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Background: Fibromyalgia women (FM) seems to get worse at menopause suggesting some influence of estrogens on its pathophysiology. We aimed to study the influence of postmenopausal hormone therapy (HT) in FM, the relationship with sleep and FM impact., Methods: We analyzed prospectively 69 menopausal women, divided in two groups, FM group (FMG; N.=32) and comparison group (CG; N.=28) submitted to HT for twelve weeks (1.2 mg/g transdermal estradiol, 100 mg micronized natural progesterone oral/daily). Data on Utian Quality of Life Questionnaire (UQOL) and Pittsburgh Sleep Quality Index (PSQI) were obtained in both groups, at entrance and twelve weeks after HT. FM patients also completed the Fibromyalgia Impact Questionnaire - Revised (FIQ-R) and fibromyalgia severity (FS)., Results: FM patients improved significantly the FIQ-R (P=0.0001, median FIQ-R score 30% lower), mainly the severity of FM, assessed by FS (P<0.0001). Both groups had improved quality of life and sleep (UQOL: P=0.0001; P=0.001, PSQI: P<0.0001; P=0.007, respectively). Differences between first and second PSQI were greater for CG than for FMG (P=0.008)., Conclusions: HT improving sleep and quality of life in both groups; it was a significant clinical improvement seen by FIQ and FS in FM patients. These changes characterize improvement of functional status and symptoms severity.

Celiac disease (CD), despite its high morbidity, is an often-underdiagnosed autoimmune enteropathy. Using a modified version of the Brazilian questionnaire of the 2013 National Health Survey, we interviewed 604 Mennonites of Frisian/Flemish origin that have been isolated for 25 generations. A subgroup of 576 participants were screened for IgA autoantibodies in serum, and 391 participants were screened for HLA-DQ2.5/DQ8 subtypes. CD seroprevalence was 1:29 (3.48%, 95% CI = 2.16-5.27%) and biopsy-confirmed CD was 1:75 (1.32%, 95% CI = 0.57-2.59%), which is superior to the highest reported global prevalence (1:100). Half (10/21) of the patients did not suspect the disease. HLA-DQ2.5/DQ8 increased CD susceptibility (OR = 12.13 [95% CI = 1.56-94.20], p = 0.003). The HLA-DQ2.5 carrier frequency was higher in Mennonites than in Brazilians ( p = 7 × 10 -6 ). HLA-DQ8 but not HLA-DQ2.5 carrier frequency differed among settlements ( p = 0.007) and was higher than in Belgians, a Mennonite ancestral population ( p = 1.8 × 10 -6 ), and higher than in Euro-Brazilians ( p = 6.5 × 10 -6 ). The glutathione pathway, which prevents reactive oxygen species-causing bowel damage, was altered within the metabolic profiles of untreated CD patients. Those with lower serological positivity clustered with controls presenting close relatives with CD or rheumatoid arthritis. In conclusion, Mennonites have a high CD prevalence with a strong genetic component and altered glutathione metabolism that calls for urgent action to alleviate the burden of comorbidities due to late diagnosis.

Despite some variability in ideal serum Adalimumab (ADA) concentrations, there is increasing evidence that higher concentrations of anti-TNF-α agents can be associated with sustained efficacy, and low or undetectable levels may lead to loss of response. This study aims to correlate serum ADA concentrations with clinical and endoscopic activity in patients with Crohn's disease (CD). A cross-sectional and multicentric study was performed with patients with CD, who used ADA for at least 24 weeks. Patients were allocated into groups according to the presence of clinical or endoscopic disease activity. Serum ADA concentrations were measured and compared between groups. Overall, 89 patients were included. A total of 27 patients had clinically active CD and 62 were in clinical remission. Forty patients had endoscopic disease activity and 49 were in endoscopic remission. The mean serum ADA concentration was 10.2 μg/mL in patients with clinically active CD and 14.3 μg/mL in patients in clinical remission ( p = 0.395). The mean serum ADA concentration in patients with endoscopic activity was 11.3 μg/mL as compared to 14.5 μg/mL in those with endoscopic remission ( p = 0.566). There was no difference between serum ADA concentrations regarding clinical or endoscopic activity in CD, as compared to patients in remission.

Background: Social isolation, imposed by the COVID-19 pandemic, may imply changes in the clinical-demographic and epidemiological profiles of burn trauma victims., Objective: Evaluate the changes in the epidemiological profile of patients with burns that resulted in hospitalization during the social isolation period due to the COVID-19 pandemic, comparing with the same period in the previous year., Methods: The medical records of burn patients who were hospitalized in our Burn Center during the local confinement period (March 18th to August 31st, 2020) and during the same period in 2019 were analyzed. Data on demographic, clinical and hospitalization aspects were studied., Results: 470 patients were evaluated. In the pediatric population, a significant increase in the number of cases up to 2 years old (P = 0.0003), median of %TBSA (P = 0.037), full-thickness burns (P < 0.0001), involvement of hands (P = 0.024), debridement (P = 0.046) and grafting (P = 0.032) procedures, and higher scores of severity (P < 0.0001) were noted. In the adult population, it was only observed an increase in the burn-hospitalization interval (P = 0.029)., Conclusion: The pediatric population was heavily impacted by the imposed period of social isolation, presenting a greater severity of burns. In contrast, the epidemiology of burns for the adult population was slightly altered during the pandemic period., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 Elsevier Ltd and ISBI. All rights reserved.)

Background: Deep brain stimulation (DBS) is a well-established procedure for treating Parkinson's disease (PD). Although its mechanisms of action are still unclear, improvements in motor symptoms and reductions in medication side effects can be achieved for a significant proportion of patients, with consequent enhancement of quality of life., Objective: To investigate the impact of DBS on the quality of life of PD patients., Methods: This was a retrospective longitudinal study with collection of historical data in a neurosurgery center, from June 2019 to December 2020. The sample was obtained according to convenience, and the Parkinson's Disease Questionnaire (PDQ-39), Unified Parkinson's Disease Rating Scale (UPDRS) III and IV, Trail-Making Test and Verbal Fluency Test were used., Results: Data were collected from 17 patients (13 with subthalamic nucleus DBS and 4 with globus pallidus pars interna DBS). Significant improvement (p=0.008) on the UPDRS III was observed in comparing the preoperative without DBS with the postoperative with DBS. About 47.0% of the patients showed post-surgical improvement in QoL (p=0.29). Thirteen patients were able to complete part A of the Trail-Making Test and four of these also completed part B. Almost 60% of the patients scored sufficiently on the semantic test, whereas only 11.8% scored sufficiently on the orthographic evaluation. No association between implant site and test performance could be traced., Conclusions: Improvements in quality of life and motor function were observed in the majority of the patients enrolled. Despite the limitations of this study, DBS strongly benefits a significant proportion of PD patients when well indicated.

Introduction: Syphilis is a transfusion-transmitted infection and the disease re-emerged in many countries, including Brazil, as a public health risk., Objective: Evaluate the prevalence of positive serology in blood donations rejected by Hemobanco (Curitiba- PR) from January 2015 to December 2020, with special focus in syphilis., Methodology: In the studied period, we analyzed the number of blood donations discarded annually for each serological test performed on blood donors' samples, according to gender and donors age., Results: Within the studied period, 134,741 blood donation were analyzed. 54.5 % of sample were male. Otherwise, it was observed a significant increase in the number of donations by females (P < 0.0001). There was an increase of 437 % in the prevalence of syphilis positive serology made by donors with ≥ 60 years. Besides that, it was noticed a significant increase in donations by aged people throughout these six years (P < 0.0001). The percentage of total blood donation deferred had a significant reduction, although it was observed an increase in the rate of discarded blood bags due to positive serology in the first 4 studied years. It was observed an increase of 20 % in positive syphilis serology., Conclusion: The greatest cause of discard of blood donations changed during the analyzed 5-years; there was an increase in seropositivity donations from donors with ≥ 60 years old. In 2015, Hepatitis B (0.8 %) was the most prevalent and in 2020, syphilis became more prevalent (0.82 %). The medical community should be aware of the rising number of cases of syphilis infection. There is an urgent need to implement actions against the dissemination of this disease., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

The CR1 gene has been widely studied in Alzheimer's disease (AD), since its first association with the disease in 2009. Even after 11 years of this discovery, the role of this gene in AD has not yet been fully elucidated and the association of its variants was not validated in Latin American populations. We genotyped five CR1 single nucleotide polymorphisms (SNPs rs6656401, rs3849266, rs2274567, rs4844610, and rs12034383) in up to 162 AD patients and 137 controls through PCR-SSP and iPLEX MassARRAY Platform (Sequenom), and measured soluble CR1 (sCR1) levels in plasma of 40 AD patients and 39 controls with an enzyme-linked immunosorbent assay (ELISA). Homozygosity for haplotype rs3849266*C&#95;rs2274567*A (CA/CA genotype) was associated with susceptibility to AD (OR = 2.94, p = 0.018). Patients presented higher sCR1 levels in plasma than controls (p = 0.038). Furthermore, patients that carry the rs2274567*G allele (p.1208Arg) presented higher sCR1 levels than A/A (p.1208His/His) homozygotes (p = 0.036). This is the first study to validate the association of CR1 polymorphisms with late-onset Alzheimer's disease, as well as to evaluate sCR1 levels in a Latin American population. SNPs present in the regulatory and coding regions of this gene may be playing a key role in the observed association, probably by interfering in Aβ plaques clearance. Inhibition may be due to the increase in local sCR1 levels observed in patients, which may result from polymorphisms leading to larger isoforms of CR1 and/or structural alterations of the protein that makes it less functional, as well as increased vesiculation of the molecules.

Pemphigus foliaceus is an autoimmune disease that is sporadic around the world but endemic in Brazil, where it is known as fogo selvagem (FS). Characterized by autoantibodies against the desmosomal cadherin desmoglein 1, FS causes painful erosions, and crusts that may be widespread. The recognition of antigens, including exposed sugar moieties, activates the complement system. Complement receptor 1 (CR1, CD35), which is responsible for the Knops blood group on erythrocytes (York and McCoy antigens), is also expressed by antigen-presenting cells. This regulates the complement system by removing opsonized antigens, blocking the final steps of the complement cascade. Membrane-bound CR1 also fosters antigen presentation to B cells, whereas soluble CR1 has anti-inflammatory properties. CR1 gene polymorphisms have been associated with susceptibility to complex diseases. In order to investigate the association of CR1 polymorphisms with FS susceptibility, we developed a multiplex sequence-specific assay to haplotype eleven polymorphisms in up to 367 FS patients and 242 controls from an endemic area and 289 from a non-endemic area. We also measured soluble CR1 (sCR1) in the serum of 53 FS patients and 27 controls and mRNA levels in the peripheral blood mononuclear cells of 63 genotyped controls. The haplotypes CR1 * 3B2B (with the York antigen-encoded by p.1408Met) and CR1 * 3A2A (with p.1208Arg) were associated with protection against FS (OR = 0.57, P = 0.027, and OR = 0.46, P = 0.014, respectively). In contrast, the CR1 * 1 haplotype (with the McCoy antigen - encoded by p.1590Glu) was associated with FS susceptibility (OR = 4.97, P < 0.001). Heterozygote rs12034383 * A/G individuals presented higher mRNA expression than homozygotes with the G allele ( P = 0.04). The lowest sCR1 levels occurred in patients with active disease before treatment ( P = 0.036). Patients in remission had higher levels of sCR1 than did healthy controls ( P = 0.013). Among those under treatment, patients with localized lesions also presented higher sCR1 levels than those with generalized lesions ( P = 0.0073). In conclusion, the Knops blood group seems to modulate susceptibility to the disease. Furthermore, corticosteroid treatment might increase sCR1 serum levels, and higher levels may play an anti-inflammatory role in patients with FS, limiting the distribution of lesions. Based on these results, we suggest CR1 as a potential new therapeutic target for the treatment of FS., (Copyright © 2019 Oliveira, Kretzschmar, dos Santos, Camargo, Nisihara, Farias, Franke, Wittig, Schmidt, Busch, Petzl-Erler and Boldt.)

Introduction: Fibromyalgia (FM) symptoms worsen in a significant portion of patients with the onset of menopause. Some patients report that their symptoms begin after menopause, suggesting a relationship between these entities. Sleep disturbance is a common condition in FM and menopause, and it is associated with chronic pain., Methods/objectives: Several electronic databases were searched, from the first available year to April 2018 to evaluate the publications that assessed the effects of menopause and sleep disturbance on the appearance or worsening of FM and the role of hormone therapy for these patients., Results: The results are summarized in three tables. The objective sleep patterns of FM patients included high sleep latency, frequent arousals and intrusion of alpha wave sleep and NREM (non-rapid eye movement) sleep in delta sleep. Poor sleep during menopause is more frequent in late perimenopause and surgical menopause, and may be related to vasomotor symptoms or not. Hormone therapy exerted a positive effect on subjective sleep quality of symptomatic menopausal women. Studies have shown a high association between FM and early and surgical menopause. Raloxifene exerted a positive effect on pain and sleep in FM patients; however one study that analyzed the effects of transdermal estrogen therapy found no improvement in subjective and objective parameters of pain., Conclusion: Further studies are needed to elucidate the nature of the association between menopause, sleep and persistent pain syndromes, such as FM, showing the role of hormone therapy in prospective placebo-controlled trials., (© 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Background: Pathophysiological mechanisms are still incompletely understood for leprosy, an urgent public health issue in Brazil. Complement receptor 1 (CR1) binds complement fragments C3b/C4b deposited on mycobacteria, mediating its entrance in macrophages. We investigated CR1 polymorphisms, gene expression and soluble CR1 levels in a case-control study with Brazilian leprosy patients, aiming to understand the role of this receptor in differential susceptibility to the disease., Methodology: Nine polymorphisms were haplotyped by multiplex PCR-SSP in 213 leprosy patients (47% multibacillary) and 297 controls. mRNA levels were measured by qPCR and sCR1 by ELISA, in up to 80 samples., Principal Findings: Individuals with the most common recombinant haplotype harboring rs3849266*T in intron 21 and rs3737002*T in exon 26 (encoding p.1408Met of the York Yka+ antigen), presented twice higher susceptibility to leprosy (OR = 2.43, p = 0.017). Paucibacillary patients with these variants presented lower sCR1 levels, thus reducing the anti-inflammatory response (p = 0.040 and p = 0.046, respectively). Furthermore, the most ancient haplotype increased susceptibility to the multibacillary clinical form (OR = 3.04, p = 0.01) and presented the intronic rs12034383*G allele, which was associated with higher gene expression (p = 0.043), probably increasing internalization of the parasite. Furthermore, there was an inverse correlation between the levels of sCR1 and mannose-binding lectin (initiator molecule of the lectin pathway of complement, recognized by CR1) (R = -0.52, p = 0.007)., Conclusions: The results lead us to suggest a regulatory role for CR1 polymorphisms on mRNA and sCR1 levels, with haplotype-specific effects increasing susceptibility to leprosy, probably by enhancing parasite phagocytosis and inflammation., Competing Interests: The authors have declared that no competing interests exist.

Background: Acute myocardial infarction (AMI) is a potentially fatal condition, being a major cause of death worldwide. Ischemia suffered during AMI causes tissue damage, leading to an inflammatory process. Moreover, myocardial injury can generate damage-associated molecular patterns that activate pattern recognition molecules including some complement proteins., Methods: Here we investigated products of complement activation, C3d and soluble C5b9 (sC5b9), as potential biomarkers for myocardial injury and inflammation, as well as serum cytokines (IL-6 and TNF-alpha), alpha-1-acid glycoprotein (AGP), and classical markers of myocardial necrosis (creatine kinase, creatine kinase-MB isoform, myoglobin and troponin-I) in a longitudinal study of patients with AMI (from admission, 6 h and 12 h post admission, and at discharge from hospital). Individuals undergoing cardiac catheterization (CC) with normal coronary arteries and asymptomatics with no history of cardiovascular disease or invasive procedures were included as controls., Results: Plasma C3d was higher in AMI at admission, 6 h, 12 h, and discharge vs CC (p < 0.0001; p = 0.0061; p = 0.0081; p = 0.044) and asymptomatic (p = 0.0001 for admission, 6 h and 12 h; p = 0.0002 for discharge). Moreover, sC5b9 was higher only at admission and 6 h vs asymptomatic (p = 0.0031 and p = 0.0019). Additionally, AGP levels were elevated at admission, 6 h, 12 h, and discharge vs asymptomatic (p = 0.0003; p = 0.0289; p = 0.0009, p = 0.0017). IL-6 concentration was low at admission and 6 h and reached a peak at 12 h (p < 0.0001 for all groups). All classical markers of myocardial necrosis presented higher concentration at 6 h., Conclusions: Our results showed that complement activation is an early event in AMI occurring before the elevation of classical markers of myocardial necrosis such as creatine kinase, creatine kinase-MB isoform, myoglobin and troponin-I. These findings indicated C3d and sC5b9 as possible biomarkers for inflammation and tissue damage in AMI., (Copyright © 2018. Published by Elsevier B.V.)

Introduction: Development of technologies to reduce transfusion risks of infectious diseases is a major characteristic of hemotherapy. Thus, each donation undergoes clinical and serological screening tests to ensure the donated blood do not offer risks to the receiver., Objective: Evaluate the prevalence of positive serology in blood donations rejected by Hemobanco (Curitiba - PR) in the period ranging from January 2003 to December 2012., Methodology: During the period studied, we observed the total number of donations and its division according to gender. We also analyzed the number of rejected donations due to seropositivity, considering the diseases investigated routinely in blood banks in Brazil, and the frequency of discards according to age groups., Results: Within the period studied, 399,280 donations were performed. 62.0% donors were male. Comparing 2003 to 2012, we noticed a significant decrease of discards, from 10.2% to 5.0%, respectively. There was a reduction of seropositivity for HIV, HBsAg and anti-HBc and an increase for Chagas Disease, hepatitis C, syphilis and HTLV. The age group with the highest prevalence for discards changed: it used to be the 40-59 years old group in 2003, and became the 20-39 years old group in 2012., Conclusion: There was an increase in the number of donations in Hemobanco and a decrease in total discards due to seropositive donations. Most donors were male. The most prevalent cause of discards only amongst seropositive donation discards is seropositivity for anti-HBc. There was a significant increase of donors aged between 20 and 39 years old., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Objective: To describe the profile of lawsuits related to drug requests filled at the Federal Justice of the State of Paraná., Methods: A cross-sectional study, and the data were obtained through consulting the lawsuits at the online system of the Federal Justice of Paraná., Results: Out of 347 lawsuits included in the study, 55% of plaintiffs were women, with a median age of 56 years. Oncology was the field with more requests (23.6%), and the highest mean costs. A wide variety of diseases and broad variety of requested drugs were found in the lawsuits. Approximately two-thirds of them were requested by the brand name, and the most often requested drugs were palivizumab and tiotropium bromide. Only 14.5% of the requested medicines were registered in the National Medication Register. The Public Defender's Office filled actions in 89.6% of cases and all lawsuits had an interim relief. The mean time for approval was 35 days and 70% of requests were granted., Conclusion: Oncology was the field with the highest demand for medicines at the Federal Justice of Paraná in 2014. A great variety of medications was requested. The Public Defender´s Office represented most lawsuits. All demands had an interim relief, and the majority of requests were granted, within an average of 35 days., Objetivo: Descrever o perfil das ações que solicitam medicamentos ajuizadas na Justiça Federal do Paraná., Métodos: Estudo transversal descritivo, cujos dados foram obtidos por meio de consulta aos processos no sistema on-line da Justiça Federal do Paraná., Resultados: Dentre os 347 processos incluídos no estudo, 55% dos autores eram mulheres, com mediana da idade de 56 anos, sendo a área mais procurada a oncologia (23,6%). A área oncológica também foi a que apresentou maiores custos médios. Foi ampla a variedade de doenças geradoras das ações e também foi consequentemente grande a variedade de medicamentos solicitados. Cerca de dois terços dos fármacos foram solicitados pelo nome comercial, e os mais requeridos foram o palivizumabe e brometo de tiotrópio. Apenas 14,5% dos medicamentos solicitados estavam cadastrados no Registro Nacional de Medicamentos. A Defensoria Pública impetrou as ações em 89,6% dos casos. Todos os processos pediam antecipação de tutela do medicamento. O tempo médio para deferimento foi de 35 dias, sendo que 70% dos pedidos foram deferidos., Conclusão: A área com maior número de casos de demanda de medicamentos na Justiça Federal do Paraná no ano de 2014 foi de Oncologia. Observou-se grande variedade de medicamentos solicitados. A maioria das ações foi impetrada pela Defensoria Pública. Todas as demandas exigiram antecipação de tutela, sendo que a maioria dos pedidos foram deferidos, num prazo médio de 35 dias.

Background: Inflammatory bowel diseases (IBD), including Crohn's disease (CD) and ulcerative colitis (UC), are characterized by chronic inflammation of the intestine that can reduce the absorption of nutrients such as vitamin D and calcium., Objective: To investigate bone alterations and serum levels of vitamin D in patients with IBD., Method: This was a cross-sectional study based on a review of medical records of patients from a private office in Curitiba, PR, Brazil. Serum levels of vitamin D and bone densitometry were measured at diagnosis of IBD. A total of 105 patients were included; 38 (58.4%) with CD; 27 (41.6%) with UC and 40 with irritable bowel syndrome (IBS) as comparison group., Results: When compared to patients with UC, CD patients showed a higher prevalence of bone alterations, being 15.8% with osteoporosis and 36.8% with osteopenia. In UC, bone alterations occurred in 29.6% of cases, 3.7% with osteoporosis and 25.9% with osteopenia. As for vitamin D levels, among CD patients, 10.5% had vitamin deficiency, 65.8% insufficiency and 23.7% were sufficient. In UC, 7.4% of cases had deficiency, 74.1% insufficiency and 18.5% had sufficient serum levels of vitamin D. In the group with IBS, deficiency was observed in 17.5% of cases, insufficiency in 55% and sufficiency in 27.5% of them. There was no significant difference between groups., Conclusion: IBD patients have a high prevalence of bone changes, especially those with CD. Serum levels of vitamin D are below the recommended in all the evaluated groups.

Background:: Occupational dermatitis affects the quality of life and productivity of workers. Studies on the subject are scarce in Brazil. It is estimated that the disease is underreported and that many affected patients do not seek health care., Objectives:: To conduct an epidemiological analysis of occupational dermatitis notified via SINAN in Brazil from January 2007 to December 2012; evaluate the profile of patients assisted; and check the main etiological agents involved., Methods:: We analyzed the compulsory notification forms of cases of occupational dermatitis filled nationwide during January 2007 to December 2012., Results:: During the study period 3027 cases of occupational dermatitis were notified in Brazil. In 61.4% of cases patients were men aged between 35-49 years (39.6%). The most described etiological agent was chromium (13.9%). The location of the body most affected was the hands, with 28.4% of cases. The construction sector is implicated in 28.7% of cases and domestic services by 18%. Allergic contact dermatitis is the most prevalent occupational dermatitis (20.6%) and the region with the highest number of notifications was the Midwest, with 376.4 cases per million inhabitants., Conclusions:: The profile of patients most affected by occupational dermatitis in Brazil during the study period was: men with elementary school, aged between 20 and 49 years old and working in the construction industry. The most common occupational dermatitis were allergic contact dermatitis caused by chromium after years of exposure, being the hands and head the parts of the body most affected., Competing Interests: None

Background: Pentraxin-3 (PTX3) is a long pentraxin that is supposed to participate in the inflammatory process and in atherosclerosis., Aim: To study PTX3 serum levels in rheumatoid arthritis (RA) patients to know if its serum levels may reflect disease activity and/or subclinical atherosclerosis., Methods: PTX3 and carotid intima media thickness (IMT) were studied in 85 RA patients (83.5% females, median age of 59years old, median disease duration of 13years) along with its demographic, clinical, serological and lipid profile. For comparison PTX3 was measured in 85 healthy volunteers., Results: PTX3 levels in RA patients were similar to controls (p=0.21) and did not correlate with inflammatory activity measured by ESR (p=0.39) CRP (p=0.18) and DAS28 (p=0.67). Serum PTX3 levels were higher in nonobese RA patients than in obese (BMI vs PTX3 with rho=-0.27; 95%IC=-0.46 to -0.06; p=0.009). In non-obese patients, PTX3 correlated negatively with carotid IMT (rho=-0.40; 95%IC=-0.66 to -0.06; p=0.01) but not in the obese ones (p=0.26). In the obese RA patients there was a negative correlation between PTX3 levels and LDL/HDL ratio (Rho=-0.29; 95%IC=-0.53-0.01; p=0.03)., Conclusions: PTX3 levels do not reflect inflammatory process in RA. However, it exerts a protective role in the process of atherogenesis in non-obese RA patients., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: 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., Methods: 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., Results: 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., Conclusions: 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.

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., (© The Author(s) 2015.)

Objective: To determine the incidence of infections in a population of systemic lupus erythematosus individuals and the characteristics of infections regarding original site, as well as to study the possible associations between infections and treatment., Methods: An analytical retrospective study using data from medical charts of systemic lupus erythematosus patients from a single university hospital. A total of 144 patients followed up for five years were included. Data collected comprised age of patients and age at onset of lupus, sex and ethnicity, disease duration before the study period, medications, cumulative dose of prednisone, occurrence of infections and their original site., Results: The most frequent infections were urinary tract infections (correlated to use of prednisone - p<0.0001 and cyclophosphamide - p=0.045), upper airways infections (correlated to use of prednisone - p=0.0004, mycophenolate mofetil - p=0.0005, and cyclosporine - p=0.025), and pneumonia (associated to prednisone - p=0.017)., Conclusion: Prednisone was the drug more often associated with presence of infections, pointing to the need for a more judicious management of this drug.

Background: Low bone mineral density is considered an extra-intestinal manifestation of celiac disease with reduced bone mass, increased bone fragility, and risk of fractures. Celiac disease is considered a condition at high risk for secondary osteoporosis and the evaluation of bone density is very important in the clinical management of these patients., Objective: The present study aimed to investigate bone alterations in celiac patients from Curitiba, South Region of Brazil at diagnosis, correlating the findings with age and gender., Methods: Patients who were included in the study were attended to in a private office of the same physician from January 2009 to December 2013. The diagnosis of celiac disease was done through clinical, serological and histological findings. All data were collected from the medical charts of the patients. After the diagnosis of celiac disease, evaluation for low bone mineral density was requested by dual-energy X-ray absorptiometry (DEXA). DEXA bone densitometer was used to estimate low bone mineral density at the lumbar spine and femur., Results: A total of 101 patients, 82 (81.2%) female and 19 (18.8%) male subjects, with mean age of 39.0±3.03 years were included. At celiac disease diagnosis, 36 (35.6%) were younger than 30 years, 41 (40.6%) were between 31 and 50 years, and 24 (23.8%) were older than 50 years. Among the evaluated patients, 69 (68.3%) presented low bone mineral density, being 47% with osteopenia and 32% with osteoporosis. Patients who were older than 51 years and diagnosed with celiac disease presented low bone mineral density in 83.3% (20/24) of the cases. As expected, age influenced significantly the low bone mineral density findings. Among women, low bone mineral density was present with high frequency (60%) from 30 to 50 years. In patients diagnosed older than 60 years (n=8), all the women (n=5) and two of the three men had osteoporosis., Conclusion: This study demonstrated that 69% of Brazilian patients with celiac disease at diagnosis had low bone mineral density, being more frequent in women older than 50 years.

Background: Restrictions imposed by the gluten-free diet generate large changes in the daily habits of the celiac patient, causing a negative impact on quality of life., Objective: This study aimed to evaluate the quality of life of patients with celiac disease on a capital in Southern Brazil., Methods: Patients older than 18 years were included, with confirmed celiac disease for at least 60 days in the period from June to October 2013. A validated questionnaire, with specific questions to assess the patient's quality of life celiac was applied. A total score ranged from 20 to 100 points; the higher the score, worse quality of life., Results: A total of 103 questionnaires were evaluated, 96 (93.2%) female, with average score 56.6±12.35 (28 to 88 points). The comparison between the questionnaire scores and family income was not significant (P=0.139). Patients diagnosed less than 1 year have poorer quality of life than those with more than 10 years (P=0.063). Patients older than 60 years had better quality of life compared with the younger ones (P=0.04)., Conclusion: There was no association between quality of life and factors such as family income, length of diet and age at diagnosis. Chronological age greater than 60 years has positively influenced the quality of life of celiac patients.

Background: Determination of fecal calprotectin can provide an important guidance for the physician, also in primary care, in the differential diagnosis of gastrointestinal disorders, meanly between inflammatory bowel diseases and irritable bowel syndrome. Objectives The aims of the present study were to prospectively investigate, in Brazilian adults with gastrointestinal complaints, the value of fecal calprotectin as a biomarker for the differential diagnosis between functional and organic disorders and to correlate the concentrations with the activity of inflammatory bowel diseases., Methods: The study included consecutive patients who had gastrointestinal complaints in which the measurement levels of fecal calprotectin were recommended. Fecal calprotectin was measured using a Bühlmann (Basel, Switzerland) ELISA kit., Results: A total of 279 patients were included in the study, with median age of 39 years (range, 18 to 78 years). After clinical and laboratorial evaluation and considering the final diagnosis, patients were allocated into the following groups: a) Irritable Bowel Syndrome: 154 patients (102 female and 52 male subjects). b) Inflammatory Bowel Diseases group: 112 patients; 73 with Crohn's disease; 38 female and 35 male patients; 52.1% (38/73) presented active disease, and 47.9% (35/73) had disease in remission and 39 patients with ulcerative colitis;19 female and 20 male patients; 48.7% (19/39) classified with active disease and 49.3% (20/39) with disease in remission. A significant difference (P<0.001) was observed between the median value of fecal calprotectin in Irritable Bowel Syndrome group that was 50.5 µg/g (IQR=16 - 294 µg/g); 405 µg/g (IQR=29 - 1980 µg/g) in Crohn's disease patients and 457 µg/g (IQR=25 - 1430 µg/g) in ulcerative colitis patients. No difference was observed between the values found in the patients with Crohn's disease and ulcerative colitis. Levels of fecal calprotectin were significantly lower in patients with inflammatory bowel diseases in remission when compared with active disease (P<0.001)., Conclusions: The present study showed that the determination of fecal calprotectin assists to differentiate between active and inactive inflammatory bowel diseases and between inflammatory bowel diseases and irritable bowel syndrome.

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.

MASP-2 is a key protein of the lectin pathway of complement system. Several MASP2 polymorphisms were associated with MASP-2 serum levels or functional activity. Here we investigated a possible association between MASP2 polymorphisms and MASP-2 serum levels with the susceptibility to rheumatic fever (RF) and rheumatic heart disease (RHD). We haplotyped 11 MASP2 polymorphisms with multiplex sequence-specific PCR in 145 patients with history of RF from south Brazil (103 with RHD and 42 without cardiac lesion [RFo]) and 342 healthy controls. MASP-2 levels were determined by ELISA. The low MASP-2 producing p.377A and p.439H variants were negatively associated with RF (P=0.02, OR=0.36) and RHD (P=0.01, OR=0.25). In contrast, haplotypes that share the intron 9 - exon 12 g.1961795C, p.371D, p.377V and p.439R polymorphisms increased the susceptibility to RHD (P=0.02, OR=4.9). MASP-2 levels were associated with MASP2 haplotypes and were lower in patients (P<0.0001), which may reflect protein consumption due to complement activation. MASP2 gene polymorphisms and protein levels seem to play an important role in the development of RF and establishment of RHD., (Copyright © 2014 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.)

Introduction: Rheumatoid arthritis (RA) is a commonly occurring systemic inflammatory auto immune disease and is believed to be associated with genetic factors. The innate immune complement protein Mannose binding lectin (MBL) and their MBL2 genetic variants are associated with different infectious and autoimmune diseases., Methods: In a Brazilian cohort, we aim to associate the functional role of circulating MBL serum levels and MBL2 variants in clinically classified patients (n = 196) with rheumatoid arthritis including their relatives (n = 200) and ethnicity matched healthy controls (n = 200). MBL serum levels were measured by ELISA and functional MBL2 variants were genotyped by direct sequencing., Results: The exon1+54 MBL2*B variant was significantly associated with an increased risk and the reconstructed haplotype MBL2*LYPB was associated with RA susceptibility. Circulating serum MBL levels were observed significantly lower in RA patients compared to their relatives and controls. No significant contribution of MBL levels were observed with respect to functional class, age at disease onset, disease duration and/or other clinical parameters such as nodules, secondary Sjögren syndrome, anti-CCP and rheumatoid factor. Differential distribution of serum MBL levels with functional MBL2 variants was observed in respective RA patients and their relatives., Conclusions: Our results suggest MBL levels as a possible marker for RA susceptibility in a Brazilian population.

Introduction: Patients with AIDS (acquired immunodeficiency syndrome) may have rheumatic complaints such as arthritis and arthralgia, dry eyes, increased salivary glands, lower back pain, enthesitis etc. Autoantibodies like ANA (antinuclear antibody) and RF (rheumatoid factor) may also be present., Objective: To study the prevalence of rheumatic complaints in AIDS patients and correlate them with the presence of ANA and RF., Methods: We studied 69 patients with AIDS (28.9% women and 71.0% men) with a mean age of 40.8 ± 8.9 years, median disease duration of 60 months, for rheumatic complaints, ANA, ENA-6 (anti-Ro, anti-La, anti-Sm, anti-RNP, anti-Scl70 and anti-Jo1) and RF. We collected demographic data, CD4+ and CD8+ cell count and values of viral load., Results: Arthralgia was present in 39.1%, sicca symptoms in 21.7%, inflammatory lumbar pain in 13.4%, enthesopathy in 6.6%, parotid enlargement in 1.4%, RF in 10.1% and ANA in 8.6%. All patients were negative for ENA-6. ANA was more common in older patients (p = 0.03) and in those with higher viral load (p = 0.006). No association was found with the presence of RF., Conclusions: The most common manifestation in this context was arthralgia. ANA presence was associated with age of the patients and viral load.

Introduction: There is a high prevalence of congenital heart disease (CHD) in Down syndrome (DS) patients. Children with DS and CHD also present greater susceptibility to pulmonary infections than those without CHD., Aim: To investigate the prevalence and types of CHD and their association with severe infections in children with DS in southern Brazil seen in a reference outpatient clinic., Methods: Children aged between six and 48 months with a diagnosis of DS were included consecutively in the period May 2001 to May 2012, and the presence of CHD and severe infections (pneumonia and sepsis) was investigated, classified and analyzed., Results: A total of 127 patients were included, of whom 89 (70.1%) had some type of CHD, 33 (37.7%) of them requiring surgical correction. Severe infections (pneumonia and sepsis) were seen in 23.6% and 5.5%, respectively. Of the cases of pneumonia, 70% had associated CHD (p=0.001) and of those with sepsis, 85% presented CHD (p=0.001)., Conclusions: Our study showed a high prevalence of CHD and its association with severe infections in children with DS seen in southern Brazil., (Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

Background/aims: Lupus nephritis (LN) is one of the most serious manifestations of SLE occurring in 66-90% of these patients. The complement system is part of the innate immunity and modulator of inflammation and the adaptative immune response. Mannan-binding lectin (MBL) and Ficolin-2 (FCN-2) are important members of the lectin pathway of complement activation. Despite the significant participation of complement in the pathogenesis of the LN, there are few reports demonstrating "in situ" deposition of complement components in renal biopsy specimens in this disorder. The present study investigated the deposition of complement components in kidney specimens of LN patients., Methods: Renal biopsies of 11 patients with SLE and LN were evaluated for immunofluorescence staining for IgG, IgA, IgM, C3, and C1q. Additionally, MBL, FCN-2 and C5b-9 were researched using monoclonal antibodies., Results: All the biopsies were positive for IgG, C3, and C1q, eight were positive IgM and five had IgA deposition in glomerular tissue. The terminal complex of complement C5b9 was positive in all cases, MBL in nine (82%) cases; seven (63.6%) of them presenting concomitantly FCN-2 deposition. Patients presenting MBL deposition had higher mean of urinary proteins (9.0 g/day) than patients with negative MBL deposition (mean of 2.3g/day)., Conclusions: In this study, we demonstrated in situ the participation of complement in the renal injury, including MBL and FCN-2 of the lectin pathway; also the strong role of C5b-9 in the pathogenesis of LN., (Copyright © 2013 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.)

Context: Autoimmune diseases are 3 to 10 times more frequently in patients with celiac disease and their relatives than in the general population., Objective: To investigate a broad spectrum of autoantibodies in celiac disease relatives from Southern Brazil, in a serological follow-up of 6-10 years, aiming to associate with other autoimmune diseases, degree of parentage, demographic and clinical data., Methods: Serum samples of 233 relatives were analyzed in two different phases: n = 186 in phase I (1997-2000) and n = 138 (being 91 = follow-up group and 47 = newly tested) in phase II (2006-2007). As controls, 100 unrelated individuals were evaluated. Autoantibodies to smooth muscle, mitochondrial, liver-kidney microssome, parietal cell and thyroid microssome were tested by indirect immunofluorescence., Results: A significant increase of autoantibodies, in both phases, was observed in the relatives when compared to the non-relatives (P = 0.0064), specifically to anti-thyroid microssome and anti-parietal cell. In both phases, the female/male proportion of autoantibodies was of 4:1 to 3:1 (P<0.041). The frequency of autoantibodies amongst 1st and 2nd degree relatives was 11.8% and 9.68% in phase I and 4% and 6.67% in phase II., Conclusion: Celiac disease relatives presented other autoantibodies and serological screening is a useful instrument for identifying autoimmune diseases along the years.

Anti-CCP (cyclic citrullinated peptide) is considered the most useful laboratory tool in the diagnosis of rheumatoid arthritis (RA). Some authors have also found this autoantibody in patients with scleroderma (SSc). The study aimed to investigate the prevalence of anti-CCP antibodies in SSc patients from Southern Brazil and their association with clinical and serological profile of the disease. We studied 76 patients with SSc and 100 healthy volunteers for presence of anti-CCP. SSc patients charts were reviewed for clinical and laboratory data. In the SSc group, the diffuse form was present in 20.5%; 62.8% had the limited form; 14.1% had overlap with systemic lupus or polymyositis and 2.5% had SSc sine scleroderma. Anti-CCP was found in nine of 78 (11.5%) SSc patients and in one of 100 healthy volunteers (p = 0.0054). No relationship was found with arthritis, skin Rodnan m score, esophageal dysmotility, myocarditis, pulmonary hypertension and lung fibrosis. Positive association was observed with arthralgias (p = 0.02). Also, no relationship was noted with the presence of anti-centromere antibodies, anti-Scl-70, anti-RNP or rheumatoid factor. Anti-CCP are more common in SSc patients than in controls. Arthralgias but not arthritis or rheumatoid factor are more frequent in anti-CCP positive patients.

Antinucleosome antibodies have been found with variable prevalence in systemic lupus erythematosus (SLE) and were associated with more severe disease. This research aims to study the prevalence of antinucleosome antibodies in a sample of Brazilian adult SLE patients and their association with clinical findings and disease activity. Ninety-two adult patients (81 females and 11 males, with mean age of 37.29 ± 10.98 years) with SLE were studied for clinical and antibody profile, disease activity by SLE disease activity index (SLEDAI), and presence of antinucleosome antibodies by ELISA. The prevalence of antinucleosome antibodies was 61.9% (mean titer, 87.8 ± 62.6 U). No relationship was found of antinucleosome presence and any of the studied clinical features. A positive association was detected with anti-DNA (p = 0.001) and SLEDAI (p < 0.0001), but not with anti-Sm, anti-Ro, anti-La, and anti-RNP. No specific disease feature could be associated with the presence of antinucleosome; however, a positive relationship was detected with disease activity measured by SLEDAI and with anti-DNA presence.

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.

Pemphigus is characterized by circulating autoantibodies directed against desmossomal antigens that, once bound to target antigens, induce disruption in the cell-cell adhesion of the epidermis and mucosal epithelium, leading to blister formation. Evidence has indicated a role for complement in the physiopathology of pemphigus, with complement deposition in intercellular spaces of skin and mucous membrane lesions. Mannan-binding lectin (MBL) and Ficolin-2 are recognition proteins of innate immunity, which by binding to specific molecular patterns on pathogens surfaces trigger the activation of complement, leading to phagocytosis and lyses of target cells and inflammation. In this study we report for the first time the deposition of MBL and ficolins in pemphigus lesions. Eight biopsies of skin lesions of pemphigus vulgaris were studied for in situ deposition of IgG and the complement components MBL, Ficolin 1, Ficolin-2, C1q, C3 and membrane attack complex C5b-9. All biopsies presented deposition of IgG and C3 in the intercellular spaces (ICS) of epidermis. MBL deposition was found in the ICS and basal membrane zone (BMZ) of all specimens, whereas C5b-9 showed deposition only in the ICS, with irregular distribution. Ficolin-2 were positive in 50% (4/8) of biopsies showing deposition in the BMZ. On the other hand, ficolin-1 and C1q were negative in all specimens. Our study suggest that MBL and to a lesser extend Ficolin-2 may bind to altered intercellular structures in the skin and lead to the activation of complement in situ, contributing to tissue damage in pemphigus.

Gender and environmental factors are known to influence the clinical heterogeneity and outcome of rheumatoid arthritis (RA). Some variables have been suggested to be associated with the severity of the disease, which can be of great value in the correct management of RA patients. The purpose of this study was to investigate the associations among anticyclic citrullinated antibody (anti-CCP2) positivity, extra-articular manifestations (EAM), gender, and tobacco exposure in a Brazilian RA population. We performed a transversal study comprising 156 RA patients which were investigated for EAM, functional class, presence of anti-CCP2, and IgM rheumatoid factor (IgM-RF). The determination of anti-CCP2 was performed using enzyme immunoassay (ELISA) kits and IgM-RF by latex agglutination test. Clinical and demographical data were obtained through review of charts. Anti-CCP positivity intensity was directly correlated with tobacco smoking, sex, and the development of rheumatoid nodules. Intense anti-CCP2 reaction was 19.8-fold higher in females vs. males, 2.7-fold higher in tobacco vs. non-tobacco users, 7.7-fold higher in female vs. male tobacco users, and 5.15-fold higher in patients with rheumatoid nodules. Tobacco smoking, gender, and rheumatoid nodules are significantly correlated with anti-CCP2 positivity in Brazilian RA patients.

Background: Clustering of autoimmune diseases is common and may be due to genetic background and exposition to environmental triggers., Objective: 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., Methods: 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., Results: 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., Conclusion: 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.

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., (Copyright © 2010 S. Karger AG, Basel.)

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.

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.

Background: Anti-Saccharomyces cerevisae antibody (ASCA) and perinuclear anti-neutrophil cytoplasmatic antibody (pANCA) remain the most well-established markers in inflammatory bowel disease (IBD), and both may be associated with disease phenotype., Aim: To determine the utility of ASCA and pANCA as markers in a Brazilian cohort of IBD patients., Materials and Methods: A total of 90 patients with ulcerative colitis (UC), 77 patients with Crohn's disease (CD), and 57 healthy individuals were included in the study. ASCA was determined by enzyme-linked immunosorbent assay (ELISA) and pANCA by immunofluorescence assay., Results: In support of diagnosis of UC, the sensitivity and specificity of pANCA were 51% and 100%, respectively. ASCA (IgA or IgG isotypes) presented sensitivity of 62% and specificity of 93% for CD. The combination of ASCA negativity and pANCA positivity (ASCA-/pANCA+) displayed sensitivity of 43% and specificity of 100% for diagnosis to UC. In CD patients, ASCA+/pANCA- presented sensitivity and specificity of 57% and 93%, respectively. Additionally, ASCA positivity correlated with early age at disease onset and ileal location in CD patients. In UC patients, pANCA positivity was correlated with pancolitis or left colitis., Conclusions: The results evidenced that low sensitivity of ASCA and pANCA markers limits their use in IBD screening in the general population; however, their specificity may contribute to differentiation between CD and UC in IBD patients. Our study lends further support to the suggestion that serologic assessment identifies different subtypes of IBD.

Context: Anti-Saccharomyces cerevisiae antibodies (ASCA), considered serologic markers for Crohn's disease, were described in patients with celiac disease, disappearing after a gluten-free diet., Objectives: Evaluation of ASCA positivity in patients with Crohn's disease and celiac disease in relation to healthy individuals., Methods: A total of 145 individuals were studied: 36 with Crohn's disease and 52 with celiac disease, that fulfilled the diagnostic criteria for both affections, and 57 healthy individuals for control. The celiac patients were divided as follow: group CeD I at diagnosis (n = 34), group CeD II with gluten-free diet compliance (n = 13) and group CeD III with transgressions to the diet (n = 5). ASCA IgA and IgG were determined by ELISA., Results: With statistical significance, ASCA IgA were positive in Crohn's disease, celiac disease at diagnosis and celiac disease with diet transgressions; ASCA IgG in Crohn's disease and in all groups with celiac disease., Conclusions: The detection of ASCA in patients with celiac disease allows to suggest that ASCA is not a specific marker for Crohn's disease, but was associated with the inflammation of the small intestine. The increased levels of positive ASCA may be due to genetic factors and increased intestinal permeability.

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.

Background: Celiac disease has been described in populations from around the world, with recent data emphasizing the occurrence of the disease in ethnic minorities. There are only a few studies evaluating celiac disease in native Indians., Aims: This study aimed to screen the anti-endomysial antibody (IgA-EmA) in Kaingang and Guarani Indians from southern Brazil, in order to establish a clinical serological evaluation of celiac disease in these individuals., Material and Methods: Serum samples from 321 individuals (125 male and 196 female; 4-86 years old) from Mangueirinha Indigenous Reserve, State of Parana, Brazil, and 180 non-Indigenous healthy individuals (62 male and 118 female; 2-81 years old) were analysed to the presence of anti-endomysial antibody class IgA by indirect immunofluorescence assay. Amongst the Indians, 158 were Kaingang, 98 Guarani and 65 of mixed race. Indians presenting complaints of diarrhea (N=12) were also evaluated to the IgG class of anti-endomisyal antibody., Results: None of the individuals showed positive results either to IgA or IgG anti-endomysial antibodies., Conclusions: Although the results indicate an absence of celiac disease in Kaingang and Guarani Indians, the authors call attention to the importance of following up indigenous children or adults presenting gastrointestinal complaints or other symptoms related to the disease. Consideration should be given to the genetic background of these individuals, allied to the inter ethnic marriages and the changing habits or occupational activities, that have gradually introduced diseases previously not described in indigenous populations.