Presence Of Other Autoimmune Diseases Research Articles

Integrative Review on Silicone Breast Implants and ASIA Syndrome: What is the relationship?

OBJECTIVE: The study aims to indicate the correlation between breast implants and the occurrence of ASIA Syndrome. METHODOLOGY: The articles were selected from the PubMed database, published between 2016 and 2021. The research used the Health Science Descriptors (DeCS) and their synonyms, in English: breast implants, silicones, autoimmune diseases and rheumatic diseases . RESULTS: Despite the divergent results between the studies analyzed, in most cases the correlation is evident between breast implants and the occurrence of autoimmune diseases. DISCUSSION: ASIA syndrome is involved as an adjuvant factor for the development of autoimmunity, as there is an exacerbation of the immunological response, with the release of cytokines and defense cells in the body with the presence of breast implants. The diagnosis is made based on the patient's symptoms and past history, in which there are characteristic symptoms. The main treatment is the extraction of breast implants. There is an important correlation between low vitamin D levels and ASIA syndrome, in addition to the presence of other autoimmune diseases. CONCLUSION: Breast implants can be an adjuvant for the occurrence of ASIA syndrome, being reaffirmed by the improvement factors after silicone removal.

Footprints of Stress in Vitiligo: Association of the 5-HTR2C rs6318 Variant

Abstract Vitiligo is a chronic, progressive autoimmune dermatological disease, and stress is known to have an impact on the development of vitiligo. However, the effect of the serotonin pathway and its impact have not been clearly explained for disease progression. Thus, this study aimed to clarify the stress induced serotonin receptor 5-HTR2C rs6318 variant and its association with vitiligo pathogenesis. Case-control study was conducted with 108 vitiligo patients and 107 age-sex matched, unrelated healthy control group. Real Time-PCR analysis was used for genotyping the 5-HTR2C variation. Genotype and allele frequencies, genotype distributions, Hardy-Weinberg Equilibrium (HWE) and vitiligo-related risk measurements were examined. Genotype correlations of the variant were also analyzed based on gender difference, age onset, Koebner phenomenon history, triggered with stress, clinical subgroups, treatment types, the presence of other autoimmune diseases, vitiligo presence in family members and other auto-immune diseases in relatives. Statistical differences in 5HT-R2C receptor genotypes and allele frequencies between patients and controls were not detected. Genotype frequencies were not in agreement with Hardy-Weinberg Equilibrium in the patients’ group (p<0.00001). The frequency of the risk allele (allele C) was not significantly different between the patient and control groups (p=0.1392). However, in the clinical subgroup analysis, the risk allele presence was detected to be significantly higher for early age onset (<40 years) vitiligo development (p=0.035, OR=Infinity, RR=1.391) and lower in Koebner phenomenon history (p=0.0276, OR= 0.219, RR=0.325). In conclusion, although there was no association between the 5-HTR2C variant rs6318 and vitiligo, current results indicate that there is an association between the 5HTR2C rs6318 variant C allele and early onset vitiligo development.

Epidemiological and clinical characteristics of alopecia areata in a tertiary care center in Western Odisha

Alpoecia areata is a common autoimmune disease often presenting with patchy areas of hair loss. Personal or family history of atopy and presence of other autoimmune diseases are often associated with this condition. The present study was conducted with the objective of finding the epidemiological factors, clinical patterns and associated comorbidities associated with alopecia areata. It was a single centre, hospital based cross sectional study undertaken between the period March 2017 to March 2020. Clinically diagnosed cases of alopecia areata were included in the study. Epidemiological data such as age, gender, locality, educational status and occupation were taken into consideration. Various clinical patterns such as patchy area of hair loss and more severe variants such as alopecia totalis, alopecia universalis etc were noted. The data were obtained through pre-structured questionnaire, findings of clinical examination and laboratory data.A total of 531 cases of clinically diagnosed alopecia areata were included with an overall prevalence of 4.1%. The mean age at onset was 27.4 ± 12.92 years. Males outnumbered females (1.31:1). Salaried persons (34.08%) belonging to upper socioeconomic status (31.6%) were predominantly affected. Hypothyroidism 29(5.46%) and bronchial asthma 14(2.6%) were the most common comorbidities. Educated, employed persons in the urban area are more susceptible to develop alopecia areata. Comorbidities like thyroid dysfunction, bronchial asthma and diabetes mellitus must be looked for in all such cases.

CLINICAL CASE OF OVERLAP SYNDROME OF AUTOIMMUNE DISEASES

We are presenting a rare case of overlap of Sjögren’s syndrome (SS) and Celiac disease (CD). It is necessary to evaluate and diagnose timely the possible presence of other autoimmune diseases in the presence of any autoimmune disease, because they have about a 25% risk of developing several of them at the same time, which is called overlap syndrome. It is very important to do screening, follow up and regular GI care of Sjögren’s syndrome patients to identify CD case and help them to avoid severe malnutrition, hyponatremia, anaemia and later intestinal malignancies.

Emotional eating and disordered eating behaviors in children and adolescents with type 1 diabetes

Disordered eating behaviors (DEB) are more common in adolescents with type 1 diabetes (T1D) than in peers without diabetes. Emotional eating is a risk factor for binge eating in children and adolescents in the general population and is associated with increased intake of high energy-dense foods rich in sugars and fats. The primary objective is to evaluate whether emotional eating is associated with the metabolic control (glycated hemoglobin, plasma lipids and uric acid) in children and adolescents with type 1 diabetes and whether subjects with DEB (DEPS-R ≥ 20) have higher emotional eating than those without DEB. The secondary objective is to evaluate whether emotional eating is associated with the different symptoms of DEB. Emotional eating is positively correlated with HbA1c, total and LDL cholesterol values in children and adolescents with T1D. Subjects with DEB have a higher emotional eating score than subjects without DEB. Disinhibition is the most common disordered eating behavior in children and adolescents with T1D and is associated with a higher emotional eating score. Early identification and treatment of emotional eating could be tools for preventing DEB in people with type 1 diabetes. A total of 212 adolescents with T1D completed two self-administered questionnaires: the Diabetes Eating Problem Survey-Revised (DEPS-R) and the Emotional Eating Scale for Children and Adolescents (EES-C). Demographic (age, sex, duration of the disease), anthropometric (weight, height, BMI, BMI-SDS), therapeutic (type of insulin therapy, daily insulin dose) and metabolic (HbA1c, total cholesterol, HDL, LDL, triglycerides, uric acid) data were taken from the patients' medical records. The presence of other autoimmune diseases was also recorded.

Inflammatory Bowel Diseases Are Associated with Polymyositis and Dermatomyositis-A Retrospective Cohort Analysis.

Background and Objectives: Polymyositis and dermatomyositis (PM/DM) are classified as polygenic autoimmune diseases, whereas inflammatory bowel disease (IBD) is considered a polygenic autoinflammatory disease. In the literature, several cases exist reporting the co-occurrence of both conditions. At the molecular level, PM/DM and IBD share common genetic determinants including interferon regulatory factor and vitamin D receptor susceptibility loci. Accumulating evidence underline several indicators that confer poor prognosis in IBD, including antinuclear antibody positivity and the presence of other autoimmune diseases, therefore the aim of this study is to assess the association between these entities. Materials and Methods: This is a population-based retrospective study using data retrieved from a large electronic medical record in Israel, the Clalit health registry. The sample included PM/DM patients and age- and sex-frequency matched controls. The prevalence of IBD in PM/DM was compared between the two groups and logistic regression was applied to control for confounding variables. Predictors of IBD in patients with PM/DM were also explored. Results: Our study included 12,278 subjects with 2085 PM/DM patients and 10,193 age- and sex- frequency-matched controls. The incidence of IBD in patients with PM/DM was significantly higher even after controlling for various confounding variables (OR of 1.73, 95% CI 1.05-2.86, p-value = 0.033). Anti-nuclear antibodies (ANA) positivity was found to be an independent predictor for IBD diagnosis in patients with PM/DM (OR 3.67, 95% CI 1.01-13.36, p = 0.048). Conclusion: Our analysis reports an association between IBD and PM/DM. Such association could point towards a common pathophysiological background. Further research is needed to further describe the clinical courses and whether a unique therapeutic approach is warranted.

Comorbid autoimmune diseases and burden of diabetes-related complications in patients with type 1 diabetes from a Mediterranean area

AimTo assess the prevalence of autoimmune diseases (AID) in patients with type 1 diabetes (T1D) and to evaluate whether the rate of diabetes-related complications differs depending on the presence of AID. MethodsCross-sectional analysis of 13,570 T1D patients aged ≥ 18 years registered in the SIDIAP database. The association between AID and diabetes-related complications was assessed by multivariable logistic regression models. ResultsThe prevalence of AID was 18.3% with thyroid AID being the most common. Patients with T1D and AID were more often female and their current age, age of diabetes onset and diabetes duration were higher. Patients with only thyroid AID experienced a lower risk of peripheral artery disease (odds ratio [OR] = 0.51, 95%; confidence interval [CI] 0.31 to 0.81) and kidney disease (OR = 0.68, 95%; 95% CI 0.54 to 0.85), whereas patients with other AID had an increased risk of ischemic heart disease (OR = 1.48, 95%; 95% CI 1.04 to 2.06). ConclusionsThe burden of diabetes-related complications in patients with T1D differs according to the type of additional AID. The presence of diabetes complications is lower in those with autoimmune thyroid disease while the presence of other AID is associated with higher rates of ischemic heart disease.

Differences in Endothelial Activation and Dysfunction Induced by Antiphospholipid Antibodies Among Groups of Patients With Thrombotic, Refractory, and Non-refractory Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by pregnancy morbidity or thrombosis and persistent antiphospholipid antibodies (aPL) that bind to the endothelium and induce endothelial activation, which is evidenced by the expression of adhesion molecules and the production of reactive oxygen species (ROS) and subsequent endothelial dysfunction marked by a decrease in the synthesis and release of nitric oxide (NO). These endothelial alterations are the key components for the development of severe pathological processes in APS. Patients with APS can be grouped according to the presence of other autoimmune diseases (secondary APS), thrombosis alone (thrombotic APS), pregnancy morbidity (obstetric APS), and refractoriness to conventional treatment regimens (refractory APS). Typically, patients with severe and refractory obstetric APS exhibit thrombosis and are classified as those having primary or secondary APS. The elucidation of the mechanisms underlying these alterations according to the different groups of patients with APS could help establish new therapies, particularly necessary for severe and refractory cases. Therefore, this study aimed to evaluate the differences in endothelial activation and dysfunction induced by aPL between patients with refractory obstetric APS and other APS clinical manifestations. Human umbilical vein endothelial cells (HUVECs) were stimulated with polyclonal immunoglobulin-G (IgG) from different groups of patients n = 21), including those with primary (VTI) and secondary thrombotic APS (VTII) and refractory primary (RI+), refractory secondary (RII+), and non-refractory primary (NR+) obstetric APS. All of them with thrombosis. The expression of adhesion molecules; the production of ROS, NO, vascular endothelial growth factor (VEGF), and endothelin-1; and the generation of microparticles were used to evaluate endothelial activation and dysfunction. VTI IgG induced the expression of adhesion molecules and the generation of microparticles and VEGF. RI+ IgG induced the expression of adhesion molecules and decreased NO production. RII+ IgG increased the production of microparticles, ROS, and endothelin-1 and reduced NO release. NR+ IgG increased the production of microparticles and endothelin-1 and decreased the production of VEGF and NO. These findings reveal differences in endothelial activation and dysfunction among groups of patients with APS, which should be considered in future studies to evaluate new therapies, especially in refractory cases.

Long-term immunosuppression and multiple transplants predispose systemic lupus erythematosus patients with cytopenias to hematologic malignancies.

Cytopenias in systemic lupus erythematosus (SLE) require clinical and laboratory workup and bone marrow (BM) examination to determine the cause and for appropriate patient management. Common causes include an increase in SLE activity, immune-mediated hemolysis, iron deficiency, antiphospholipid antibody syndrome, infection, or the effect of medications. We retrospectively evaluated the clinical and laboratory findings of patients with SLE and cytopenias who had undergone BM studies to determine the indicators of malignancy.We retrospectively reviewed medical records of patients with SLE who presented with cytopenias for their disease course, medications, laboratory parameters and documented the spectrum of morphological changes in BM including CD34 expression.Twenty patients with SLE had undergone BM biopsy for evaluation of cytopenias. 14/20 (70%) of the patients had reactive BM, and the rest had hematologic malignancies involving the BM. Of these 14 patients, 8 had hypocellular marrow with loss of precursor cells (low CD34), 4 had left shift in myeloid lineage, 3 had serous atrophy, and 1had multilineage dysplasia. The 6 patients with hematologic malignancies included 2 with diffuse large B cell lymphoma, and one each of natural killer/T cell lymphoma, post-transplant lymphoproliferative disorder, Hodgkin lymphoma, and myelodysplastic syndrome evolving to acute myelogenous leukemia. The presence of autoantibodies, SLE activity, and lupus nephritis were comparable in patients with and without neoplasia. However, the duration of the use of multiple immunosuppressants, years since renal transplant (22 vs 10), multiple transplants, and the presence of other autoimmune diseases were greater in those with neoplasia. Two of the 14 patients with non-neoplastic BM and 1 with the neoplastic BM had nonhematological malignancy.Clinical and laboratory findings, the number of transplants, and the use of immunosuppressive agents can guide physicians to identify patients with a higher risk of developing hematologic malignancy. BM findings of cytopenia in SLE are often due to increased disease activity causing global cell death and dysmaturation. SLE patients presenting with cytopenias, with a history of long-term exposure to immunosuppressive drugs, should be regularly screened for hematologic and nonhematologic malignancies.

Celiac disease in pediatric patients according to HLA genetic risk classes: a retrospective observational study

BackgroundCeliac disease (CD) is an autoimmune enteropathy in which HLA-DQ haplotypes define susceptibility. Our aim was to evaluate if belonging to a certain HLA-DQ class risk could be associated to the clinical, serological and histological presentation of CD.MethodsWe performed a retrospective observational monocentric study including all 300 patients diagnosed with CD, who underwent HLA typing. Clinical, serological and histological data was collected from clinical records and their association with HLA-DQ class risk was verified through statistical tests.ResultsIn our sample mean age at onset was 6.7 ± 4.2 years, with a prevalence of females (n = 183; 61%), typical symptoms (n = 242; 80.6%) and anti-tTG IgA ≥ 100 U/mL (n = 194; 64.7%). Family history was present only in 19% (n = 57) of patients, and it was not significantly associated with any of the clinical and demographical data analyzed or the belonging to a certain HLA-DQ class risk. We found in the male population more frequently a coexistence of CD and atopic syndrome (males: n = 47; 40.2%; females: n = 50; 27.3%; p = 0.020).Early age of onset, instead, was associated with typical symptoms (m = 6.4 ± 4; p = 0.045) and elevated liver enzymes (m = 5 ± 3.8; p < 0.001), while later age of onset was associated with presence of other autoimmune diseases (m = 8.2 ± 4; p = 0.01).We observed statistically significant influences of HLA class risk on antibodies and liver enzymes levels: G1, G4 and G2 classes showed more frequently anti-tTG IgA ≥ 100 U/mL (n = 44; 80%, n = 16; 69.6%, n = 48; 67.6% respectively; p-value = 0.037), and in patients from G2 class we found enhanced liver enzymes (n = 28; 39.4%; p-value = 0.005). HLA class risk was still significantly associated with anti-tTG ≥ 100 (p = 0.044) and with hypertransaminasemia (p = 0.010) after a multiple logistic regression adjusted for the effect of gender, age at onset and family history.ConclusionsWe failed to prove an association between HLA-DQ genotypes and the clinical features in our CD pediatric patients. Although, our results suggest an effect of the DQB1–02 allele not only on the level of antibodies to tTG, but possibly also on liver involvement.

Clinical outcomes of transverse myelitis with myelin oligodendrocyte glycoprotein antibody versus negative cases among adults in Isfahan, Iran: A comparative study

Background: The aim of this study was to evaluate the status of anti-myelin oligodendrocyte glycoprotein (MOG) antibodies in patients with transverse myelitis (TM) and compare the clinical and imaging characteristics of MOG immunoglobulin G (IgG)-positive with negative cases. Methods: This cohort study enrolled 71 patients diagnosed with new-onset of TM who were being followed at a referral university clinic in Isfahan, Iran, from November 2016 to January 2019. Magnetic resonance imaging (MRI) images and blood samples for anti-MOG, anti-aquaporin 4 (anti-AQP4) (using the cell-based technique), and vasculitis-related antibodies were collected from patients. Outcomes were assessed by the evolution of the Expanded Disability Status Scale (EDSS) score and brain and spinal cord imaging findings within three months. All patients underwent imaging and clinical assessment during a mean period of one year as a follow-up. We compared the characteristics of clinical and radiological outcomes in MOG-IgG-positive and negative cases. Results: Of the total population studied, there were 26.8% men and 73.2% women, with a mean age of 33 ± 10 years. 12 (16.9%) patients were seropositive for MOG antibody and 17 (89.5%) were positive for anti-AQP4 antibodies. There was no significant association between anti-MOG antibody seropositivity and age, gender distribution, the presence of other autoimmune diseases, and number and interval of relapses. However, the involvement site of the spine at first imaging was significantly different between seronegative and seropositive patients. Conclusion: In patients with MOG antibody disease (MOG-AD) TM, the MRI findings suggest a preferential involvement of the cervical-thoracic section in seropositive cases which may help differentiate from non-MOG demyelination TM.

Anaesthesia and orphan disease: A patient with neuromyotonia undergoing single lung ventilation.

Anaesthesia and orphan disease: A patient with neuromyotonia undergoing single lung ventilation.

Latent autoimmune thyroid disease

ObjectiveTo determine the prevalence of thyroid autoantibodies and the associated factors in euthyroid subjects. Methods300 euthyroid subjects, chosen by stratified sampling from an inception cohort of 1335 individuals, were included. Thyroid function was evaluated by measuring the serum levels of TSH (0.3–4.5 μIU/mL) and FT4 (5.2–12.7μg/dL). Anti-peroxidase (TPOAbs), anti-thyroglobulin (TgAbs), and anti-TSH receptor (TrAbs) antibodies were evaluated with 23 additional autoantibodies as well as vitamin D (VitD) levels. The analysis included sociodemographic, clinical, and environmental characteristics. Data were analyzed by bivariate and multivariate tests. ResultsThyroid autoimmunity was observed in 15.3% of the subjects (TPOAbs 11.3% and TgAbs 2.0%). In six individuals, both autoantibodies were positive. TrAbs were not detected in any individual. Familial thyroid disease (β ​= ​3.4, 95% CI: 1.2–9.5, P ​= ​0.021), the presence of other autoimmune diseases (β ​= ​10.8, 95% CI: 1.6–72.9, P ​= ​0.014) VitD insufficiency (P ​= ​0.030), never smoke (β ​= ​6.9, 95% CI: 1.6–30.4, P ​= ​0.010), drinking more than 4 cups of coffee (β ​= ​3.8, 95% CI: 1.1–13.1, P ​= ​0.036), and a higher number of years exposed to wood smoke (P ​= ​0.04) were associated with thyroid autoimmunity. In the case of TPOAbs, familial thyroid disease (β ​= ​4.9, 95% CI: 1.7–14.0, P ​= ​0.003), never smoke (β ​= ​5.7, 95% CI: 1.4–21.0, P ​= ​0.002), and drinking more than 4 cups of coffee (β ​= ​3.6, 95% CI: 1.1–13.1, P ​= ​0.047) were associated with their positivity. In addition, the presence of anti–SS–A/Ro52 (β ​= ​36.7, 95% CI: 2.5–549.9, P ​= ​0.009) and anti-Ku antibodies (β ​= ​10.2, 95% CI: 1.1–100.7, P ​= ​0.046) was also associated with TPOAbs. The presence of African ancestry (β ​= ​10.5, 95% CI: 1.7–63.2, P ​= ​0.01), anti–SS–A/Ro52 (β ​= ​15.8, 95% CI: 1.2–198.6, P ​= ​0.03), and anti-CENP-B antibodies (β ​= ​31.2, 95% CI: 1.8–565.9 ​P ​= ​0.02) were associated with TgAbs. ConclusionLatent thyroid autoimmunity is not rare. Environmental, genetic, and immunological factors as well as ancestry are associated risk factors. These results would facilitate the implementation of screening strategies in order to provide timely diagnosis and treatment.

Factors Associated With Potential Progressive Course of Primary Biliary Cholangitis: Data From Real-world US Database.

Although relatively, primary biliary cholangitis (PBC) is an important cause of nonalcoholic chronic liver disease which may lead to liver transplantation. PBC patients with alkaline phosphatase (ALP) ≥1.5× the upper limit of normal (ULN) tend to have a more aggressive course. The study was designed to identify factors associated with ALP≥1.5×ULN or cirrhosis in PBC and to evaluate concomitant health care resource utilization. We used a large real-world database that contained comprehensive and continuous electronic medical recored/claims data from over 500 health care practices or systems from the United States. Of 195 million patients included in the database, 36,317 were adults with PBC. After applying exclusion criteria, 15,875 patients comprised the final PBC cohort (63.0±13.5 y, 78% female, 71% privately insured, 5% covered by Medicaid, 57% with other autoimmune diseases, 46% with cirrhosis); 6083 (38%) had ALP≥1.5×ULN. Patients with ALP≥1.5×ULN were more frequently female, less covered by Medicaid, had more pruritus, cirrhosis, and other autoimmune diseases (P<0.05). In multivariate analysis, older age, female gender, the presence of other autoimmune diseases, and having compensated or decompensated cirrhosis were independently associated with having ALP≥1.5×ULN in PBC (P<0.05). In contrast, being male was associated with higher risk of cirrhosis in PBC [odds ratio 2.3 (95% confidence interval, 2.1-2.5)]. Patients with ALP≥1.5×ULN and/or with cirrhosis also incurred substantially more health care resource utilization (P<0.05). Many clinical, sociodemographic, and economic factors are associated with a potentially more aggressive profile of PBC with elevated ALP. These data may inform clinicians to implement management strategies to optimize care of these patients.

Factors Affecting Generalization of Ocular Myasthenia Gravis in Patients With Positive Acetylcholine Receptor Antibody

To evaluate the associated factors of conversion of ocular myasthenia gravis (OMG) to generalized myasthenia gravis (GMG) among patients with seropositive acetylcholine receptor antibody (AchR Ab). Retrospective cohort study. Setting: Retrospective chart review. Seventy-one OMG patients with seropositive AchR Ab presented during July 2009 and December 2016. The exclusion criteria were patients who (1) first presented with GMG, (2) were unable to identify the time of onset of OMG or GMG, (3) were unable to provide information about previous treatments before the onset of GMG, and (4) had incomplete or lost medical records. We collected demographic and clinical characteristics, including onset of OMG and GMG, presence of other autoimmune disorders, history of smoking, presence of thymic abnormalities, and medications received. Conversion to GMG and time to conversion. Thirty-six patients experienced conversion to GMG. Overall incidence of GMG was 14 (95% confidence interval [CI] 10.09-19.4) per 100 patient-years. Probability of conversion at 2 years was .37 (95% CI .27-.49). Overall median conversion time was 4.97 years. Cox proportional hazard model showed that risk factors were female sex (HR 2.52, 95% CI 1.04-6.10), history of smoking (HR 3.42, 95% CI 1.40-8.45), and thymic abnormalities (HR 1.82, 95% CI 0.91-3.67). Protective factors against conversion to GMG were receiving immunosuppressive agents (HR 0.42, 95% CI 0.19-0.97) and pyridostigmine (HR 0.37, 95% CI 0.14-0.93). OMG patients with seropositive AchR Ab should be informed that taking pyridostigmine and/or immunosuppressive agents as well as smoking cessation might prevent conversion to GMG.

Non-albuminuric reduced eGFR phenotype in children and adolescents with type 1 diabetes

AimsTo analyze the factors associated with non-albuminuric reduced estimated glomerular filtration rate (NAeGFR-) phenotype in young people with type 1 diabetes (T1DM). MethodsIn this cross-sectional study were enrolled 140 outpatient diabetic children (age 7–18 years), consecutively observed in the period 2016–2017. Eighteen subjects with microalbuminuria (defined as albumin excretion rate ≥ 30 mg/24 h) were excluded. Fasting HbA1c, uric acid (UA), neutrophils and lymphocytes count were recorded. Estimated glomerular filtration rate (eGFR) was calculated using the Schwartz’s bed-side formula and reduced eGFR was defined by a value <90 mL/min/1.73 m2. ResultsOut of 122 subjects analyzed, 76 (62%) showed normal eGFR and 46 (38%) showed NAeGFR- phenotype. They were characterized by higher prevalence of male sex (57% vs 33%, p = 0.010), autoimmune diseases (26% vs 12%, p = 0.043), high UA levels (4.0 ± 0.9 vs 3.3 ± 0.9 mg/dl, p < 0.0001) and high Neutrophils/Lymphocytes ratio (1.5 [1.2–2.0] vs 1.3 [1.0–1.8], p = 0.023). ConclusionsIn our population, the prevalence on NAeGFR- phenotype is 38% and it is associated with male sex, high levels of UA, presence of other autoimmune diseases and low-grade inflammation. It should encourage pediatricians to monitor early both eGFR and UA in order to intercept diabetic youth more likely prone to develop progressive renal impairment.

MON-594 Report Of A Large Series Of Patients With Graves' Disease (with/without Graves' Ophthalmopathy) And Review Of The Literature, About The Association With Other Autoimmune Diseases

The two main clinical presentations of autoimmune thyroid disorders (AITD) are Graves’ disease (GD) and autoimmune thyroiditis (AT). The associations between thyroid disorders and other organ specific, or systemic autoimmune diseases in the same patient has been suggested, albeit discordant results have been reported in the literature. We focused on the association between GD and other autoimmune syndromes by searching in the literature. Moreover, we have carried out a prospective study investigating about the prevalence of other autoimmune disorders in 3209 GD patients [984 of whom with Graves’ ophthalmopathy (GO)], with respect to 1069 healthy controls, or 1069 with multinodular goiter, or 1069 patients with AT. Patients and controls were matched by age and gender, come from the same area, and had a similar iodine intake. A significant increase of the prevalence of autoimmune disorders in GD patients, with respect to the controls, has been observed. In particular, the most frequently observed associations were: vitiligo (2.6%), chronic autoimmune gastritis (2.4%), rheumatoid arthritis (1.9%), polymyalgia rheumatica (1.3%), multiple sclerosis (0.3%), celiac disease (1.1%), type 1 diabetes (0.9%), systemic lupus erythematosus and sarcoidosis (<0.1%), Sjogren disease (0.8%). In addition, 1.5% patients with GD had three associated autoimmune disorders. The prevalence of autoimmmune disorder in GO patients is higher (18.9%), with respect to GD patients without GO (15.6%). The pattern of the associated autoimmune disorders in GD was not significantly different from that observed in AT patients. These findings suggest to appropriately screen GD patients, who are still sick, or who develop new unspecific symptoms (even if during an appropriate treatment of hyperthyroidism), for the presence of other autoimmune disorders.

Stopping immunosuppressive treatment in autoimmune hepatitis (AIH): Is it justified (and in whom and when)?

Initial treatment of autoimmune hepatitis (AIH) with prednisolone±azathioprine is based on randomised controlled trials. Many patients receive long-term immunosuppressive treatment to prevent disease relapse; this strategy has a weaker evidence base. To consider whether immunosuppressive treatment (IST) withdrawal in AIH is justified and to develop a rationale for patient selection. We reviewed published papers between 1972 and 2018, which addressed the outcomes of IST withdrawal and/or complications of IST in AIH. (1) AIH relapse rates after withdrawal of IST vary between 25% and 100%. There is heterogeneity in these studies regarding relapse definition, IST duration prior to withdrawal and criteria for biochemical and histological remission prior to withdrawal. (2) Factors associated with relapse following IST withdrawal include: (a) absence of an identifiable initial disease trigger, (b) presence of other autoimmune diseases, (c) longer time to biochemical remission and (d) elevated serum transaminases on treatment withdrawal. Reports of associations between relapse and age, IST duration and failure of histological remission have been inconsistent. (3) Continued IST reduces risk of AIH relapse over at least 5years. However, there is no evidence that routine (as opposed to selective) long-term IST improves disease outcome. (4) Patients with AIH have an increased risk of extrahepatic cancer, notably non-melanoma skin cancer, to which long-term IST may contribute. Long-term corticosteroid therapy is associated with weight gain, low-trauma fractures, diabetes and possibly vascular disease. While further studies are needed, evidence supports a strategy of IST withdrawal in some patients with AIH who have achieved remission.

The association of other autoimmune diseases in patients with Graves' disease (with or without ophthalmopathy): Review of the literature and report of a large series.

Graves' disease (GD) and autoimmune thyroiditis (AT) are the two main clinical presentations of AITD, and their clinical hallmarks are thyrotoxicosis and hypothyroidism, respectively. GD, and AT, can be associated with other organ specific, or systemic autoimmune diseases in the same patient. However discordant results have been reported in the literature about the possible associations. Here, we review the association of GD and other autoimmune syndromes. Furthermore, we report the results of our prospective study that investigated the prevalence of other autoimmune disorders in 3209 GD patients (984 with Graves' ophthalmopathy), with respect to 1069 healthy controls, or 1069 patients with AT, or 1069 with multinodular goiter (matched by age, gender, coming from the same area, with a similar iodine intake). On the whole, 16.7% of GD patients had another associated autoimmune disease; and the most frequently observed were: vitiligo (2.6%), chronic autoimmune gastritis (2.4%), rheumatoid arthritis (1.9%), polymyalgia rheumatica (1.3%), multiple sclerosis (0.3%), celiac disease (1.1%), diabetes (type 1) (0.9%), systemic lupus erythematosus and sarcoidosis (<0.1%), Sjogren disease (0.8%). Moreover, 1.5% patients with GD had three associated autoimmune disorders. Interestingly, patients with Graves' ophthalmopathy (GO) had another autoimmmune disorder more frequently (18.9%), with respect to GD patients without GO (15.6%). However the pattern of the associated autoimmune disorders in GD was not significantly different from that observed in AT patients. In conclusion, we suggest GD patients who are still sick, or who develop new unspecific symptoms (even if during an appropriate treatment of hyperthyroidism) should be appropriately screened for the presence of other autoimmune disorders.

A Retrospective Analysis of Ten Patients with Normal Alkaline Phosphatase and Primary Biliary Cholangitis

Introduction: Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease which can be diagnosed when a patient tests positive for positive antimitochondrial antibody (AMA) and has a high alkaline phosphatase (AP) without the need for undergoing a liver biopsy. However, medical literature has documented that PBC can occur among patients with normal AP. As a result in patients worked up for common liver conditions like hepatitis and fatty liver, an incidental diagnosis of concomitant PBC can be missed if AMA is not ordered. We routinely perform AMA on patients who are evaluated for liver disease based on either imaging studies or abnormal liver tests and over last ten years came across patients with positive AMA and normal or near normal liver tests. We want to present this case series to highlight the importance of working up patients thoroughly with a battery of liver tests and if AMA is positive doing a liver biopsy to confirm the diagnosis of PBC.2732 Figure 1. Results of the study showing patient demographics, AMA range, ALP levels, presence of other autoimmune diseases and Liver biopsy resultsMethods: The charts of 10 patients with normal cholestatic liver enzymes and AMA positivity who underwent liver biopsy between 2012-2017 were retrospectively analyzed. Records were reviewed, cause of death verified when applicable, and all available clinical and biochemical data were collected, including detailed reports of liver histology. Only patients naïve to ursodeoxycholic acid were included in this case series. Results: Among the patient population, eight were females; median age was 56 years; median AMA titer was 50.3(lab reference 0-20); extrahepatic autoimmune disorders were present in five of ten patients including systemic lupus erythmatosis, Rheumatoid arthritis, Sjogren's syndrome, scleroderma and undifferentiated connective tissue disorder(Table 1). Two patients had cirrhosis on liver biopsy. The liver histology findings revealed PBC in all ten patients: 4 patients had early PBC changes, 2 patients had PBC with cirrhosis; 2 patients had PBC stage 2/4; and one patient each had changes of PBC associated with HCV and nonalcoholic steatohepatitis. Conclusion: AMA positive patients with normal cholestatic liver enzymes should be considered for a liver biopsy to establish a diagnosis of PBC. Based on this case series and data recently published in the literature, AMA should be ordered in patients worked up for common liver diseases. In the future, cost analysis of ordering AMA and the downstream benefit of deferring decompensated liver disease among PBC patients should be done.