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The complement receptor of the immunoglobulin superfamily (CRIg, encoded by the VSIG4 gene) is a macrophage receptor involved in the clearance of immune complexes and autologous cells. Our results suggest that the VSIG4 rs1044165T allele is a risk factor for severe functional status of rheumatoid arthritis in women, possibly by affecting VSIG4 gene expression.

BACKGROUND: Celiac disease (CD) is an autoimmune disease characterized by immune reaction mostly to wheat gluten. The diagnosis is based on clinical, serological and histological findings in patients ingesting gluten. Cases that the clinical profile indicates CD and the autoantibodies are negative bring so a dilemma for the professional, as the risk of missed the diagnosis or a delay at the same. OBJECTIVE: To show the importance of correct diagnosis of cases with seronegative celiac disease (SNCD). METHODS: Ten cases of SNCD Brazilian patients were retrospectively studied (2013 to 2019). Data of clinical complaints, autoantibodies, IgA serum levels, histological findings and HLA-DQ2/DQ-8 were compiled. Dual-X densitometry, delay at diagnosis, previous autoimmune diseases and family history of CD were also checked. RESULTS: All SNCD patients presented clinical symptoms of CD, with confirmed diagnosis by histological findings of the duodenal mucosa and HLA-DQ2 and/or HLA-DQ8 positivity. All patients had normal IgA levels and negative autoantibodies (IgA-anti-transglutaminase and anti-endomysial). Dual-X densitometry detected osteopenia in two women and osteoporosis in two males, all with low levels of vitamin D. Delay diagnostic ranged from 1 to 19 years. Familiar occurrence of CD was reported in 40% of the cases. After one year of gluten-free diet, eight patients refer improve of symptoms, while duodenal biopsies, done in five cases, showed histological improvement. CONCLUSION: Patients who demonstrate the clinical profile of celiac disease with negative serology and normal levels of IgA, especially those who have family members with celiac disease, should be submitted to duodenal biopsies to look for histological findings. RESUMO CONTEXTO: A doença celíaca (DC) é uma doença autoimune caracterizada por reação imune principalmente ao glúten do trigo. O diagnóstico é baseado em achados clínicos, sorológicos e histológicos em pacientes que ingerem glúten. Casos em que o perfil clínico indica DC e os autoanticorpos são negativos trazem um dilema para o profissional, como o risco de não realizar ou atrasar o diagnóstico da DC. OBJETIVO: Mostrar a importância do diagnóstico correto de casos com doença celíaca soronegativa (DCSN). MÉTODOS: Dez casos de pacientes brasileiros com DCSN foram estudados retrospectivamente (2013 a 2019). Foram compilados dados de queixas clínicas, autoanticorpos, níveis séricos de IgA, achados histológicos e HLA-DQ2 / DQ-8. Densitometria, atraso no diagnóstico, doenças autoimunes prévias e histórico familiar de DC também foram verificados. RESULTADOS: Todos os pacientes com DCSN apresentaram sintomas clínicos de DC, com diagnóstico confirmado por achados histológicos da mucosa duodenal e positividade para HLA-DQ2 e/ou HLA-DQ8. Todos os pacientes apresentavam níveis normais de IgA e autoanticorpos negativos (IgA-anti-transglutaminase e anti-endomisial). A densitometria detectou osteopenia em duas mulheres e osteoporose em dois homens, todos com baixos níveis de vitamina D. O atraso no diagnóstico variou de 1 a 19 anos. A ocorrência familiar de DC foi relatada em 40% dos casos. Após 1 ano de dieta isenta em glúten, oito pacientes referem melhora dos sintomas, enquanto as biópsias duodenais, realizadas em cinco casos, mostraram melhora histológica. CONCLUSÃO: Pacientes que apresentam quadro clínico de doença celíaca com sorologia negativa e níveis normais de IgA, principalmente aqueles que possuem familiares com doença celíaca, devem ser submetidos à biópsia duodenal para pesquisa de achados histológicos.

Background: Mannose binding lectin (MBL) appears to be involved in susceptibility to rheumatoid arthritis (RA), in the inflammatory process and in the genesis of atherosclerotic disease. Objective: To study the association of MBL serum levels and its genotypic variation with carotid arteries intimal thickness (IMT) in RA patients from Southern Brazil. Methods: MBL serum levels, MBL2 genotyping and IMT were investigated in 90 RA patients along with their demographic, clinical and laboratory profile. MBL levels and MBL2 genotyping were evaluated in 90 healthy controls. Results: A significant lower MBL serum concentration was observed in patients with RA in relation to controls (528 ng/mL vs 937.5 ng/mL, p = 0.05, respectively). The median IMT in RA patients was 0.59 mm (0.51 to 0.85 mm). There was no correlation between levels of MBL with disease activity, erythrocyte sedimentation rate, autoantibodies presence or IMT (p = NS). A weak and negative correlation was found between MBL and CRP levels (Rho = −0.24; p = 0.02;). The MBL2 variant at codon 54 (variant B) and HYPA haplotype were the most frequently observed in the RA sample (67.5% and 31.7%). MBL2 wild type (A/A) were associated with lower IMT when compared with heterozygotes (A/O; p = 0.04) and low producers (O/O; p = 0.05). In addition, high producers genotypes had lower levels of CRP when compared with medium (p = 0.04) or with low producers (p = 0.05). Conclusion: RA patients had lower MBL levels than controls. MBL were negatively associated with CRP serum levels; low MBL genotypes producers increased thickness of the IMT than high producers. Keywords: Mannose binding lectin, Rheumatoid arthritis, Atherosclerosis

Background: Patients with autoimmune thyroid diseases (ATD) such as Graves’ disease (GD) and Hashimoto thyroiditis (HT) may have non-organ specific autoantibodies such as antinuclear antibodies (ANA) and rheumatoid factor (RF). Aim: To study the prevalence of rheumatic autoantibodies in a group of ATD patients without known rheumatic diseases and to evaluate its association with the patients’ epidemiological and treatment profiles. To follow positive non-organ specific autoantibody-positive ATD individuals to investigate whether they will develop a rheumatic disorder. Methods: A sample of 154 ATD patients (70 HT and 84 GD; mean age 45.3 ± 14.2) had determination of ANA by immunofluorescence, using hep-2 cells as substrate, extractable nuclear antigen profile by ELISA kits and RF by latex agglutination. Epidemiological and treatment profiles were obtained through chart review. These patients were followed for the mean period of 5 years, between 2010 and 2015. Results: Positive ANA was found in 17.5% (27/154) of the patients: anti-Ro/SS-A in 4/154 (2.5%); anti-RNP in 4/154 (2.5%), and anti-La/SS-B in 3/154 (1.9%). None had anti-Sm antibodies. RF was detected in 12/154 (7.7%) of ATD patients and was more common in older individuals (p = 0.007). There was a positive association between the presence of RF and ANA (p = 0.03; OR 3.89; 95% CI 1.1–13.3). None of the patients with positive autoantibodies developed clinical rheumatic diseases during the period of observation. Conclusion: We found rheumatic autoantibodies in 17.5% of ATD patients without rheumatic diseases. None of them were associated with the appearance of clinical rheumatic disorder during the period of 5 years.

Autoimmune thyroid disease (ATD) patients may have a higher prevalence of anti-parietal cell antibodies (APCA) than normal population. To study the prevalence of APCA in a cohort of ATD patients to know its association with patient’s clinical profile and gastrointestinal complaints. APCA was sought for by indirect immunofluorescence test in 243 ATD patients: 136 (55.9%) with Graves’ disease and 107 (44.0%) with Hashimoto’s thyroiditis. A structured questionnaire for gastrointestinal symptoms, previous history of thrombosis, arthralgia and other autoimmune diseases in the patients and their families was applied. Positive and negative APCA individuals were compared. Positive patients were invited to perform upper gastrointestinal endoscopy and biopsy of duodenum segments. Sera from 100 healthy individuals from the same geographic area were used as controls. APCA was present in 20.1% (49/243) of ATD patients: 21.3% (29/136) in the Graves’ sample and 18.6% (20/107) in the Hashimoto’s sample (p = 0.61). Patients with positive APCA had more anemia (p = 0.03; OR = 2.89; 95% CI = 1.03–8.07) and less heartburn (p = 0.01; OR = 0.4; 95% CI = 0.20–0.83). Among the group of 49 APCA-positive patients, 24 agreed with upper endoscopy and it was found that 54.1% had atrophic gastritis. There is a high prevalence of positive APCA in ATD patients. APCA are more common in those with anemia and less common in those with complaints of heartburn. Almost half of positive APCA patients had atrophic gastritis.

The complement system contributes to the pathogenesis of systemic lupus erythematosus (SLE). Mannose-binding lectin (MBL) is a key molecule of the lectin pathway of complement and seems to be related to the clinical manifestations of this disease. We evaluated the serum levels of MBL and its relationship with disease onset and clinical findings in SLE patients. Serum samples were analysed in 195 patients and 145 healthy controls from southern Brazil. Patients with high MBL levels (above 2000 ng/ml) showed a significant increase in the frequency of thrombocytopaenia ( p = 0.007; OR = 2.71; 95% CI = 1.32–5.55); and seizures ( p = 0.034; OR = 2.61; 95% CI = 1.07–6.37). A positive correlation between disease activity and MBL levels (>2000 ng/ml; p = 0.031, rho = 0.279) as well as of MBL concentration with accumulated organ damage ( p = 0.021; rho = 0.232) was observed. Our results suggest a role for MBL in the development of clinical manifestations such as thrombocytopaenia and seizures in SLE patients. These findings corroborate the participation of the lectin pathway of complement in the pathophysiologic mechanisms underlying clinical manifestations of SLE.

ResumoObjetivosAvaliar a frequência de quatro marcadores sorológicos em pacientes com AR e seus familiares e identificar possíveis associações com achados clínicos da doença.MétodosEstudo analítico transversal. Determinaram‐se os níveis de anticorpos antipeptídeo citrulinado cíclico (anti‐CCP), anticorpos antivimentina citrulinada‐mutada (anti‐MCV) e fator reumatoide (FR) IgA por Elisa e de FR‐IgM por aglutinação em látex em 210 pacientes com AR, 198 familiares e 92 controles saudáveis do sul do Brasil. Coletaram‐se dados clínicos e demográficos por meio da revisão de prontuários e questionários.ResultadosObservou‐se maior positividade para todos os anticorpos em pacientes com AR em comparação com os familiares e controles (p

Infections are usually involved in the pathogenesis of spondyloarthritis (SpA). Mannose-binding lectin (MBL) is a component of the innate immune system with an important role in microbial defense.To study the prevalence of MBL deficiency in SpA patients as well as its influence in the clinical profile of these diseases.We studied 89 SpA patients and 89 healthy individuals, paired for age and gender. MBL serum levels were measured by ELISA test. Individuals with levels ≤100 ng/mL were considered deficient. SpA patients had determination of Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Ankylosing Spondylitis Disease Activity Score (ASDAS)-CRP, Bath Ankylosing Spondylitis Functional Index (BASFI), C reactive protein (CRP), erythrocyte sedimentation rate (ESR), and review of their clinical profile.SpA patients had MBL levels ranging from 100 to 4100 ng/mL (median = 375 ng/mL); controls levels ranged from 100 to 4703 ng/mL (median = 1204 ng/mL; p0.0001). The prevalence of MBL deficiency was 27/89 (30.3%) in SpA patients and 12/89 (13.5%) in controls, with p = 0.01; OR = 2.5 (95% IC = 1.2-5.3). No association/correlation was found between MBL levels with BASDAI, BASFI, age at disease onset, ASDAS-CRP, ESR, CRP, presence of uveitis, HLAB27, peripheral arthritis, or SpA subtype (all p = NS).MBL levels may be linked with the occurrence of SpA but do not influence its phenotype.

Autoimmune liver diseases (ALDs) are known to be associated with systemic autoimmune rheumatic diseases (SARDs) and their autoantibodies. We aimed to study the prevalence of SARDs and related autoantibodies, as well as their prognostic implications in a group of patients with ALDs.This was a cross-sectional study. Sixty patients with ALDs (38.3% with autoimmune hepatitis; 11.7% with primary biliary cirrhosis; 25% with primary sclerosing cholangitis and 25% with overlap syndrome) were studied for the presence of SARDs and their autoantibodies.There was autoimmune rheumatic disease in 20% of the studied sample. Systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) were the commonest (11.6% and 5%, respectively). Antinuclear antibodies (ANAs) were present in 35% of the patients, followed by anti-Ro (20.0%); anti-nucleosome (18.3%); rheumatoid factor (10%) anti-CCP (8.3%); anti-RNP (8.3%); anti-ds-DNA (6.6%); anti-La (3.3%); anti-Sm (3.3%), anti-ribosomal P (3.3%). Anti-Ro (p = 0.0004), anti-La (p = 0.03), anti-RNP (p = 0.04) and anti-Sm (p = 0.03) were commonly found in patients with SARD, but not anti-DNA, anti-nucleosome and anti-ribosomal P. No differences were found in liver function tests regarding to the presence of autoantibodies.There was a high prevalence of SARD and their autoantibodies in ALD patients. Anti-Ro, anti-La, anti-RNP and anti-Sm positivity points to an association with systemic autoimmune rheumatic diseases. The presence of autoantibodies was not related to liver function tests.

Mean platelet volume (MPV) is a parameter that evaluates the platelet size. Clinical applications of MPV are limited because of its poor standardization in routine laboratories. This study analyzed the effect of anticoagulants on MPV measurements by impedance technology.Blood from 36 healthy volunteers was collected in vacuum tubes filled with K2EDTA and sodium citrate, analyzed immediately (basal) and at 1, 2, and 3 hours after venipuncture.Comparisons between the anticoagulants demonstrated a significant difference (p0.05) after 1 hour of exposure with K2EDTA, causing a time-dependent increase on MPV measured. No significant changes in MPV were observed with sodium citrate with 3 hours of exposure (p0.05).The use of sodium citrate is highly indicated for assessment of MPV when the measurement time after blood collection is estimated to be more than 1 hour.

Background B factor (BF) from the alternative complement pathway seems to participate in the pathophysiology of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Objective To study the allotypic variability of BF in SLE and their associations with clinical and autoantibodies profile. Methods BF allotypes were determined by high-voltage agarose gel electrophoresis, under constant cooling, followed by immunofixation with anti-human BF antibody, in 188 SLE patients and 103 controls. Clinical and serological data were obtained from medical examination and records. Results No significant differences of BF variants between patients and controls were found, neither in relation to epidemiologic or clinical manifestations. Associations of phenotype BF SS07 and allotype BF*S07 were found with anticardiolipin IgM (aCl-IgM) antibodies ( p = 0.014 and p = 0.009 respectively), but not with aCl-IgG, lupus anticoagulant (LA), anti β2GPI or clinical APS. A significant decrease in BF*F allotype ( p = 0.043) and BF SF phenotype ( p = 0.018) was detected in patients with anti-phospholipid antibodies as a whole (aCl-IgG, aCl-IgM, LA and anti β2GPI). Conclusions There is a link between phenotype BF SS07 and allotype BF*S07 with aCl-IgM in SLE patients; BF*F allotype could be considered a marker of protection against the development of antiphospholipid antibodies in these patients.

Summary The aim of the study was to investigate the allotypic variability of complement factor B (BF) in patients and relatives with rheumatoid arthritis (RA) and its association with serological biomarkers and clinical features of the disease. BF allotypes were determined by high-voltage agarose gel electrophoresis in serum samples of 180 patients with RA, 198 relatives and 98 controls from Southern Brazil. Anticyclic citrullinated peptide (anti-CCP), antimutated citrullinated vimentin (anti-MCV) and IgA-rheumatoid factor (RF) were determined by ELISA and IgM-RF by latex agglutination in all samples. No significant differences were found in the allotypic variants of BF between patients with RA, relatives and controls, nor associations with gender and age of RA onset. BF*S07 allotype was significantly associated with extra-articular manifestations (EAMs; Secondary Sjogren Syndrome, pneumonitis, rheumatoid nodules) in patients with RA (P = 0.02; OR = 6.62). Patients with phenotype BF F had lower positivity for anti-MCV biomarker (P = 0.02; OR = 0.22) and those with allotype BF*S had higher prevalence of this autoantibody (P = 0.02; OR = 3.77). An increased frequency of RF-IgA was detected in relatives of patients with RA with BF FS07 phenotype (P = 0.02; OR = 7.78). Complement BF variability did not influence the development of RA in the studied patients, but BF variants may act as markers of disease prognosis, such as development of EAMs, corroborating with the role of the alternative pathway in the pathogenesis of RA.

Background Leprosy patients may present several osteoarticular complaints, which require further evaluation of inflammatory diseases, such as rheumatoid arthritis (RA). Therefore, an adequate clinical assessment in addition to testing for rheumatoid factors (RF) and anticyclic citrullinated peptide antibodies (anti-CCP), can be useful in order to establish the correct diagnosis. Method In this study, the relation of RF and anti-CCP with rheumatological manifestations was evaluated in 97 leprosy patients from Southern Brazil. The results were compared to RA patients and healthy controls from the same geographical area and ethnic background. Results Neuropathy was observed in 71.1% and arthritis in 35.1% of the leprosy patients. A high frequency of RF positivity was observed among the leprosy patients (41.2%, 40/97), with RF immunoglobulin A (IgA) significantly associated with arthritis (OR = 7.9, 95% CI = 1.5–40.6 P = 0.008). Anti-CCP was observed in 9.3% (9/97) of the patients, with anti-CCP2 being the most frequent subtype. Only 4.1% (4/97) of the patients were RF and anti-CCP concomitantly positive. RF IgM showed a significant association with leprosy when compared to healthy controls (P

Background/Aims: Arthropathy is the most common extraintestinal manifestation observed in patients with Crohn's disease (CD). The present study aimed to screen rheumatoid arthritis (RA) using anti-CCP antibodies and rheumatoid factor (RF) in CD patients from Southern Brazil. Additionally, the presence of arthralgia and spondyloarthritis (SpA) was evaluated. CD patients, previously diagnosed using clinical data, imaging tests, endoscopic and histological findings, were included consecutively. Methods: A total of 100 patients participated in the study, of which 60% were female, with a mean age of 41.9 ± 12.04 (16-83 years). As controls, sera from 100 healthy individuals from the same geographic area were analyzed. Results: Arthralgias were present in 55% of the patients, being more frequent in women (65.5%; 36/55), than in males (34.5%). No association was found between arthralgia and the treatment method used. Six patients (6/100) had SpA previously diagnosed. In the CD group, anti-CCP was positive only in one patient, while RF was positive in 7 patients (7%; 7/100). The anti-CCP positive patient (woman, 38 years old, RF positive), fulfilled the ACR criteria and was diagnosed as RA. In the control group, anti-CCP antibodies were detected in 1% (1/100) and RF was positive in 6 of the samples (6%). Conclusion: Our data showed low frequency of anti-CCP antibodies and RF in Brazilian patients with CD. Additionally, we found a high prevalence of arthralgia in these patients, with 6% of them diagnosed with SpA.

To investigate the association between mannose-binding lectin (MBL) serum level and MBL2 polymorphisms, and the frequency of spontaneous miscarriages in rheumatoid arthritis (RA) patients.One hundred seventy seven women (mean age 50 years) with RA from Southern Brazil were studied and 4.5% had a history of abortion (8/177). The MBL levels were determined by ELISA. MBL2 polymorphisms in the promoter (-550H/L, -221X/Y), 5' untranslated region (4 P/Q) and exon 1 (p.Gly54Asp: B allele, p.Arg52Cys: D allele and p.Gly57Glu: C allele; collectively labelled O) were genotyped by sequencing.Mannose-binding lectin levels of RA patients ranged from ≤100 ng/mL to 6640 ng/mL (median 541.5 ng/mL). There was a significant difference in MBL median levels (100 ng/mL vs. 625 ng/mL, respectively, p = .001) and frequency of MBL deficiency (75.0% vs. 24.1%, p = .007, OR = 10.3, 95%CI = 1.9-55.4), in patients with a history of miscarriage vs those without it. Patients with RA and miscarriage had more frequently haplotypes related with low MBL levels (p = .007, OR = 10.5, 95%CI = 1.3-84) than high producers. Moreover, LYPB haplotype and O allele were significantly associated with the occurrence of miscarriage (p = .001, OR = 9.7, 95%CI = 2.4-39.1 and p = .009, OR = 5.9, 95%CI = 1.4-23.4, respectively).The results suggest that MBL deficiency and the presence of MBL2 gene polymorphisms that lead to MBL deficiency are risk factors for the occurrence of miscarriage in patients with RA.

Background: Anemia during childhood is one of the biggest public health problems worldwide, including Brazil. Insufficient or abnormal production of hemoglobin, loss of iron and excessive destruction of red blood cells are the most common causes of anemia. Among the reasons of anemia, iron deficiency accounts for 50% of anemia cases in developing countries. Affected individuals present a wide range of clinical problems, including delayed neuropsychomotor progression, impaired cellular immunity and reduction of intellectual capacity. This study aimed to evaluate the prevalence of anemia in children attending public schools in the metropolitan region of Curitiba, Paraná, Brazil. Method: A retrospective study was conducted of 409 children aged 8–12 years old included in an extension project of the Universidade Federal do Paraná. The results of complete blood count and hemoglobin electrophoresis of all children were evaluated. Anemia was considered when the hemoglobin levels were

Background The binding lectin mannose (MBL) is a serum protein of collectin family that appears to be involved in the inflammatory process and in the genesis of atherosclerotic disease. Objectives To study the association of serum levels of MBL and its genotypic variation with carotid arteries intimal thickness (IMT) in rheumatoid arthritis (RA) patients from Southern Brazil. Methods Serum level, MBL genotyping and IMT were studied in 90 RA patients along with their demographic, clinical and laboratory profile. MBL levels were measured in 90 healthy controls. Results There was significant difference between mean serum levels of MBL in patients with RA and controls (528 ng/mL vs 937.5 ng/mL, p=0.05, respectively). The median IMT in RA patients was 0.59 mm (0.51 to 0.85 mm). There was no correlation between levels of MBL with disease activity measured by DAS-28 (disease activity score-28), erythrocyte sedimentation rate (ESR), autoantibodies presence or IMT (p=NS). A negative correlation was found between MBL levels with CRP levels (p=0.02). The mutation vat codon 54 (variant B) and HYPA haplotype were the most frequent (67.5% and 31.7%, respectively) in the RA sample. Dominant genotypes (A/A) are associated with lower IMT when compared with heterozygotes (A/O; p=0.04) and homozygous recessive (O/O; p=0.05). Also dominant genotypes had lower CRP when compared with heterozygous (p=0.04) or with recessive genotypes (p=0.05). Conclusions RA patients had lower MBL levels than controls. MBL serum levels are negatively associated with CRP; low producers of MBL had increased thickness of the IMT than high producers. References Scarno A, Perrotta FM, Cardini F, Carboni A, Annibali G, Lubrano E, et al. Beyond the joint: Subclinical atherosclerosis in rheumatoid arthritis. World J Orthop [Internet]. 2014;5(3):328–35. Available from: http://www.wjgnet.com/2218-5836/full/v5/. Ip WK, Lau YL, Chan SY, Mok CC, Chan D, Tong KK, et al. Mannose-binding lectin and rheumatoid arthritis in southern Chinese. Arthritis Rheum. 2000;43(8):1679–87. J Maury CP, Aittoniemi J, Tiitinen S, Laiho K, Kaarela K, Hurme M, et al. Variant mannose-binding lectin 2 genotype is a risk factor for reactive systemic amyloidosis in rheumatoid arthritis. Jounal Intern Med. 2007;262:466–9. Graudal NA, Homann C, Madsen HO, Svejgaard A, Jurik AG, Graudal HK, et al. Mannan Binding Lectin in Rheumatoid Arthritis. A Longitudinal Study. J Rheumatol. 1998;25(4):629–35. Carvalho EG, Utiyama SRR, Kotze LMS, Reason ITM. Lectina ligante de manose (MBL): caracteristicas biolόgicas e associacao com doencas. Res bras alerg imunopatol. 2007;30(5):187–93. Disclosure of Interest None declared

Objective: The objective of this study was to determine the prevalence of celiac disease (CD) in adults with autoimmune thyroid disease (ATD) from the endocrinology outpatient setting in a university hospital in Southern Brazil. Subjects and methods: From the years 2007 to 2011, 254 patients with ATD were enrolled consecutively, Grave’s disease was diagnosed in 143 (56.3%) and Hashimoto’s thyroiditis in 111 (43.7%) of them. All patients answered a questionnaire related to symptoms that could be associated with CD and serum samples to screen for IgA anti-endomysial (EmA-IgA) were collected. EmA-IgA-positive patients were offered upper gastrointestinal endoscopy and biopsy of duodenum. Results: A total of 254 patients were included; 222 (87.4%) female, mean age 45.4 ± 13.43 years (18 to 79 years). EmA-IgA was positive in seven patients (2.7%) and five done endoscopy with biopsy. Of these, three diagnosis of CD was confirmed (1.2%). All the three patients with CD had higher EmA-IgA titration, were female and had Hashimoto’s thyroiditis. Like other patients with ATD, CD patients had nonspecific gastrointestinal symptoms, such as heartburn and gastric distention. In our study, one in each 85 patients confirmed the diagnosis of CD. Conclusion: We found a prevalence of 1.2% (1:85) of confirmed CD among Brazilian patients with ATD. Although some IgA-EmA positive patients had Graves’ disease and one was male, all three patients with confirmed CD were female and had Hashimoto’s thyroiditis. Arq Bras Endocrinol Metab. 2014;58(6):625-9

Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease driven by genetic, hormonal, and environmental factors. Despite the advances in diagnostic and therapeutic approaches in the last decades, SLE still leads to significant morbidity and increased mortality. Although a cure for SLE is still unknown, treatment is required to control acute disease exacerbation episodes (flares), decrease the frequency and severity of subsequent lupus flares, address comorbidities, and prevent end-organ damage. While conventional SLE pharmacotherapy may exhibit suboptimal efficacy and substantial toxicity, a growing knowledge of the disease pathogenesis enabled the research on novel therapeutic agents directed at specific disease-related targets. In this paper, we review the recent progress in the clinical investigation of biologic agents targeting B cells, T cells, cytokines, innate immunity, and other immunologic or inflammatory pathways. Although many investigational agents exhibited insufficient efficacy or inadequate safety in clinical trials, one of them, belimumab, fulfilled the efficacy and safety regulatory requirements and was approved for the treatment of SLE in Europe and the USA, which confirms that, despite all difficulties, advances in this field are possible.

Patients with juvenile idiopathic arthritis (JIA) may need further care in the adult clinic as this disease frequently has continuous inflammatory activity during adult life. To identify which pediatric JIA patients will need continuing care into adulthood. We compared the clinical, serological, and demographic data of 45 JIA patients followed up by the pediatric clinic to those of 49 JIA patients in the adult rheumatology clinic. Patients in the adult clinic have older age at disease onset (p

Background/Aims: In this study, a clinical and serological follow-up of 8–10 years was performed in relatives of celiac disease (CD) patients from southern Brazil. The occurrence of new CD cases in the families and the use of two different IgA-tTG enzyme-linked immunosorbent assay (ELISA) kits were also evaluated. Methods: Serum samples of 233 relatives, 186 recruited between 1997 and 2000 (phase I) and 138 between 2006 and 2007 (phase II: 91 of the follow-up group and 47 newly tested), were analyzed. As a comparison group, 100 unrelated healthy individuals were evaluated. IgA-EmA was tested by indirect immunofluorescence and IgA-tTG by ELISA. Results: A significant increase in IgA-EmA/IgA-tTG was detected in relatives of patients with CD when compared to controls (p ≤ 0.001). The positivity of antibodies was higher in females (2.4:1 in phase II; p = 0.039), and its high frequency amongst siblings (∼18.81%) highlights the risk of CD in these individuals. The distribution of antibodies by age suggested that CD can occur at any age in relatives, calling attention to the newly tested relatives >60 years of age (p = 0.0657). A better performance of ELISA kits with human tTG was observed. The confirmation of 13 biopsy-proven new CD cases (5.6%; 13/233) at present points out the predisposition to CD in these individuals and the high specificity of concurrently positive antibodies in relatives, especially when both are present in high titers. Conclusion: These results emphasize the familial risk to develop CD and the value of serological screening as an instrument for identifying this disease.

Association between autoimmune liver diseases and scleroderma has been described. The purpose of this study was to study the prevalence of antimithocondrial antibody (AMA), antismooth muscle antibodies (SMA), and liver-kidney-microsomal (LKM-1) autoantibody in a cohort of 63 scleroderma patients and 100 healthy controls. The autoantibodies AMA, SMA, and LKM were determined by indirect immunofluorescence. Patients' charts were reviewed for demographic data, scleroderma form, and clinical and anti-nuclear antibody profile, aiming a comparison between patients with and without liver autoantibodies. Nine patients (14.3%) were positive for at least one of the liver autoantibodies; only one patient had both AMA and SMA positive. Antibody SMA was positive in 6.4% (4/63) patients; AMA was present in 9.52% (6/63) of them; none were positive to LKM-1. In the control group just one patient (1%) was SMA positive; the other autoantibodies were negative. There is an increased prevalence of liver autoantibodies in patients with scleroderma than in control population. These patients should be carefully followed for liver dysfunction.

Clustering of autoimmune diseases is common and may be due to genetic background and exposition to environmental triggers.The aim is to carry out a laboratory and clinical study of the prevalence of gastrointestinal organ-specific autoantibodies in rheumatoid arthritis (RA) patients and their relatives.Serum samples of 156 RA patients, 200 relatives, and 100 healthy controls were studied for anti-smooth muscle antibody (ASMA), anti-mitochondrial (AMA), anti-parietal cell (APCA), anti-liver-kidney microsome (LKM), and anti-endomysium antibodies (IgA-EmA) by indirect immunofluorescence.A total of eight out of the 156 (5.1%) RA patients were positive for the autoantibodies (ASMA = 1; AMA = 2, APCA = 5). In the relative group, 12/200 (6%) had at least one positive autoantibody (ASMA = 1; AMA = 2, APCA = 7, IgA-EmA = 2). In the control group, two out of the 100 (2%) healthy controls were positive (ASMA = 1, APCA = 1). No statistical difference was found between RA patients, their relatives, and controls in relation to the frequency of autoantibodies evaluated.Although RA patients and their relatives have positivity of AMA, ASMA, and APCA without statistical difference in relation to healthy individuals, the findings may be of value for adequate clinical approach of these subjects.

Objectives. To evaluate the prevalence of anti-cyclic citrullinated peptide (anti-CCP) antibodies and RF in RA patients and their relatives from Southern Brazil. Methods. Anti-CCP2 and IgM-RF were evaluated in 156 RA patients and 200 relatives. Sera from 100 healthy unrelated individuals were used as control. The anti-CCP2 was detected by ELISA and the IgM-RF using the latex agglutination test. Results. We identified 117 anti-CCP2 (75%)-positive and 106 RF (67.9%)-positive patients. Anti-CCP2 was increased in relatives (5.5%; 11/200) when compared with unrelated individuals (1%; P = 0.050). Titre of anti-CCP2 in RA patients did not differ from relatives [140.4 (75.7) vs 115.6 (84.2) U, respectively; P = 0.30]. Positive relatives were younger than patients for anti-CCP2 (P = 0.0081), RF (P < 0.001) and both concomitantly (P = 0.012), and although there was no difference for anti-CCP2 positivity according to gender, increased RF positivity and concomitant anti-CCP2/RF were observed in the female relatives (P = 0.067 and 0.082, respectively). No difference regarding the relative degree of tobacco use in relatives was detected. Among the 11 anti-CCP2-positive relatives, 2 females had RA diagnosis established and 6 individuals presented with joint symptoms suggestive of RA. Conclusion. The results demonstrate a significant positivity of anti-CCP2 in relatives of RA patients from Brazil and reinforce the importance of serological tools to identify undiagnosed RA.

The aim of this study was to evaluate the prevalence of autoantibodies in silica-exposed patients with and without silicosis and without any known rheumatic disease. We studied 61 males exposed to silica for a mean time of 12.2 +/- 10.2 years of exposure. A total of 72.1% (44/61) of them presented with pulmonary silicosis. As control group we included 62 healthy males. In all samples we screened for rheumatoid factor (latex agglutination), antinuclear antibodies (indirect immunofluorescence), anti Scl-70 (ELISA) and ANCA (indirect immunofluorescence technique). One patient (1.6%) of the silica group had weakly positive ANA (titer 1:80, centromeric pattern); one (1.6%) had atypical ANCA and seven patients (11.4%) presented positive rheumatoid factor (values range from 8 to 32 UI/ml). One control patient had a positive RF and none of them had positive ANA or ANCA. All patients and controls were negative for anti-Scl-70. The finding of positive RF was higher in the silica-exposed patients (p = 0.032; Fisher). All patients with positive RF had pulmonary silicosis. In the silica-exposed group we could not find a relationship between the presence of RF and age (p = 0.21; Mann-Whitney), smoking habits (p = 0.25; Fisher) but a positive relationship was found with exposure time to silica dust (p = 0.005; Mann-Whitney). We conclude that there was 11.4% prevalence of low titer RF in the silica-exposed patients without known rheumatic disease. RF was more common in patients with longer exposure to silica dust and appeared only in those with silicosis. The presence of ANA, Scl-70 and ANCA was the same as in the control population.

Low concentration of mannan-binding lectin (MBL) has been related to unexplained spontaneous abortion (SA), which has also been observed in an increased frequency in patients with celiac disease (CD). In this study, plasma levels of MBL were determined in patients with CD and irritable bowel syndrome (IBS) in order to investigate whether there is an association of MBL levels and the occurrence of SA in these patients. MBL concentration was determined in 46 patients with CD (28 without and 18 with report of SA) and 38 patients with IBS (25 without and 13 with report of SA). A higher frequency of SA was observed in women with CD when compared to IBS patients (23.2 vs. 13.9%; P = 0.046). No significant difference was observed in MBL concentrations between patients with CD, IBS, and healthy controls, nor between patients with or without occurrence of SA. These results suggest that the serum levels of MBL and the occurrence of SA in women with CD and IBS are not causally related.

Aims: Down syndrome (DS) patients present several immunological disturbances, with high rates of infections, malignancies and autoimmune phenomena. The present study aims to evaluate the prevalence of autoantibodies in children and adolescents with DS that are not usually investigated, and to establish possible clinical and laboratory associations. Methods: One hundred and fifty Caucasoid DS patients from southern Brazil (93M, 57F; median age 4 years) and 105 healthy children (58M, 47F; median age 8 years) were evaluated for the presence of anti-mitochondrial (AMA), smooth-muscle (SMA), liver-kidney microsomal (LKM), nuclear (ANA), gastric parietal cell (GPC) and neutrophil cytoplasmic (ANCA) antibodies, by indirect immunofluorescence, and rheumatoid factor (RF), by turbidimetry. Results: Forty-three DS patients (28.6%) showed positivity to at least one autoantibody, in comparison with eight of the controls (7.6%; P

OBJETIVO: Determinar as concentracoes de hormonio estimulante da tireoide (TSH) e a presenca de anticorpos antitireoperoxidase (anti-TPO) em pacientes com sindrome de Down (SD) atendidos no ambulatorio do Hospital de Clinicas da Universidade Federal do Parana. METODOS: Foram incluidos no estudo 72 pacientes com SD, nao aparentados e selecionados consecutivamente, com media de idade de 6,15 anos. Oitenta criancas sadias, pareadas com os pacientes, foram utilizadas como controles. Em todas as amostras foram determinadas as concentracoes sericas de TSH e de anti-TPO, atraves do metodo de dosagem imunometrica. RESULTADOS: Trinta pacientes com SD (42,9%) apresentaram alteracoes nas concentracoes de TSH, sendo que 4,3% tinham valores menores que 0,5µUI/ml e 38,6%, valores superiores a 5µUI/ml (5,1 a 22) (media de 5,56 ± 4,18µUI/ml). Nos controles, a concentracao media de TSH foi 2,76µUI/ml (± 1,14), evidenciando-se um aumento significativo nos niveis de TSH nos pacientes com SD (p < 0,001). De forma similar, caracterizou-se uma diferenca significativa na positividade para o anti-TPO nos pacientes (15,4%) em relacao aos controles (0%; p < 0,001). Observou-se ainda aumento significativo nas concentracoes de TSH nos pacientes com idade superior a 9 anos (media de 6,86 ± 4,6µUI/ml) quando comparados aos menores de 9 anos (media de 5,24 ± 3,81µUI/ml; p = 0,006), bem como na positividade do anti-TPO (6/20 vs. 5/52; p = 0,041). CONCLUSOES: Os resultados demonstraram alta prevalencia de alteracoes das dosagens de TSH e de doenca tireoidiana nos pacientes com SD, principalmente naqueles com idade superior a 9 anos. Os dados indicam que a avaliacao da funcao tireoidiana nos pacientes com SD deve receber atencao especial dos profissionais de saude que atendem esses pacientes.

Introduction/Aim: Patients with celiac disease (CD) can develop a gluten related autoimmune disorder that affects not only the small intestine but other tissues as well. An increased prevalence of autoimmune diseases has been reported, particularly autoimmune thyroiditis. The aim of this study was to characterize thyroid disorders in patients with CD. Patients/Methods: Fifty-two patients with CD (43 female, 9 male; mean age, 41.1 years) were studied. Nine were on a gluten-free diet (GFD). They were divided into four groups: Group 1, without thyroid involvement (n = 30); Groups 2A-C, with thyroid involvement (n = 22); Group 2A, subclinical hypothyroidism (n = 11); Group 2B, clinical hypothyroidism (n = 10); and Group 2C, other thyroid disorders (n = 1). CD was confirmed by serologic and histologic criteria. Thyroid involvement was detected by measurement of thyroid stimulating hormone (TSH) and anti-thyroperoxidase antibodies (anti-TPO). Results: Increased levels of TSH and/or anti-TPO levels were detected in Groups 2A (21.1%) and 2B (19.2%). The patients of Group 2B presented clinical symptoms of hypothyroidism before the diagnosis of CD, and 5 of these patients were receiving levothyroxine. One woman (Group 2C; 1.92%) had a medullary carcinoma. There was statistical significance between the age when thyroid disease was diagnosed (current age) and the age of CD diagnosis between Groups 1 and 2B. Patients with thyroid involvement presented associated diseases such as diabetes mellitus (2), Down's syndrome (2), ulcerative colitis (1), and dermatitis herpetiformis (2). Conclusions: Our findings demonstrated an increased prevalence of thyroid disorders (hypothyroidism, 19.2%; and subclinical hypothyroidism, 21.2%), and other associated diseases in celiac patients, even on a GFD, increasing with the age of the patients. Screening for associated diseases is recommended for patients with CD, independent of age at diagnosis or treatment duration.

Background It has been reported that vaccination against hepatitis B is less effective among people with Down syndrome than in the general population. We aimed to evaluate the rate of seroconversion to hepatitis B vaccine in children with Down syndrome from Brazil. Methods A total of 120 people with Down syndrome were included. All of them received the vaccine at intervals of 0, 30 and 180 days and serum samples were tested for the presence of antibodies to the hepatitis B surface antigen (anti-HBs) 30 days after the last dose. Results In the studied group, 58.3% (70/120) were male and 41.7% (50/120) female, with the median age of 5 years (range 2–15 years). Fifty-eight of 120 (48.3%) developed anti-HBs after vaccination. No association was found between gender and/or age and vaccine response. Conclusions The low rate of seroconversion in response to hepatitis B vaccine suggests that all patients with Down syndrome immunized against hepatitis B should be followed and monitored by clinicians.

O sistema complemento e parte fundamental da imunidade inata e contribui na remocao de complexos imunes e na ativacao de processos inflamatorios. Essas proteinas representam um meio rapido e eficiente de protecao do hospedeiro contra microorganismos invasores. Associacoes entre complemento e doencas sao observadas em situacoes de deficiencia do complemento, anormalidades na regulacao do complemento e nas inflamacoes. Ativacao impropria ou excessiva do complemento pode levar a consequencias lesivas em virtude da grave destruicao tecidual inflamatoria. Evidencias clinicas e experimentais ressaltam o papel do complemento na patogenese de inumeras doencas inflamatorias, que incluem nao apenas doencas por imune-complexos e auto-imunes, como tambem falencia de orgaos subsequentes a sepse, traumas multiplos e queimaduras. O polimorfismo genetico tem sido descrito para diversos componentes de membrana, proteinas soluveis de controle e receptores do sistema complemento. Atualmente, o polimorfismo dos componentes do complemento pode ser estudado usualmente, tanto pela pesquisa fenotipica das variantes proteicas (fenotipagem) como pela caracterizacao genomica do DNA (genotipagem), e tem contribuido para o entendimento da patogenese de diferentes enfermidades. Doencas imunologicamente mediadas estao associadas as variantes alotipicas do complemento, em particular lupus eritematoso sistemico, artrite reumatoide, esclerose multipla, doenca celiaca, deficiencia de IgA e IgG4, entre outras. A presente revisao tem por objetivo dar uma visao dos conhecimentos atuais na area da genetica do complemento, bem como da participacao desse sistema na patogenia de diferentes doencas.

BACKGROUND: Celiac disease, or gluten-sensitive enteropathy, is a strongly inherited condition. Although the genetic association of CD with the DQ2 and DQ8 HLA haplotypes has been known for long, others HLA and non-HLA genes are also important in the development of the disease. Celiac disease results of the combined effect of different normally functioning genes' products. The tissue damage in celiac disease is immunologically mediated and several effector mechanisms are responsible for the disease expression. The interplay between genetic, immunological and environmental factors explains the large spectrum of clinical, histological and serological alterations observed in the different stages of the disease development, pointing out to the polygenic nature of celiac disease. CONCLUSION: The recent advances in the understanding of the immunopathogenesis, genetics and diagnoses of celiac disease have allowed the revision of strict concepts and previous criteria and their adequation to the new evidences, aiming a better diagnostic and orientation to celiac patients and relatives.

A esclerose sistêmica (ES) é uma doença inflamatória crônica do tecido conjuntivo, de etiologia desconhecida, caracterizada por fibrose e dano micro vascular dos órgãos afetados. Há várias evidências de que a ativação do sistema imune celular participa de maneira expressiva na sua patogênese. OBJETIVO: Analisar numericamente as subpopulações linfocitárias no sangue periférico de pacientes com ES e sua possível relação com fatores clínicos e laboratoriais. MÉTODOS: Foram analisadas as subpopulações linfocitárias de 42 pacientes com ES e 28 controles normais, através da citometria de fluxo utilizando os antígenos linfocitários: CD2, CD3, CD4, CD8, CD19, CD25, CD45RA, CD56, CD71, HLA-DR , TCRα/β e TCRγ/δ. RESULTADOS: Nos pacientes com ES comparados com o grupo-controle, foram encontrados valores porcentuais normais para os linfócitos T CD2+, CD3+, CD3+CD4+, CD3+CD8+, CD25+, CD4+CD45RA+, CD8+CD45RA+, CD71 + e para a relação CD4+/CD8+. Em contraste, os linfócitos T CD3+TCRγ/δ mostraram-se com porcentuais diminuídos nos pacientes com ES difusa, de longa duração, com envolvimento pulmonar caracterizado por doença pulmonar restritiva, envolvimento muscular e presença de antilcorpo anti-Scl-70. Os pacientes com a forma difusa de ES apresentaram, tanto na fase recente como na tardia, aumento na porcentagem de linfócitos T CD4+ e CD8+ expressando moléculas de ativação HLA-DR. Na fase tardia da doença, não importando a forma clínica, foi encontrado aumento na porcentagem de linfócitos T CD4+CD45RA+. Os pacientes com ES limitada e fase recente mostraram diminuição na porcentagem dos linfócitos B CD19+. Os pacientes com doença difusa e fase tardia apresentaram diminuição na porcentagem dos linfócitos NK CD56+. CONCLUSÕES: Estes resultados sugerem que as alterações dos linfócitos T, B e NK possam estar envolvidas no processo de desencadeamento e/ ou perpetuação da ES, havendo a possibilidade de ter utilidade prognóstica em alguns grupos de pacientes. Systemic sclerosis (SSc) is a chronic inflammatory connective tissue disease of unknown etioiogy characterized by fibrosis and microvascuiar injury in affected organs. It has become clear that th e activation of celluiar immune system piays a central roie in the pathogenesis of SSc. OBJECTIVE: The purpose oE this study was to anaiyze numerically iymphocytic subpopuiations in the biood of systemic sclerosis patients and their relations with clinicai and iaboratory mani[estations. METHODS: We studied a group of 42 patients with SSc and a group oE 28 matched normai controis by flow cytometry using the following iymphocyte celi-surrace markers: CD2, CD3, CD4, CDS, CD19, CD25, CD45RA, CD56, CD71, HLADR, TCRα/β and TCRγ/δ. RESULTS: SSc patients had similar percentages of CD2+, CD3+, CD3+CD4+, CD3+CDS+, CD25+, CD4+CD45RA+, CDS+CD45RA+, CD71+ cells and CD4+/ CDS+ celi ratio when compared to . normal controis. ln contrast, the percentages of TCRγ/δ cells were significantiy iower in SSc patients with diffuse and iate-stage disease with puimonary and muscle invoivement and the presence of anti-Scl-70 antibodies. Patients with diffuse SSc in early and iate-stage disease had significantly increased percentages of HLA-DR in CD4+ and CDS+ celis; patients with iate-stage disease had increased percentages of CD4+CD45RA+ T celis; patients with iimited and eariy-stage disease had small pereentages of B eelis (CD19+): and patients with diffuse and iate-stage disease had small pereentages of NK eells (CD56 +). CONCLUSIONS: These results suggest that T, B and NK eell alterations may be invoived in the onset of the disease and/ or in the perpetuation of disease, and may eventuaJJy be usefui as a prognostie indieator in seleeted patient subgroups.

BACKGROUND Antibodies to cyclic citrullinated peptide (anti-CCP) have been found in different proportions in the juvenile idiopathic arthritis (JIA) population. The majority of studies have been done in children or mixed population (children plus adults). AIM The objective of the study was to study the prevalence of anti-CCP in JIA adult patients. METHODS Anti-CCP3 was searched for in 49 adult patients with JIA and associated with clinical and demographics data. As comparisons, 156 patients with adult rheumatoid arthritis (RA) and 100 healthy volunteers were studied. RESULTS Nine patients (18.3%) were positive for anti-CCP3. All of them had the polyarthritis form. This antibody was more common in JIA than in control subjects (P = 0.0002) and less common in JIA than in adult RA patients (P < 0.0001), but the rheumatoid factor polyarticular form of JIA had the same prevalence as in adult RA patients (P = 0.33).In JIA patients, anti-CCP had a positive association with the presence of rheumatoid factor (P < 0.0001), worse functional status (P = 0.04), need for orthopedic surgery (P = 0.01), and later disease onset (P = 0.0007). CONCLUSIONS In adult patients with JIA, the prevalence of anti-CCP3 is 18%, and its presence may define a sample of patients with worse prognosis.

Objective : To analyze if it is worthwhile to screen Brazilian osteoporotic patients for celiac disease (CD).Subjects and methods : One hundred patients with osteoporosis and 97 controls were evaluated for IgA-EmA (IgA anti-endomysial antibodies) by indirect immunofluorescence method and IgG-anti-tTG (tissue transglutaminase) by ELISA assay. Positive patients were invited to have gastrointestinal endoscopy with jejunal biopsy.Results : Two patients had positive IgG-anti-tTG test and one of them also showed positive IgA-EmA. Only the latter had a positive duodenal biopsy for CD. None of the controls were positive for either auto-antibodies.Conclusion : We observed low prevalence of CD in osteoporotic Brazilian patients. This finding does not support routine screening for CD in patients with osteoporosis in our geographic region. Arq Bras Endocrinol Metab. 2014;58(3):270-3 Objetivo : Analisar a necessidade de investigar doença celíaca (DC) em pacientes brasileiros com osteoporose. Sujeitos e métodos : No total, cem pacientes com osteoporose e 97 controles foram pesquisados IgA-EmA (IgA antiendomísio) pelo método de imunofluorescência indireta e IgG-anti-tTG (transglutaminase tecidual) pelo teste de ELISA. Os pacientes positivos foram convidados a realizar endoscopia gastrointestinal com biópsia jejunal. Resultados : Duas pacientes foram positivas para o teste IgG-anti-tTG. Destas, somente uma também foi positiva para IgA-EmA. Esta última possuía achados de biópsia jejunal sugestivos de DC. Nenhum dos controles foi positivo para nenhum dos anticorpos. Conclusão : Foi observada baixa prevalência de DC nos pacientes brasileiros com osteoporose. Esse achado não oferece suporte para pesquisa rotineira de DC em pacientes com osteoporose em nossa região geográfica. Arq Bras Endocrinol Metab. 2014;58(3):270-3

The coexistence of celiac disease together with a range of autoimmune disorders has already been reported. The aims of this study were to perform a broad spectrum of autoantibodies in celiac patients (N = 56), their first-degree relatives (N = 118), and compare the data with healthy controls (N = 101) and patients with inflammatory bowel disease (N = 42; Crohn's disease, N = 18 and ulcerative colitis, N = 24). All serum samples were tested by indirect immunofluorescence to the anti-endomysium antibodies (EmA), anti-neutrophil cytoplasmic (ANCA), anti-smooth-muscle (SMA), anti-mitochondrial (AMA), anti-nuclear (ANA), anti-liver-kidney microsomal (LKM), anti-gastric parietal cells (GPCA), and anti-thyroid microsome (TMA). EmA were detected in 100% of celiac patients ingesting gluten and in 16.1% of the first-degree relatives, while ANCA were positive only in patients with ulcerative colitis (45.6%) and Crohn's disease (16.5%). Fourteen CD patients (25%) were positive for at least one of the other autoantibodies, with significant prevalence of TMA, ANA, and GPCA, while the relatives showed 17.8% of positivity, with an increased prevalence of ANA and TMA. These results emphasize the value of screening for different autoantibodies in celiac patients and their relatives and corroborate the need for evaluation and follow-up of these individuals.