Incidence, prevalence, and co-occurrence of autoimmune disorders over time and by age, sex, and socioeconomic status: a population-based cohort study of 22 million individuals in the UK

OP0007 INCIDENCE, PREVALENCE AND CO-OCCURRENCE OF AUTOIMMUNE DISORDERS, TRENDS OVER TIME AND BY AGE, SEX AND SOCIOECONOMIC STATUS. A POPULATION-BASED STUDY IN 22 MILLION INDIVIDUALS.

Celiac Disease Beyond the Gut

Prevalence and Relative Risk of Other Autoimmune Diseases in Subjects with Autoimmune Thyroid Disease

Background: Celiac Disease (CD) is an autoimmune intestinal disorder triggered by gluten exposure in genetically predisposed individuals. It is well described its association with other autoimmune diseases (AIDs), mainly type 1 diabetes mellitus (T1DM) and autoimmune thyroiditis (AT). Age and sex seem to have a role in driving the risk pattern for developing other AIDs among individuals with CD throughout life. Objectives: To study the prevalence of AIDs among pediatric and adult individuals with CD and recognize clinical parameters potentially correlated to such association. Methods: Retrospective study of medical records followed by telephone interview with individuals with CD followed at the Gastroenterology Unit of a tertiary level University hospital. Results: We assessed 328 patients, 235 (72%) females, currently aged from 2 to 74 years (yr), mean age at CD diagnosis (CD-dx) 22 yr (±15.6). From the total, 72 patients (22%) presented at least one associated AID, being 47 females (65.3%), and the CD-dx of this subgroup was 32.5 yr (±12.0). Thirty-one patients presented T1DM (43%): 16 females, mean CD-dx 9 yr (±8.5). Twenty-two patients (30.5%) presented associated AT (Graves' disease or Hashimoto's thyroiditis): 14 females (66.3%), mean CD-dx 35.2 yr (±25.5). Dermatitis herpetiformis was present in 17 patients (23.6%): 12 females (70.6%), mean CD-dx 29.8 yr (± 0.7). Systemic lupus erythematosus was present in 3 patients (4.2%), all females, mean CD-dx 37.5 yr (±7.8). Eight patients (10.8%) presented more than two associated AIDs: 4 females; mean CD-dx 20 yr (±1.4). The overall mean time between the diagnosis of CD and the second AID was 4.3 yr (±2.8). The individuals whose diagnosis of CD was in pediatric ages presented a 72% higher risk of developing a second autoimmunity (p=0.005) compared to adults. The risk of developing T1DM among male celiac patients was 76% higher than females (p=0.013), regardless of age. The risk of developing a third immunity was also 3.2 times higher in males (p=0.047). Conclusion: Although other non-celiac AIDs were more prevalent among females, males are at higher risk for developing T1DM as well as a third autoimmunity. The pediatric group showed to be more vulnerable to develop subsequent AIDs than adults. Those data strength the importance of clinical and serological screening for other AIDs among patients with CD, especially children and adolescents.

Presence of Other Autoimmune Diseases in Subjects with Autoimmune Thyroid Disease

BackgroundAtopic dermatitis is the most common chronic inflammatory skin disease and presents a major public health burden worldwide. Recent observational studies revealed the potential association between atopic dermatitis with autoimmune disorders. However, there is no meta-analysis of the prevalence or incidence of autoimmune diseases in atopic dermatitis. Therefore, considering the potential clinical implications of these associations, we aimed to assess the risk of autoimmune diseases in patients with atopic dermatitis using this method.MethodsPubMed, Embase, and Web of Science were searched from inception to October, 2020. Observational studies which provided estimate effects with 95% CI or raw data were included. The quality of selected studies was evaluated using the Newcastle–Ottawa Scale. Odds ratio and relative risks were pooled using a random effects model and expressed with 95% confidence intervals.ResultsFourteen observational studies were included in this systematic review and meta-analysis. The random-effects meta-analysis of case–control and cross-sectional studies showed a significant association of atopic dermatitis with mutiple autoimmune diseases, including alopecia areata, celiac disease, Crohn’s disease, rheumatoid arthritis, systematic lupus erythematosus, ulcerative colitis and vitiligo. Furthermore, pooling of the results of cohort studies showed that patients with atopic dermatitis were more likely to develop these autoimmune diseases.ConclusionOur meta-analysis showed that patients with atopic dermatitis were at higher risk of multiple autoimmune diseases including alopecia areata, celiac disease, Crohn’s disease, rheumatoid arthritis, systematic lupus erythematosus, ulcerative colitis and vitiligo. It is important for early detection of the affected group so that timely management can be initiated. Dermatologists and allergists should be aware of the autoimmune diseases in patients with atopic dermatitis and develop interventions if necessary. Also, limited by the present research, we still require more large-scale studies to further establish the association between atopic dermatitis and autoimmune diseases.

Purpose A number of patients claim to suffer from diverse systemic symptoms after surgical placement of a polypropylene (PP) implant, including unexplained fatigue, joint pain, and muscle weakness. As these complaints could resemble systemic disease, or Autoimmune disease (AID), commotion among patients exists that PP could induce such disease. The aim of this study was to identify the incidence of AID after PP-implantation, compared to controls where no PP was implanted. Methods In this retrospective cohort study with Dutch health insurer ‘Coöperatie VGZ’, over four million insurees were screened between January 2013 and January 2022, to identify operative procedures for inguinal hernia repair and incisional hernia repair. Cholecystectomy patients were included as controls without PP-implantation. Patients were followed from two years before, to three years after surgery, in which AID diagnoses were registered, as well as age, sex, and socio-economic status. Primary outcomes were incidence of AID after implantation of a PP mesh, and incidence of Not-Otherwise-Specified AID, compared to an index-operation without implant. Results The cohort consisted of 37,723 patients (19,464 inguinal/incisional hernia, 86% male vs. 18,259 cholecystectomies, 32% male). Baseline characteristics were comparable after correction by Propensity Score Matching (N = 1,500/group, Table 1). Incidence of new AID during follow-up was not statistically different between both groups (intervention 0.01 ± 0.11; control 0.01 ± 0.12; p = 0.4). Conclusion Incidence of AID after implantation of PP is comparable to surgery without PP-implantation, in the three years following surgery. Based on this cohort study, PP-implants do not increase medium-term risk for developing AID.Table 1.Patient characteristics after Propensity Score MatchingCharacteristicsNTotal, N = 3,000aHernia-repair with mesh, N = 1,500aCholecystectomy,N = 1,500ap-valuebSex3,000>0.9 Male2,550 (85%)1,275 (85%)1,275 (85%) Female450 (15%)225 (15%)225 (15%)Age3,00061.22 (14.39)61.12 (14.43)61.31 (14.35)0.8SES3,000-0.07 (1.92)-0.06 (1.89)-0.09 (1.95)0.5AID_before3,0000.6 Absent2,949 (98%)1,477 (98%)1,472 (98%) Present51 (1,7%)23 (1.5%)28 (1.9%)AID_after3,0000.01 (0.11)0.01 (0.11)0.01 (0.12)0.4an/N (%); Mean (SD)bFisher's Exact Test for Count Data; Wilcoxon rank sum testAbbreviations: AID, Autoimmune Disease; SES, socio-economic status.

Incidence and Risk Factors for Secondary Autoimmune Diseases (AD) After Cord Blood Transplantation (CBT). Retrospective Analysis on Behalf of Eurocord and the EBMT Autoimmune Diseases Working Party.

Development of Autoimmune Diseases in Women with HPA-1a Alloimmunization and Fetal-Neonatal Alloimmune Thrombocytopenia (FNAIT)

Clinical presentation of celiac disease in the pediatric population

Epidemiology and Estimated Population Burden of Selected Autoimmune Diseases in the United States

BackgroundAutoimmune polyendocrine syndromes (APS) are a heterogeneous group of clinical conditions characterized by functional alteration of one or more endocrine glands and often associated with other systemic autoimmune diseases. Four...

BACKGROUND AND OBJECTIVES:Celiac disease (CD) is an immune-mediated enteropathy, induced by gluten in genetically susceptible individuals. The objective of this study was to describe the clinical pattern of CD in children from the western region of Saudi Arabia.DESIGN AND SETTING:Retrospective, hospital-based.PATIENTS AND METHODS:This study included children with a biopsy-proven diagnosis of CD made between September 2002 and July 2007. Children were admitted to the endoscopy unit for a small-bowel biopsy if they had gastrointestinal symptoms suggestive of CD or if they were positive for a CD-antibody screen performed for the high-risk groups.RESULTS:Eighty children were identified with a diagnosis of CD. Their mean (SD) age was 9.6 (4.9) years (range, 0.5-18 years). There were 44 (55%) female patients. Forty-one (51%) patients were detected during screening of high-risk groups, while 39 (49%) patients had classical symptoms of malabsorption. The screening also detected asymptomatic patients. Of 65 patients tested, 11 (17%) had elevated liver function tests, which reverted to normal after introduction of a gluten-free diet (GFD) except in one case. Seventy-three (91%) patients were positive for anti-tissue transglutaminase antibodies, 18 (23%), for IgG anti-gliadin antibodies; and 46 (58%), for IgA anti-gliadin antibodies. Forty-one (56%) patients showed good adherence to GFD as assessed by dietary history and the decline in anti-tTG level.CONCLUSION:CD may present with classical symptoms or be identified through screening programs. Growth and laboratory abnormalities usually improve after introduction of a GFD. Adherence to a GFD remains a problem; therefore, thorough assessment and counseling at the time of diagnosis and ongoing care are crucial.

PV211 / #363Poster Topic:AS23 - SLE-Diagnosis, Manifestations, & OutcomesBackground/PurposeSystemic lupus erythematosus (SLE) is a complex autoimmune disease that can impact multiple organs, including joints, kidneys, skin, heart, blood cells, lungs, and nervous system. SLE patients face a higher risk of various comorbidities and treatment-related complications, with an increased mortality rate compared to the general population. Studies on observational cohorts have identified cardiovascular diseases, diabetes mellitus type 2 (T2DM), osteoporosis, certain types of cancer, and autoimmune endocrine disease, such as Hashimoto’s thyroiditis (HT), Graves’ disease (GD), type 1 diabetes mellitus (T1DM) and hyperparathyroidism, as common cause of morbidity in SLE patients. However, to our knowledge, no data currently exist on the connection between SLE and Autoimmune Polyendocrine Syndromes (APS), which are rare diseases characterized by multiple autoimmune conditions affecting at least 1 endocrine organ. APS 1 is due to gene AIRE mutations; APS 2 is characterized by Addison’s disease (AD) associated with HT or GD and/or T1DM; HT or GD with any other autoimmune diseases (excluding AD and hypoparathyroidism) fall under APS 3; APS 4 includes remaining combinations of autoimmune forms having an impact on endocrine organs. This study aimed to investigate the prevalence of Autoimmune Polyendocrine Syndromes (APS) in patients with Systemic Lupus Erythematosus (SLE) and to assess whether APS predicts higher disease activity or worse outcomes.MethodsClinical charts of 417 SLE patients referring to our Center between 2021 and 2023 were analyzed. APS cases were identified using ORPHA code definitions; 185 APS-free SLE patients, randomly enrolled, served as controls. Demographic, clinical and serological data were collected.ResultsForty-seven (11%) SLE patients have another autoimmune disease affecting the glands that allows the diagnosis of APS: 39 were diagnosed with HT, 6 with GD, and 3 with T1DM. Forty-five patients were affected by APS type 3, and 2 by APS type 4; no patients were diagnosed with APS type 1 or 2. Table 1 show the sequence in which autoimmune diseases manifest. SLE was the first manifestation of APS in 22 patients (47%). HT was the first autoimmune manifestation for 21 (45%) patients, GD was the first for 2 (4%) patients and 2 women started with rheumatoid arthritis (2%) and autoimmune urticaria (2%), respectively. SLE was the second manifestation in 23 (49%) patients and the fourth for 2 (4%) patients. The comparison between APS+ and APS- patients, as shown in Table 2, revealed no significant differences in clinical or serological features, except for pulmonary hypertension (p=0.044) and renal microangiopathy (p=0.044). At the last evaluation, approximately 80% of both groups’ patients were in clinical remission and approximately half of the patients were still on steroid therapy. APS+ patients had a slightly higher median damage index (SLICC-SDI), although this was not associated with increased disease activity.Table 1.Sequence of autoimmune diseases presentation in patients with SLE and APSTable 2.Comparision of data in SLE patients with and without APSConclusionsThe prevalence of APS among SLE patients is significantly higher than in the general population (11% vs 0,005%), confirming the well-known association between autoimmune thyroiditis and SLE. However, APS+ patients do not appear to have a more aggressive disease or develop more complications. The only clinical conditions statistically associated with APS (renal microangiopathy and pulmonary hypertension) are so rare that no definite conclusions can be drawn. Limitations of the study include a small sample size and single-timepoint data, highlighting the need for larger multicenter studies to clarify the link between SLE and APS.

To describe the frequency of polyautoimmunity and multiple autoimmune syndrome in patients with rheumatoid arthritis (RA) and patients with systemic lupus erythematosus (SLE). This was a cross-sectional observational study of patients with RA, SLE, and controls without autoimmune rheumatic disease. Cases were those with RA according to the 2010 American College of Rheumatology/European League Against Rheumatism criteria and SLE according to the 2019 American College of Rheumatology/European League Against Rheumatism criteria, consecutively recruited in a rheumatology clinic. Controls were subjects with no rheumatic autoimmune disease (AIDs) recruited in the same area. Patients filled out a questionnaire on polyautoimmunity. Variables of interest were polyautoimmunity (RA or SLE with other AIDs), whereas secondary variables were rheumatic, skin, endocrine, digestive, and neurological AIDs. Multiple autoimmune syndrome is defined as the presence of 3 or more AIDs and a family history of AIDs. Statistical analyses performed were descriptive, bivariate, and multivariate (dependent variable: polyautoimmunity). The study population comprised 109 patients with RA, 105 patients with SLE, and 88 controls. Polyautoimmunity was recorded in 15 patients with RA (13.8%), 43 with SLE (41%), and 2 controls (2.2%). The most frequent AID in RA was Sjögren syndrome (53.3%), followed by Hashimoto thyroiditis and psoriasis; the most frequent AIDs in SLE were Sjögren syndrome (55.8%) and antiphospholipid syndrome (30.2%), followed by Hashimoto thyroiditis. Obesity was associated with polyautoimmunity in RA (odds ratio [OR], 3.362; p = 0.034). In SLE, joint damage (OR, 2.282; p = 0.038) and anti-RNP antibodies (OR, 5.095; p = 0.028) were risk factors for polyautoimmunity, and hydroxychloroquine was a protective factor (OR, 0.190; p = 0.004). Polyautoimmunity is frequent in RA and even more frequent in SLE. It was associated with obesity in RA and with joint damage and anti-RNP in SLE. Hydroxychloroquine was a protector.