subsequent dissemination of autoimmune diseases is discussed.

Animals↗

PubMed2003

Chemokines determine local lymphoneogenesis and a reduction of circulating CXCR4+ T and CCR7 B and T lymphocytes in thyroid autoimmune diseases.

Chemokines and their corresponding receptors are crucial for the recruitment of lymphocytes into the lymphoid organs and for its organization acting in a multistep process. Tissues affected by autoimmune disease often contain ectopic lymphoid follicles which, in the case of autoimmune thyroid disorders, are highly active and specific for thyroid Ags although its pathogenic role remains unclear. To understand the genesis of these lymphoid follicles, the expression of relevant cytokines and chemokines was assessed by real time PCR, immunohistochemistry and by in vitro assays in autoimmune and nonautoimmune thyroid glands. Lymphotoxin alpha, lymphotoxin beta, C-C chemokine ligand (CCL) 21, CXC chemokine ligand (CXCL) 12, CXCL13, and CCL22 were increased in thyroids from autoimmune patients, whereas CXCL12, CXCL13, and CCL22 levels were significantly higher in autoimmune glands with ectopic secondary lymphoid follicles than in those without follicles. Interestingly, thyroid epithelium produced CXCL12 in response to proinflammatory cytokines providing a possible clue for the understanding of how tissue stress may lead to ectopic follicle formation. The finding of a correlation between chemokines and thyroid autoantibodies further suggests that intrathyroidal germinal centers play a significant role in the autoimmune response. Unexpectedly, the percentage of circulating CXCR4(+) T cells and CCR7(+) B and T cells (but not of CXCR5) was significantly reduced in PBMCs of patients with autoimmune thyroid disease when they were compared with their intrathyroidal lymphocytes. This systemic effect of active intrathyroidal lymphoid tissue emerges as a possible new marker of thyroid autoimmune disease activity.

Autoantibodies↗

PubMed2003

IL-1 receptor-associated kinase 1 regulates susceptibility to organ-specific autoimmunity.

Infections often precede the development of autoimmunity. Correlation between infection with a specific pathogen and a particular autoimmune disease ranges from moderately strong to quite weak. This lack of correspondence suggests that autoimmunity may result from microbial activation of a generic, as opposed to pathogen-specific host-defense response. The Toll-like receptors, essential to host recognition of microbial invasion, signal through a common, highly conserved pathway, activate innate immunity, and control adaptive immune responses. To determine the influence of Toll/IL-1 signaling on the development of autoimmunity, the responses of wild-type (WT) mice and IL-1R-associated kinase 1 (IRAK1)-deficient mice to induction of experimental autoimmune encephalomyelitis were compared. C57BL/6 and B6.IRAK1-deficient mice were immunized with MOG 35-55/CFA or MOG 35-55/CpG DNA/IFA. WT animals developed severe disease, whereas IRAK1-deficient mice were resistant to experimental autoimmune encephalomyelitis, exhibiting little or no CNS inflammation. IRAK1-deficient T cells also displayed impaired Th1 development, particularly during disease induction, despite normal TCR signaling. These results suggest that IRAK1 and the Toll/IL-1 pathway play an essential role in T cell priming, and demonstrate one means through which innate immunity can control subsequent development of autoimmunity. These findings may also help explain the association between antecedent infection and the development or exacerbations of some autoimmune diseases.

Adjuvants, Immunologic↗

PubMed2000

Deficiency of the Fas apoptosis pathway without Fas gene mutations is a familial trait predisposing to development of autoimmune diseases and cancer.

Fas/Apo-1 (CD95) triggers programmed cell death (PCD) and is involved in immune response control and cell-mediated cytotoxicity. In the autoimmune/lymphoproliferative syndrome (ALPS), inherited loss-of-function mutations of the Fas gene cause nonmalignant lymphoproliferation and autoimmunity. We have recently identified an ALPS-like clinical pattern (named autoimmune lymphoproliferative disease [ALD]) in patients with decreased Fas function, but no Fas gene mutation. They also displayed decreased PCD response to ceramide, triggering a death pathway partially overlapping that used by Fas, which suggests that ALD is caused by downstream alterations of the Fas signaling pathway. Decreased Fas function is also involved in tumor development, because somatic mutations hitting the Fas system may protect neoplastic cells from immune surveillance. This work assessed the inherited component of the ALD defect by evaluating Fas- and ceramide-induced T-cell death in both parents and 4 close relatives of 10 unrelated patients with ALD. Most of them (22 of 24) displayed defective Fas- or ceramide-induced (or both) cell death. Moreover, analysis of the family histories showed that frequencies of autoimmunity and cancer were significantly increased in the paternal and maternal line, respectively. Defective Fas- or ceramide-induced T-cell death was also detected in 9 of 17 autoimmune patients from 7 families displaying more than a single case of autoimmunity within first- or second-degree relatives (multiple autoimmune syndrome [MAS] patients). Autoimmune diseases displayed by ALD and MAS families included several organ-specific and systemic forms. These data suggest that ALD is due to accumulation of several defects in the same subject and that these defects predispose to development of cancer or autoimmune diseases other than ALPS/ALD.

Adolescent↗

PubMed1999

[Study of autoimmunity in progeny of pregnant women with systemic lupus erythematosus].

OBJECTIVE: To study the effect of systemic lupus erythematosus (SLE) on physical, mental development and plasma antibody level of SLE in their progenies. METHODS: Routine physical examinations of 49 children from 48 SLE mothers were conducted. Compared immuno-fluorescence anti-nuclear antibody (IFANA), anticardiolipin (ACL), extractable nuclear antigen (ENA) and anti-ds-DNA plasma levels of SLE mothers and their progenies with that levels during pregnancy and in umbilical blood. RESULTS: The physical development (height and weight) in 47 out of 49 children were within normal range while the remaining 2 were in the lower limit. The autoimmune antibodies were all negative in the umbilical blood with autoimmune negative mothers, while the anti-ribonucleoprotein (anti-RNP), anti-Smith surface antigen (anti-SSA), anti-specific soluble ribonucleic acid (anti-SSB) and ACL could be transferred to fetus through placenta. During follow up study, compared the autoimmune positive rates in progenies with that of mothers, the positive rates of IFANA and anti-ds-DNA decreased significantly (P < 0.01), while no changes in ACL. Compared the autoimmune positive rates in progenies with that of their own umbilical levels, the positive rates of IFANA, anti-RNP, anti-SSA decreased significantly (P < 0.01), while no difference existed in ACL. Boys showed faster disappearance of autoimmune positive rates than that of girls. CONCLUSIONS: SLE did not show significant effects on the physical development of their progenies. Most autoimmune antibodies existed in umbilical blood were transferred through placenta during pregnancy and would disappear within 9 years after birth. Autoimmune antibodies decreased quicker in boys, and it indicated that girls should be follow-up more carefully. Autoimmune antibodies in the umbilical blood is an easy method for the screening of SLE progeny.

Adult↗

PubMed2001

[Clinical and pathological study of autoimmune hepatopathy].

OBJECTIVE: To study the clinical and histologic characteristics of autoimmune hepatopathy, and to explore the diagnostic criteria and therapy. METHODS: Sixty-two patients with autoimmune hepatopathy were studied between 1996 and 1998 to compare primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) in the clinical symptoms, signs, laboratory findings and histologic features. RESULTS: PBC was 59.68% and AIH 40.32% in the autoimmune hepatopathy. The morbidity in middle and old aged female patients with autoimmune hepatopathy was 83.8%. The ratio of male and female was 1:10. Whereas, the ratio of male and female was 1:5 in AIH patients and average age was 35 years old. Seropositive autoimmune antibodies were detected in 53 serum sample (PBC 85.29%; AIH 78.95%). Positive rates of the mitochondrial antibody and subtype-M2 were 100% for PBC. IgM increasing was 80% in PBC (P<0.05) and 82.35% in AIH (P<0.025). Histologic features in PBC group consisted of cholangitis, destruction or proliferation of the bile duct and inflammation within the portal tract; while in AIH group inflammation, necrosis and fibrosis of periportal region, the aggregation of lymphocytes, mononuclear cells and plasma cells in the portal tract or periportal area were found. CONCLUSIONS: PBC and AIH are the most common two types in the patients with autoimmune hepatopathy. AIH-I is more often seen clinically. AMA-M2 is the special autoimmune antibody of PBC. The efficacy of prednisolone is better for AIH than PBC.

Adult↗

PubMedDate not supplied

[ Etiopathogenesis of autoimmune thyroiditis].

Human autoimmune thyroid diseases include both autoimmune hypothyroidism (thyroiditis) and autoimmune hyperthyroidism (Graves' disease). Either of the two feature profuse thyroidal infiltrates of CD4+ and CD8+ T-cells and a biased intrathyroidal T-cell-receptor repertoire. Despite strong epidemiologic evidence in favor of a genetic component in the etiology of autoimmune thyroiditis, few hereditary risk factors have been consistently identified, which include a well-characterized series of HLA genes. These may account for the progression from a harmless autoimmune response characterized solely by production of autoantibodies to thyroglobulin to pathogenic autoimmunity where injury occurs to the thyroid cells. We hereafter summarize the role of inherited risk factors along with that of environmental risk factors. It was suggested that iodine increase the autoantigenic potency of thyroglobulin, a major pathogenic antigen in the induction of autoimmune thyroiditis. The clinical entities included together as autoimmune thyroiditis is shortly reviewed, along with the presentation of the common pathogenetic pathways. Unique features of each member of the group are further emphasized.

CD4-Positive T-Lymphocytes↗