Abstract Introduction Takayasu’s arteritis (TAK) patients are at an elevated risk of metabolic syndrome and cardiovascular diseases (CVD). Currently, there are no well-validated biomarkers to assess this risk in this population. Previous research in different cohorts has linked serum levels of osteoprotegerin (OPG) and its polymorphisms to accelerated atherosclerosis and a marker of poor prognosis in CVD. Thus, we assessed this protein as a potential biomarker of CVD in TAK patients. Objectives To evaluate the serum levels of OPG and its SNPs (single nucleotide polymorphisms) in TAK patients and healthy controls, and to associate these parameters with clinical data. Methods This bicentric cross-sectional study included TAK patients who were compared with healthy individuals (control group). The serum levels of OPG and the frequency of OPG SNPs [1181G > C (rs2073618), 245 A > C (rs3134069), 163T > C (rs3102735), and 209 C > T (rs3134070)] were compared between the both groups and associated with clinical data. Results In total, 101 TAK patients and 93 controls were included in the study. The serum levels of OPG (3.8 ± 1.9 vs. 4.3 ± 1.8pmol/L, respectively; P = 0.059), and its four polymorphisms were comparable between both groups. In an additional analysis of only TAK patients, serum OPG levels and its four genes were not associated with any CVD parameters, except for higher OPG levels among patients without dyslipidemia. Conclusion No significant differences were observed in serum OPG levels or in the genotype frequencies of OPG SNPs between the patient and control groups. Similarly, no correlation was found between laboratory parameters and clinical data on CVD risk in TAK patients. Takayasus Takayasu s (TAK . (CVD) Currently wellvalidated well validated population (OPG Thus single crosssectional cross sectional group. group group) 1181G G rs2073618, rs2073618 rs , (rs2073618) 24 rs3134069, rs3134069 (rs3134069) rs3102735, rs3102735 (rs3102735) 20 rs3134070 (rs3134070) total 10 9 3.8 38 3 8 (3. 19 1 1. vs 43 4 4. 18pmolL pmolL 8pmol L pmol 1.8pmol/L respectively 0.059, 0059 0.059 0 059 0.059) dyslipidemia Similarly (CVD rs207361 (rs2073618 2 rs313406 (rs3134069 rs310273 (rs3102735 rs313407 (rs3134070 3. (3 005 0.05 05 rs20736 (rs207361 rs31340 (rs313406 rs31027 (rs310273 (rs313407 ( 00 0.0 rs2073 (rs20736 rs3134 (rs31340 rs3102 (rs31027 0. rs207 (rs2073 rs313 (rs3134 rs310 (rs3102 rs20 (rs207 rs31 (rs313 (rs310 rs2 (rs20 rs3 (rs31 (rs2 (rs3 (rs

Abstract Background Data on post-acute COVID-19 in autoimmune rheumatic diseases (ARD) are scarce, focusing on a single disease, with variable definitions of this condition and time of vaccination. The aim of this study was to evaluate the frequency and pattern of post-acute COVID-19 in vaccinated patients with ARD using established diagnosis criteria. Methods Retrospective evaluation of a prospective cohort of 108 ARD patients and 32 non-ARD controls, diagnosed with SARS-CoV-2 infection (RT-PCR/antigen test) after the third dose of the CoronaVac vaccine. Post-acute COVID-19 (≥ 4 weeks and > 12 weeks of SARS-CoV-2 symptoms) were registered according to the established international criteria. Results ARD patients and non-ARD controls, balanced for age and sex, had high and comparable frequencies of ≥ 4 weeks post-acute COVID-19 (58.3% vs. 53.1%, p = 0.6854) and > 12 weeks post-acute COVID-19 (39.8% vs. 46.9%, p = 0.5419). Regarding ≥ 4 weeks post-acute COVID-19, frequencies of ≥ 3 symptoms were similar in ARD and non-ARD controls (54% vs. 41.2%, p = 0.7886), and this was also similar in > 12 weeks post-acute COVID-19 (68.3% vs. 88.2%, p = 0.1322). Further analysis of the risk factors for ≥ 4 weeks post-acute COVID-19 in ARD patients revealed that age, sex, clinical severity of COVID-19, reinfection, and autoimmune diseases were not associated with this condition (p > 0.05). The clinical manifestations of post-acute COVID-19 were similar in both groups (p > 0.05), with fatigue and memory loss being the most frequent manifestations. Conclusion We provide novel data demonstrating that immune/inflammatory ARD disturbances after third dose vaccination do not seem to be a major determinant of post-acute COVID-19 since its pattern is very similar to that of the general population. Clinical Trials platform (NCT04754698). postacute post acute COVID19 COVID 19 COVID-1 (ARD scarce disease criteria 10 nonARD non SARSCoV2 SARSCoV SARS CoV 2 SARS-CoV- RTPCR/antigen RTPCRantigen RT PCR/antigen PCR antigen test vaccine Postacute Post ( 1 sex 58.3% 583 58 (58.3 vs 531 53 53.1% 0.6854 06854 0 6854 39.8% 398 39 8 (39.8 469 46 9 46.9% 0.5419. 05419 0.5419 . 5419 0.5419) COVID19, 19, 54% 54 (54 412 41 41.2% 0.7886, 07886 0.7886 , 7886 0.7886) 68.3% 683 68 (68.3 882 88 88.2% 0.1322. 01322 0.1322 1322 0.1322) reinfection 0.05. 005 0.05 05 0.05) 0.05, immuneinflammatory immune inflammatory population NCT04754698. NCT04754698 NCT (NCT04754698) COVID1 COVID- SARS-CoV RTPCR PCRantigen 58.3 5 (58. 53.1 0.685 0685 685 39.8 (39. 46.9 0541 0.541 541 (5 41.2 0788 0.788 788 68.3 6 (68. 88.2 0132 0.132 132 00 0.0 NCT0475469 (NCT04754698 58. (58 53. 0.68 068 39. (39 46. 054 0.54 41. 078 0.78 78 68. (68 88. 013 0.13 13 0. NCT047546 (NCT0475469 0.6 06 (3 0.5 07 0.7 7 (6 01 0.1 NCT04754 (NCT047546 NCT0475 (NCT04754 NCT047 (NCT0475 NCT04 (NCT047 NCT0 (NCT04 (NCT0 (NCT

Resumo Fundamentos Poucos estudos avaliaram pacientes idosos com Arterite de Takayasu (AT). Objetivo Avaliar o progresso de AT em diferentes grupos etários em seus possíveis efeitos sobre o tratamento medicamentoso e atividade da doença. Métodos este estudo transversal, retrospectivo, do tipo coorte incluiu 66 pacientes com AT. Os pacientes foram entrevistados, e dados dos 12 meses anteriores foram coletados dos prontuários médicos eletrônicos. Os pacientes foram divididos em quatro quartis de acordo com idade atual, e comparados quanto aos dados clínicos e laboratoriais, tratamento, comorbidades, status da doença, e status funcional. Um p<0,05 foi estabelecido como estatisticamente significativo. Resultados Os grupos foram definidos como Q1(22-36 anos, n=16), Q2(37-42 anos, n=18), Q3(43-49 anos, n=17), e Q4(51-66 anos, n=15). A frequência de pacientes com atividade da doença, fadiga, comorbidades e comprometimentos vasculares, e o índice de extensão da doença (DEI. Tak) foram comparáveis entre os grupos. Pacientes com idade mais avançada apresentaram maior duração da doença (p=0,001) e maior comprometimento do status funcional (Q2 versus Q3, p=0,003); menos pacientes usaram prednisona (Q1:43,8%; Q2:33,3%; Q3:11,8%; e Q4:6,7%; p=0,049) e agentes imunossupressores [Q1:100,0%; Q2:66,7%; Q3:58,8% e Q4:46,7%; Q1 versus Q3 (p=0,043) e Q1 versus Q4 (p=0,005) nas análises post-hoc]. Além disso, os níveis de danos da doença, sintomas de uma nova ocorrência de AT, e complicações nos 12 meses precedentes não foram diferentes entre os grupos. Conclusão Pacientes com AT e idade mais avançada requerem mínima intervenção medicamentosa e apresentam maior comprometimento no status funcional, o que pode ser atribuído a fatores relacionados ao envelhecimento. . (AT) transversal retrospectivo 6 entrevistados 1 eletrônicos atual laboratoriais p005 p 0 05 p<0,0 significativo Q12236 Q 22 36 Q1(22-3 anos n=16, n16 n n=16 , 16 n=16) Q23742 Q2 37 42 Q2(37-4 n=18, n18 n=18 18 n=18) Q34349 43 49 Q3(43-4 n=17, n17 n=17 17 n=17) Q45166 51 Q4(51-6 n=15. n15 n=15 15 n=15) fadiga vasculares DEI. DEI (DEI Tak p=0,001 p0001 001 (p=0,001 (Q p=0,003 p0003 003 p=0,003) Q143,8% Q1438 43,8% 8 (Q1:43,8% Q233,3% Q2333 33,3% 33 3 Q2:33,3% Q311,8% Q3118 11,8% 11 Q3:11,8% Q46,7% Q467 6,7% 7 Q4:6,7% p=0,049 p0049 049 Q1100,0% Q11000 100,0% 100 [Q1:100,0% Q266,7% Q2667 66,7% Q2:66,7% Q358,8% Q3588 58,8% 58 Q3:58,8 Q446,7% Q4467 46,7% 46 Q4:46,7% p=0,043 p0043 043 (p=0,043 p=0,005 p0005 005 (p=0,005 posthoc. posthoc post hoc post-hoc] disso envelhecimento (AT p00 p<0, Q1223 2 Q1(22- n1 n=1 Q2374 4 Q2(37- Q3434 Q3(43- Q4516 5 Q4(51- p=0,00 p000 00 (p=0,00 Q143 Q143,8 438 43,8 (Q1:43,8 Q233 Q233,3 333 33,3 Q2:33,3 Q311 Q311,8 118 11,8 Q3:11,8 Q46 Q46,7 67 6,7 Q4:6,7 p=0,04 p004 04 Q1100 Q1100,0 1000 100,0 10 [Q1:100,0 Q266 Q266,7 667 66,7 Q2:66,7 Q358 Q358,8 588 58,8 Q3:58, Q446 Q446,7 467 46,7 Q4:46,7 (p=0,04 post-hoc p0 p<0 Q122 Q1(22 n= Q237 Q2(37 Q343 Q3(43 Q451 Q4(51 p=0,0 (p=0,0 Q14 Q143, 43, (Q1:43, Q23 Q233, 33, Q2:33, Q31 Q311, 11, Q3:11, Q46, 6, Q4:6, Q110 Q1100, 100, [Q1:100, Q26 Q266, 66, Q2:66, Q35 Q358, 58, Q3:58 Q44 Q446, 46, Q4:46, p< Q12 Q1(2 Q2(3 Q34 Q3(4 Q45 Q4(5 p=0, (p=0, (Q1:43 Q2:33 Q3:11 Q4:6 Q11 [Q1:100 Q2:66 Q3:5 Q4:46 Q1( Q2( Q3( Q4( p=0 (p=0 (Q1:4 Q2:3 Q3:1 Q4: [Q1:10 Q2:6 Q3: Q4:4 p= (p= (Q1: Q2: [Q1:1 (p (Q1 [Q1: [Q1 [Q

Abstract Background Few studies have assessed elderly patients with Takayasu’s arteritis (TAK). Objectives To evaluate the progression of TAK in different age groups and its possible effects on drug treatment and disease activity. Methods This cross-sectional and retrospective cohort study included 66 TAK patients. Patients were interviewed and data of the 12 preceding months were collected from electronic medical records. The patients were divided into four quartiles according to current age and compared for clinical and laboratory data, treatment, comorbidities, disease status, and functional status. Statistical significance was set at p<0.05. Results The groups were Q1(22-36 years, n=16), Q2(37-42 years, n=18), Q3(43-49 years, n=17), and Q4(51-66 years, n=15). The frequency of patients with disease activity, fatigue, comorbidities and vascular impairments, and the TAK disease extent index were also comparable between the groups. With age, disease duration was longer (p=0.001), fewer patients used prednisone (Q1:43.8%, Q2:33.3%, Q3:11.8%, and Q4:6.7%; p=0.049) and immunosuppressive drugs [Q1:100.0%, Q2:66.7%, Q3:58.8%, and Q4:46.7%; Q1 versus Q3 (p=0.043), and Q1 versus Q4 (p=0.005) in post-hoc analyses], and patients had greater functional status impairment (Q2 versus Q3, p=0.003). In addition, the levels of disease damage, new TAK symptoms, and complications in the preceding 12 months were not different between the groups. Conclusions Older patients with TAK require minimal drug treatment, and have greater impairment of functional status, which may be attributed to aging-related factors. Takayasus Takayasu s TAK. . (TAK) activity crosssectional cross sectional 6 1 records p005 p 0 05 p<0.05 Q12236 Q 22 36 Q1(22-3 years n=16, n16 n n=16 , 16 n=16) Q23742 Q2 37 42 Q2(37-4 n=18, n18 n=18 18 n=18) Q34349 43 49 Q3(43-4 n=17, n17 n=17 17 n=17) Q45166 51 Q4(51-6 n=15. n15 n=15 15 n=15) fatigue impairments p=0.001, p0001 p=0.001 001 (p=0.001) Q143.8%, Q1438 43.8%, 8 (Q1:43.8% Q233.3%, Q2333 33.3%, 33 3 Q2:33.3% Q311.8%, Q3118 11.8%, 11 Q3:11.8% Q46.7% Q467 6.7% 7 Q4:6.7% p=0.049 p0049 049 Q1100.0%, Q11000 100.0%, 100 [Q1:100.0% Q266.7%, Q2667 66.7%, Q2:66.7% Q358.8%, Q3588 58.8%, 58 Q3:58.8% Q446.7% Q4467 46.7% 46 Q4:46.7% p=0.043, p0043 p=0.043 043 (p=0.043) p=0.005 p0005 005 (p=0.005 posthoc post hoc analyses, analyses analyses] (Q p=0.003. p0003 p=0.003 003 p=0.003) addition damage symptoms agingrelated aging related factors (TAK p00 p<0.0 Q1223 2 Q1(22- n1 n=1 Q2374 4 Q2(37- Q3434 Q3(43- Q4516 5 Q4(51- p000 p=0.00 00 (p=0.001 Q143 Q143.8% 438 43.8% (Q1:43.8 Q233 Q233.3% 333 33.3% Q2:33.3 Q311 Q311.8% 118 11.8% Q3:11.8 Q46 Q46.7 67 6.7 Q4:6.7 p=0.04 p004 04 Q1100 Q1100.0% 1000 100.0% 10 [Q1:100.0 Q266 Q266.7% 667 66.7% Q2:66.7 Q358 Q358.8% 588 58.8% Q3:58.8 Q446 Q446.7 467 46.7 Q4:46.7 (p=0.043 (p=0.00 p0 p<0. Q122 Q1(22 n= Q237 Q2(37 Q343 Q3(43 Q451 Q4(51 p=0.0 Q14 Q143.8 43.8 (Q1:43. Q23 Q233.3 33.3 Q2:33. Q31 Q311.8 11.8 Q3:11. Q46. 6. Q4:6. Q110 Q1100.0 100.0 [Q1:100. Q26 Q266.7 66.7 Q2:66. Q35 Q358.8 58.8 Q3:58. Q44 Q446. 46. Q4:46. (p=0.04 (p=0.0 p<0 Q12 Q1(2 Q2(3 Q34 Q3(4 Q45 Q4(5 p=0. Q143. 43. (Q1:43 Q233. 33. Q2:33 Q311. 11. Q3:11 Q4:6 Q11 Q1100. 100. [Q1:100 Q266. 66. Q2:66 Q358. 58. Q3:58 Q4:46 (p=0. p< Q1( Q2( Q3( Q4( p=0 (Q1:4 Q2:3 Q3:1 Q4: [Q1:10 Q2:6 Q3:5 Q4:4 (p=0 p= (Q1: Q2: Q3: [Q1:1 (p= (Q1 [Q1: (p [Q1 [Q

BACKGROUND Trypanosoma cruzi shows an exuberant genetic diversity. Currently, seven phylogenetic lineages, called discrete typing units (DTUs), are recognised: TcI-TcVI and Tcbat. Despite advances in studies on T. cruzi and its populations, there is no consensus regarding its heterogeneity. OBJECTIVES This study aimed to perform molecular characterisation of T. cruzi strains, isolated in the state of São Paulo, to identify the DTUs involved and evaluate their genetic diversity. METHODS T. cruzi strains were isolated from biological samples of chronic chagasic patients, marsupials and triatomines through culture techniques and subjected to molecular characterisation using the fluorescent fragment length barcoding (FFLB) technique. Subsequently, the results were correlated with complementary information to enable better discrimination between the identified DTUs. FINDINGS It was possible to identify TcI in two humans and two triatomines; TcII/VI in 19 humans, two marsupials and one triatomine; and TcIII in one human host, an individual that also presented a result for TcI, which indicated the possibility of a mixed infection. Regarding the strains characterised by the TcII/VI profile, the correlation with complementary information allowed to suggest that, in general, these parasite populations indeed correspond to the TcII genotype. MAIN CONCLUSIONS The TcII/VI profile, associated with domestic cycles and patients with chronic Chagas disease, was the most prevalent among the identified DTUs. Furthermore, the correlation of the study results with complementary information made it possible to suggest that TcII is the predominant lineage of this work.

ABSTRACT OBJECTIVE To review articles that assessed work-related outcomes such as workability, work productivity, presenteeism, absenteeism, sick leave, return to work, and employment status of Brazilian patients with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögren’s syndrome, and systemic autoimmune myopathies. METHODS This study was conducted in Medline databases (PubMed), SciELO, and Lilacs through a combination of descriptors of interest. Studies published until December 2020 were considered in the search strategy. RESULTS Eight out of 90 articles met the eligibility criteria and were included in this review. The studies are highly heterogeneous. Most of them are cross-sectional, and all of them address rheumatoid arthritis or systemic lupus erythematosus. A common denominator among these studies is the high proportion of patients outside the labor market. CONCLUSIONS In general, the studies show unfavorable labor outcomes and impaired participation in the Brazilian workforce among the samples of patients assessed. There is a need to better understand several topics about Brazilian patients with systemic autoimmune diseases and their work context, as well as to conduct studies focusing on rarer diseases and on the themes of return and reintegration to work.

ABSTRACT OBJECTIVE To review articles that assessed work-related outcomes such as workability, work productivity, presenteeism, absenteeism, sick leave, return to work, and employment status of Brazilian patients with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögren’s syndrome, and systemic autoimmune myopathies. METHODS This study was conducted in Medline databases (PubMed), SciELO, and Lilacs through a combination of descriptors of interest. Studies published until December 2020 were considered in the search strategy. RESULTS Eight out of 90 articles met the eligibility criteria and were included in this review. The studies are highly heterogeneous. Most of them are cross-sectional, and all of them address rheumatoid arthritis or systemic lupus erythematosus. A common denominator among these studies is the high proportion of patients outside the labor market. CONCLUSIONS In general, the studies show unfavorable labor outcomes and impaired participation in the Brazilian workforce among the samples of patients assessed. There is a need to better understand several topics about Brazilian patients with systemic autoimmune diseases and their work context, as well as to conduct studies focusing on rarer diseases and on the themes of return and reintegration to work.

Abstract Background: Patients using immunosuppressive drugs may have unfavorable results after infections. However, there is a lack of information regarding COVID 19 in these patients, especially in patients with rheumatoid arthritis (RA). Therefore, the aim of this study was to evaluate the risk factors associated with COVID 19 hospitalizations in patients with RA. Methods: This multicenter, prospective cohort study is within the ReumaCoV Brazil registry and included 489 patients with RA. In this context, 269 patients who tested positive for COVID 19 were compared to 220 patients who tested negative for COVID 19 (control group). All patient data were collected from the Research Electronic Data Capture database. Results: The participants were predominantly female (90.6%) with a mean age of 53 ±12 years. Of the patients with COVID 19, 54 (20.1%) required hospitalization. After multiple adjustments, the final regression model showed that heart disease (OR =4.61, 95% CI 1.06–20.02. P < 0.001) and current use of glucocorticoids (OR =20.66, 95% CI 3.09–138. P < 0.002) were the risk factors associated with hospitalization. In addition, anosmia was associated with a lower chance of hospitalization (OR =0.26; 95% CI 0.10–0.67, P < 0.005). Conclusion: Our results demonstrated that heart disease and the use of glucocorticoids were associated with a higher number of hospital admissions for COVID 19 in patients with RA. Trial registration: Brazilian Registry of Clinical Trials RBR 33YTQC.

Abstract Background: Modifiable cardiovascular risk factors (MCRFs), such as those related to aerobic capacity, muscle strength, physical activity, and body composition, have been poorly studied in Takayasu arteritis (TAK). Therefore, the aim of the study was to investigate MCRFs and their relationships with disease status and comorbidities among patients with TAK. Methods: A multicenter cross-sectional study was conducted between 2019 and 2020, in which 20 adult women with TAK were compared with 16 healthy controls matched by gender, age, and body mass index. The following parameters were analyzed: aerobic capacity by cardiopulmonary test; muscle function by timed-stands test, timed up-and-go test, and handgrip test; muscle strength by one-repetition maximum test and handgrip test; body composition by densitometry; physical activity and metabolic equivalent by IPAQ, quality of life by HAQ and SF-36; disease activity by ITAS2010 and NIH score; and presence of comorbidities. Results: Patients with TAK had a mean age of 41.5 (38.0-46.3) years, disease duration of 16.0 (9.5-20.0) years, and a mean BMI of 27.7±4.5 kg/m2. Three out of the 20 patients with TAK had active disease. Regarding comorbidities, 16 patients had systemic arterial hypertension, 11 had dyslipidemia, and two had type 2 diabetes mellitus, while the control group had no comorbidities. TAK had a significant reduction in aerobic capacity (absolute and relative VO2 peak), muscle strength in the lower limbs, increased visceral adipose tissue, waist-to-hip ratio, reduced walking capacity, decreased weekly metabolic equivalent, and quality of life (P< 0.05) as compared to controls. However, there were no correlations between these MCRFs parameters and disease activity. Conclusions: TAK show impairment in MCRFs; therefore, strategies able to improve MCRF should be considered in this disease.

Abstract Background: To assess the prevalence and clinical relevance of anti-Jo-1 autoantibodies in a representative sample of patients with definite dermatomyositis (DM). Methods: This retrospective cohort study took place from 2005 to 2020 and assessed 118 adult patients from a tertiary center who were diagnosed with definite DM. A commercial kit was used to detect anti-Jo-1 autoantibodies. Results: The presence of anti-Jo-1 autoantibodies was observed in 10 out of 118 (8.5%) patients with definite DM. The following variables were comparable between individuals with and without anti-Jo-1 autoantibodies: age at diagnosis, sex, ethnicity, disease duration, follow-up period, recurrence rate, complete clinical response, death rate, and cancer incidence. There was no difference in clinical features between groups, except for an increased prevalence of “mechanic's hands,” joint involvement, and lung disease, as well as a reduced occurrence of skin findings in patients positive for anti-Jo-1 autoantibodies. No anti-Jo-1-positive patients went into remission; they required greater use of glucocorticoids and immunosuppressive drugs. Conclusions: Anti-Jo-1 positivity was found in 8.5% of patients with definite DM. This autoantibody was associated with an antisynthetase syndrome phenotype and might predict clinical outcomes in patients with definite DM.

Abstract Background: The protein chitinase-3-like-1 (YKL-40) is rarely analyzed in patients with myositis. Therefore, we aimed to evaluate YKL-40 serum levels; correlate them with laboratory and clinical parameters, disease status, and treatment schemes; and analyze the YKL-40 expression in the muscle tissues of patients with antisynthetase syndrome (ASSD). Methods: This cross-sectional single-center study included 64 adult patients with ASSD who were age-, gender-, and ethnicity-matched to 64 healthy control individuals. Their YKL-40 serum levels were analyzed using the Enzyme-Linked Immunosorbent Assay (ELISA) kit method, while YKL-40 expression in muscle tissues was analyzed using an immunohistochemical technique. Disease status was assessed using the International Myositis Assessment and Clinical Studies Group (IMACS) set scores. Results: The patients’ mean age was 44.8 ± 11.8 years, and median disease duration was 1.5 (0.0-4.0) years. These patients were predominantly female (82.8%) and Caucasian (73.4%). Most patients had stable disease. The median YKL-40 serum level was significantly higher in patients with ASSD when compared to the healthy individuals: 538.4 (363.4-853.1) pg/mL versus 270.0 (201.8-451.9) pg/mL, respectively; P < 0.001. However, YKL-40 serum levels did not correlate with any clinical, laboratory, disease status, or therapeutic parameters (P > 0.050), except tumor necrosis factor alpha (TNF-α) serum levels (Spearman’s correlation, rho = 0.382; P = 0.007). YKL-40 was highly expressed by inflammatory cells found in muscle biopsy specimens. Conclusions: High YKL-40 serum levels were observed in patients with ASSD and correlated positively with TNF-α serum levels. Moreover, YKL-40 was expressed by the inflammatory cells of the muscle tissue.

Abstract Background: To analyze the frequency and clinical relevance of anti-Mi-2 autoantibody in a representative sample of patients with dermatomyositis. Methods: This longitudinal inception cohort study, from 2001 to 2017, included 87 definite adult dermatomyositis. Anti-Mi-2 analysis was performed using a commercial kit. Results: Seventeen patients (19.5%) had anti-Mi-2 and 70 (80.5%) did not have this autoantibody. The following parameters were equally distributed between the patients with versus without anti-Mi-2: mean age at the disease diagnosis onset, median follow-up time, constitutional symptoms (baseline), cutaneous cumulative lesions, dysphagia, joint and pulmonary involvement. There was also no difference between the groups in relation to follow-up time, disease relapsing, treatment, disease status, deaths and occurrence of neoplasia. In contrast, patients with anti-Mi2 antibodies had higher frequency of elevated serum levels of muscle enzymes at disease onset (median: creatine phosphokinase 6240 [3800-9148] U/L and aldolase 60.0 [35.0-138.0] U/L), lower frequency of pulmonary involvement at disease onset (5.9%), less current glucocorticoid dose (median: 0 [0-10] mg/day), and higher frequency of disease remission during follow-up (58.8%) in comparison with patients without anti-Mi-2 autoantibody (484 [115-4880] and 12.1 [6.3-70.0] U/L, 40.0%, 0 [0-10] mg/day, 27.1%, respectively). Conclusion: The anti-Mi-2 autoantibody was found in one fifth of patients with dermatomyositis. This autoantibody was associated with a lower occurrence of pulmonary involvement, a higher frequency of disease in remission, and elevated levels of muscle enzymes. There was also no correlation regarding the frequency of disease relapsing or neoplasia development.

Abstract Background: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM). Main body: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Ten recommendations were developed addressing the management and therapy of systemic autoimmune myopathies. Conclusions: Robust data to guide the therapeutic process are scarce. Although not proven effective in controlled clinical trials, glucocorticoid represents first-line drugs in the treatment of SAM. Intravenous immunoglobulin is considered in induction for refractory cases of SAM or when immunosuppressive drugs are contra-indicated. Consideration should be given to the early introduction of immunosuppressive drugs. There is no specific period determined for the suspension of glucocorticoid and immunosuppressive drugs when individually evaluating patients with SAM. A key component for treatment in an early rehabilitation program is the inclusion of strengthbuilding and aerobic exercises, in addition to a rigorous evaluation of these activities for remission of disease and the education of the patient and his/her caregivers.

Abstract Background: This research is recommended by the Myopathy Committee of the Brazilian Society of Rheumatology for the investigation and diagnosis of systemic autoimmune myopathies. Body: A systematic literature review was performed in the Embase, Medline (PubMed) and Cochrane databases, including studies published until October 2018. PRISMA was used for the review, and the articles were evaluated, based on the Oxford levels of evidence. Ten recommendations were developed addressing different aspects of systemic autoimmune myopathy investigation and diagnosis. Conclusions: The European League Against Rheumatism/ American College of Rheumatology (EULAR/ACR) classification stands out for the diagnosis of systemic autoimmune myopathies. Muscular biopsy is essential, aided by muscular magnetic resonance images and electroneuromyography in complementary research. Analysis of the factors related to prognosis with the evaluation of extramuscular manifestations, and comorbidities and intense investigation regarding differential diagnoses are mandatory.

Abstract: Background: There have been no studies to date on the frequency and reactivity of aanti-melanoma differentiation-associated gene 5 (anti-MDA-5) in samples from the Brazilian population with dermatomyositis. Objectives: To analyze this autoantibody in the Brazilian population. Methods: This was a single-center cross-sectional study in which 131 consecutive adult patients (109 dermatomyositis and 22 clinically amyopathic dermatomyositis) with active disease were evaluated from 2000 to 2016. Analysis of the anti-MDA-5 autoantibody was performed by ELISA. Results: The presence of this autoantibody was observed in 14.7% and 22.7% of patients with dermatomyositis and clinically amyopathic dermatomyositis, respectively. In the case of dermatomyositis, the autoantibody was associated less frequently with Raynaud’s phenomenon and periungual hyperemia (P<0.05). In clinically amyopathic dermatomyositis, the presence of this autoantibody was not associated statistically with any demographic, clinical, laboratory, or imaging characteristics. Study limitations: The cross-sectional study design did not allow establishing a temporal correlation between anti-MDA-5 autoantibody and various study variables. In addition, pulmonary function tests were not performed in the patients. Conclusions: The frequency of anti-MDA-5 autoantibody was comparable to that of other populations with dermatomyositis, but with a different reactivity than described in the literature. In addition, there was a phenotypic variability between our patients with clinically amyopathic dermatomyositis and those described in the literature. Further studies are needed to confirm the current study’s findings and elucidate this autoantibody’s reactivity in Brazilians with idiopathic inflammatory myopathies.

Abstract: Background: There are scarce studies in the literature about hyaluronic acid in systemic autoimmune myopathies. Objectives: To analyze the serum level of hyaluronic acid in patients with dermatomyositis and polymyositis. Methods: Cross-sectional study, single-center, that evaluated hyaluronic acid in 18 dermatomyositis and 15 polymyositis (Bohan and Peter criteria), newly diagnosed, with clinical and laboratory activity, with no previous drug treatment. The patients were also age-, gender- and ethnicity-matched to 36 healthy individuals. The hyaluronic acid was analyzed by ELISA/EIA kit anti-hyaluronic acid. Results: There was a higher serum level of hyaluronic acid in patients with autoimmune myopathies, in relation to control group (P<0.05). Moreover, the serum level of this glycosaminoglycan was higher in dermatomyositis, when compared to polymyositis. Both groups were comparable with regard to demographic, clinical and laboratory data, except for the presence of skin lesions in the first group. Study limitations. The presence of hyaluronic acid in cutaneous lesions, particularly of patients with dermatomyositis, was not analyzed neither quantified. In addition, due to disease rarity and the establishment of strict inclusion and exclusion criteria, there was a small sample in the present study. Conclusions: As an example of others systemic autoimmune diseases, it is possible that the hyaluronic acid is involved in the pathogenesis of autoimmune myopathies, and particularly when associated with cutaneous lesions.