Unraveling the genetic collagen connection: clinical and therapeutic insights on genetic connective tissue disorders.

Hereditary connective tissue disorders include more than 200 conditions affecting different organs and tissues, compromising the biological role of the extracellular matrix through interference in the synthesis, development, or secretion of collagen and/or its associated proteins. The clinical phenotype includes multiple signs and symptoms, usually nonspecific but of interest to rheumatologists because of musculoskeletal involvement. The patient´s journey to diagnosis is long, and physicians should include these disorders in their differential diagnoses of diseases with systemic involvement. In this review, insights for the diagnosis and treatment of osteogenesis imperfecta, hypermobility spectrum disorder/Ehlers-Danlos syndrome, Marfan, Loeys-Dietz, and Stickler syndromes are presented.

Epidemiological analysis of patients with psoriatic arthritis in follow-up at the brazilian Unified Health System.

INTRODUCTION/OBJECTIVES: Psoriatic arthritis (PsA) is a chronic multisystem osteoarticular disease that requires specialized care. Most Brazilians depend on the public healthcare provided by the Unified Health System (Sistema Único de Saúde, SUS). This study aimed to describe the epidemiological characteristics of patients with PsA in follow-up in SUS, focusing on the incidence and prevalence of the disease, comorbidities, and hospitalizations. METHODS: We collected data from the Outpatient Data System of SUS (Sistema de Informações Ambulatoriais do SUS, SIA/SUS) regarding outpatient visits and hospitalizations in the Brazilian public healthcare system from January 2008 to March 2021 using the Techtrials Disease Explorer® platform and the medical code related to PsA were selected. RESULTS: We evaluated 40,009 patients and found a prevalence of 24.4 cases of visits due to PsA per 100,000 patients in follow-up in SUS. Female patients were predominant (54.38%). The incidence of visits due to PsA has been increasing in recent years and we observed an incidence of 8,982 new visits in 2020. The main comorbidities of these patients were osteoarthritis, lower back pain, shoulder injuries, oncological diseases, crystal arthropathies, and osteoporosis. Hospitalizations were mainly due to treating clinical or cardiovascular conditions and performing orthopedic procedures. CONCLUSION: The number of visits due to PsA in SUS has increased in recent years, mainly on account of new diagnoses of the disease, although the prevalence found in this study's population was lower than that observed in the general population.

Epidemiological analysis of patients with psoriatic arthritis in follow-up at the brazilian Unified Health System

Abstract Introduction/Objectives Psoriatic arthritis (PsA) is a chronic multisystem osteoarticular disease that requires specialized care. Most Brazilians depend on the public healthcare provided by the Unified Health System (Sistema Único de Saúde, SUS). This study aimed to describe the epidemiological characteristics of patients with PsA in follow-up in SUS, focusing on the incidence and prevalence of the disease, comorbidities, and hospitalizations. Methods We collected data from the Outpatient Data System of SUS (Sistema de Informações Ambulatoriais do SUS, SIA/SUS) regarding outpatient visits and hospitalizations in the Brazilian public healthcare system from January 2008 to March 2021 using the Techtrials Disease Explorer® platform and the medical code related to PsA were selected. Results We evaluated 40,009 patients and found a prevalence of 24.4 cases of visits due to PsA per 100,000 patients in follow-up in SUS. Female patients were predominant (54.38%). The incidence of visits due to PsA has been increasing in recent years and we observed an incidence of 8,982 new visits in 2020. The main comorbidities of these patients were osteoarthritis, lower back pain, shoulder injuries, oncological diseases, crystal arthropathies, and osteoporosis. Hospitalizations were mainly due to treating clinical or cardiovascular conditions and performing orthopedic procedures. Conclusion The number of visits due to PsA in SUS has increased in recent years, mainly on account of new diagnoses of the disease, although the prevalence found in this study's population was lower than that observed in the general population.

Brazilian Society of Rheumatology 2020 guidelines for psoriatic arthritis.

Psoriatic arthritis (PsA) is a chronic and systemic immune disease characterized by inflammation of peripheral and/or axial joints and entheses in patients with psoriasis (PsO). Extra-articular and extracutaneous manifestations and numerous comorbidities can also be present. These recommendations replace the previous version published in May 2013. A systematic review of the literature retrieved 191 articles that were used to formulate 12 recommendations in response to 12 clinical questions, divided into 4 sections: diagnosis, non-pharmacological treatment, conventional drug therapy and biologic therapy. These guidelines provide evidence-based information on the clinical management for PsA patients. For each recommendation, the level of evidence (highest available), degree of strength (Oxford) and degree of expert agreement (interrater reliability) are reported.

Biosimilar-to-Biosimilar Switching: What is the Rationale and Current Experience?

Over time, clinicians have become increasingly comfortable embracing the prescription of biosimilars-highly similar versions of innovator or reference biological agents-for their patients with inflammatory diseases. Although a switch from a reference product to a licensed biosimilar version (or vice versa) is a medical decision robustly supported by the stepwise accumulation of clinical trial evidence concerning comparable safety, immunogenicity, and efficacy between these products, a switch from one biosimilar to another biosimilar of the same reference product, or a cross-switch, is not. Similarity among biosimilars of a reference product is not a regulatory agency concern and therefore is unlikely to be investigated in randomized controlled trials in the foreseeable future. Yet in clinical practice, across a diverse range of patients, the option to cross-switch from one biosimilar to another can and does arise for valid reasons such as convenience or tolerability issues, or driven by third parties (e.g., payers). In the absence of clinical trial data, clinicians must attempt to objectively evaluate the emerging real-world cross-switching evidence within the context of what is known about the science underpinning a designation of biosimilar. That knowledge then needs to be integrated with what clinicians know about their patients and their disease on a case-by-case basis. This review aims to consolidate relevant emerging real-world data and other key information about biosimilar-to-biosimilar cross-switching for prescribing clinicians. In the absence of clear clinical guidelines addressing this topic at present, this review may serve to facilitate discretionary and educated treatment decision making.

The landscape of systemic lupus erythematosus in Brazil: An expert panel review and recommendations.

PURPOSE: The objective of this review is to address the barriers limiting access to diagnosis and treatment of systemic lupus erythematosus (SLE) and lupus nephritis (LN) in Brazil, specifically for patients in the public healthcare system, arguably those with the least access to innovation. DESIGN: A selected panel of Brazilian experts in SLE/LN were provided with a series of relevant questions to address in a multi-day conference. During the conference, responses were discussed and edited by the entire group through numerous drafts and rounds of discussion until a consensus was achieved. RESULTS: The authors propose specific and realistic recommendations for implementing access to innovative diagnostic tools and treatment alternatives for SLE/LN in Brazil. Moreover, in creating these recommendations, the authors strived to address barriers and impediments for technology adoption. The multidisciplinary care required for SLE/LN necessitates the collective participation of all involved stakeholders. CONCLUSION: A great need exists to expand the adoption of innovative diagnostic tools and treatments for SLE/LN not only in Brazil but also in most countries, as access issues remain an urgent demand. The recommendations presented in this article can serve as a strategy for new technology adoption in other countries in a similar situation.

High levels of immunosuppression are related to unfavourable outcomes in hospitalised patients with rheumatic diseases and COVID-19: first results of ReumaCoV Brasil registry.

OBJECTIVES: To evaluate risk factors associated with unfavourable outcomes: emergency care, hospitalisation, admission to intensive care unit (ICU), mechanical ventilation and death in patients with immune-mediated rheumatic disease (IMRD) and COVID-19. METHODS: Analysis of the first 8 weeks of observational multicentre prospective cohort study (ReumaCoV Brasil register). Patients with IMRD and COVID-19 according to the Ministry of Health criteria were classified as eligible for the study. RESULTS: 334 participants were enrolled, a majority of them women, with a median age of 45 years; systemic lupus erythematosus (32.9%) was the most frequent IMRD. Emergency care was required in 160 patients, 33.0% were hospitalised, 15.0% were admitted to the ICU and 10.5% underwent mechanical ventilation; 28 patients (8.4%) died. In the multivariate adjustment model for emergency care, diabetes (prevalence ratio, PR 1.38; 95% CI 1.11 to 1.73; p=0.004), kidney disease (PR 1.36; 95% CI 1.05 to 1.77; p=0.020), oral glucocorticoids (GC) (PR 1.49; 95% CI 1.21 to 1.85; p<0.001) and pulse therapy with methylprednisolone (PR 1.38; 95% CI 1.14 to 1.67; p=0.001) remained significant; for hospitalisation, age >50 years (PR 1.89; 95% CI 1.26 to 2.85; p=0.002), no use of tumour necrosis factor inhibitor (TNFi) (PR 2.51;95% CI 1.16 to 5.45; p=0.004) and methylprednisolone pulse therapy (PR 2.50; 95% CI 1.59 to 3.92; p<0.001); for ICU admission, oral GC (PR 2.24; 95% CI 1.36 to 3.71; p<0.001) and pulse therapy with methylprednisolone (PR 1.65; 95% CI 1.00 to 2.68; p<0.043); the two variables associated with death were pulse therapy with methylprednisolone or cyclophosphamide (PR 2.86; 95% CI 1.59 to 5.14; p<0.018). CONCLUSIONS: Age >50 years and immunosuppression with GC and cyclophosphamide were associated with unfavourable outcomes of COVID-19. Treatment with TNFi may have been protective, perhaps leading to the COVID-19 inflammatory process.

Brazilian recommendations for the use of nonsteroidal anti-inflammatory drugs in patients with axial spondyloarthritis.

Spondyloarthritis (SpA) is a group of chronic inflammatory systemic diseases characterized by axial and/or peripheral joints inflammation, as well as extra-articular manifestations. Over some decades, nonsteroidal anti-inflammatory drugs (NSAIDs) have been the basis for the pharmacological treatment of patients with axial spondyloarthritis (axSpA). However, the emergence of the immunobiologic agents brought up the discussion about the role of NSAIDs in the management of these patients. The objective of this guideline is to provide recommendations for the use of NSAIDs for the treatment of axSpA. A panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis of randomized clinical trials for 15 predefined questions. The Grading of Recommendations, Assessment, Development and Evaluation methodology to assess the quality of evidence and formulate recommendations were used, and at least 70% agreement of the voting panel was needed. Fourteen recommendations for the use of NSAIDs in the treatment of patients with axSpA were elaborated. The purpose of these recommendations is to support clinicians' decision making, without taking out his/her autonomy when prescribing for an individual patient.

Brazilian recommendations for the use of nonsteroidal anti-inflammatory drugs in patients with axial spondyloarthritis

Abstract Spondyloarthritis (SpA) is a group of chronic inflammatory systemic diseases characterized by axial and/or peripheral joints inflammation, as well as extra-articular manifestations. Over some decades, nonsteroidal anti-inflammatory drugs (NSAIDs) have been the basis for the pharmacological treatment of patients with axial spondyloarthritis (axSpA). However, the emergence of the immunobiologic agents brought up the discussion about the role of NSAIDs in the management of these patients. The objective of this guideline is to provide recommendations for the use of NSAIDs for the treatment of axSpA. A panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis of randomized clinical trials for 15 predefined questions. The Grading of Recommendations, Assessment, Development and Evaluation methodology to assess the quality of evidence and formulate recommendations were used, and at least 70% agreement of the voting panel was needed. Fourteen recommendations for the use of NSAIDs in the treatment of patients with axSpA were elaborated. The purpose of these recommendations is to support clinicians' decision making, without taking out his/her autonomy when prescribing for an individual patient.(AU)

The musculoskeletal impairment negatively impacts the quality of life of children and adolescents with psoriasis.

BACKGROUND: The severity of nail disease, the presence of arthralgia and fatigue are predictors of development of psoriatic arthritis (PsA) in patients with psoriasis (Pso). In children, little is known about the musculoskeletal (MSK) impairment in patients with Pso and its effect on health-related quality of life (HRQoL). OBJECTIVES: To determine the frequencies of pain and MSK inflammation (i.e., arthritis, enthesitis, and sacroiliitis) among children and adolescents with Pso and its relationship to HRQoL and fatigue. METHODS: Pediatric patients with Pso underwent a rheumatologic physical examination to evaluate synovitis, enthesalgia, sacroiliac joint (SIJ) pain and tender points of fibromyalgia. The core set of domains recommended by the GRAPPA - OMERACT to be measured in PsA studies was assessed. Ultrasound (US) was performed in clinical cases of enthesitis, and magnetic resonance imaging (MRI) was performed in cases of SIJ pain. RESULTS: Forty-three participants (10 ± 2.9 years old) were evaluated. Pain on palpation of the entheses was observed in 10 (23.2%) patients and pain on SIJ palpation was observed in 3 (7%). No patient presented with synovitis; one presented with enthesitis on US, but MRI did not confirm sacroiliitis in any case. Patients with MSK pain had greater skin disease severity (PASI 5.4 vs. 2, p <  0.01), worse fatigue, and lower HRQoL scores on all instruments used. The estimated risk of HRQoL impairment was eight times higher in the presence of MSK pain, which was an independent predictive factor. With a NAPSI greater than 30, the probability of pain was greater than 80%. CONCLUSION: MSK pain is frequent among children with Pso, related to the severity of skin and nail disease, and negatively affects HRQoL. The typically used complementary exams might not detect the inflammatory process caused by Pso.

Home storage of biological medications administered to patients with rheumatic diseases.

BACKGROUND: The inadequate storage of biopharmaceuticals may result in an ineffective therapeutic response since poor conservation can lead to the emergence of protein aggregates and cause immunogenicity in patients, which can increase the risk of adverse events by inducing the production of anti-drug antibodies. This can also lead to significant economic losses for public health, given the high cost of these medicines. The aim of this study was to verify whether the home storage of biopharmaceuticals dispensed by the Unified Public System was in accordance with the manufacturers' specified standards and whether external variables interfered with the correct home storage. METHODS: This was a prospective observational study. Patients with a confirmed diagnosis of rheumatoid arthritis, ankylosing spondylitis or psoriatic arthritis who were using a biologic exclusively dispensed by Unified Public System were included. Storage temperature was measured by digital thermometer inserted into the refrigerator of the participant's home. Fisher's exact test was performed to cross-reference the temperature data and the qualitative variables obtained using an epidemiologic questionnaire. Mean, minimum, maximum values and standard deviation were described in the quantitative data. Mann-Whitney non-parametric test was performed to the association between temperature excursion and the number of people in the house. RESULTS: A total of 81 participants were included and 67 (82.71%) did not maintain home storage correctly. The maximum temperature observed among all patients was 15.5 °C, the minimum was - 4.4 °C and the average was 5.6 °C (standard deviation 2.8); 10 (12.3%) had at least one negative temperature measured. The average time for participants who had an inadequate temperature record was 8 h and 31 min. Nine participants (90%) who stored the medication into the shelf/drawer below the freezer had a temperature excursion (p = 0.011). Most of the participants (88.5%) who stored their biopharmaceutical near the back side, close to the wall of the refrigerator had a negative temperature record (p < 0.001). CONCLUSION: Most of the study participants (82.71%) did not maintain adequate home storage conditions for their biopharmaceutical. Intrinsic factors of household refrigerators may be involved in temperature deviations.

The Brazilian Society of Rheumatology guidelines for axial spondyloarthritis - 2019.

Spondyloarthritis is a group of chronic inflammatory systemic diseases characterized by axial and/or peripheral joints inflammation, as well as extra-articular manifestations. The classification axial spondyloarthritis is adopted when the spine and/or the sacroiliac joints are predominantly involved. This version of recommendations replaces the previous guidelines published in May 2013.A systematic literature review was performed, and two hundred thirty-seven studies were selected and used to formulate 29 recommendations answering 15 clinical questions, which were divided into four sections: diagnosis, non-pharmacological therapy, conventional drug therapy and biological therapy. For each recommendation the level of evidence supporting (highest available), the strength grade according to Oxford, and the degree of expert agreement (inter-rater reliability) is informed.These guidelines bring evidence-based information on clinical management of axial SpA patients, including, diagnosis, treatment, and prognosis.

The Brazilian Society of Rheumatology guidelines for axial spondyloarthritis - 2019

Abstract Spondyloarthritis is a group of chronic inflammatory systemic diseases characterized by axial and/or peripheral joints inflammation, as well as extra-articular manifestations. The classification axial spondyloarthritis is adopted when the spine and/or the sacroiliac joints are predominantly involved. This version of recommendations replaces the previous guidelines published in May 2013. A systematic literature review was performed, and two hundred thirty-seven studies were selected and used to formulate 29 recommendations answering 15 clinical questions, which were divided into four sections: diagnosis, non-pharmacological therapy, conventional drug therapy and biological therapy. For each recommendation the level of evidence supporting (highest available), the strength grade according to Oxford, and the degree of expert agreement (inter-rater reliability) is informed. These guidelines bring evidence-based information on clinical management of axial SpA patients, including, diagnosis, treatment, and prognosis.

The musculoskeletal impairment negatively impacts the quality of life of children and adolescents with psoriasis

Abstract Background The severity of nail disease, the presence of arthralgia and fatigue are predictors of development of psoriatic arthritis (PsA) in patients with psoriasis (Pso). In children, little is known about the musculoskeletal (MSK) impairment in patients with Pso and its effect on health-related quality of life (HRQoL). Objectives To determine the frequencies of pain and MSK inflammation (i.e., arthritis, enthesitis, and sacroiliitis) among children and adolescents with Pso and its relationship to HRQoL and fatigue. Methods Pediatric patients with Pso underwent a rheumatologic physical examination to evaluate synovitis, enthesalgia, sacroiliac joint (SIJ) pain and tender points of fibromyalgia. The core set of domains recommended by the GRAPPA - OMERACT to be measured in PsA studies was assessed. Ultrasound (US) was performed in clinical cases of enthesitis, and magnetic resonance imaging (MRI) was performed in cases of SIJ pain. Results Forty-three participants (10 ± 2.9 years old) were evaluated. Pain on palpation of the entheses was observed in 10 (23.2%) patients and pain on SIJ palpation was observed in 3 (7%). No patient presented with synovitis; one presented with enthesitis on US, but MRI did not confirm sacroiliitis in any case. Patients with MSK pain had greater skin disease severity (PASI 5.4 vs. 2, p < 0.01), worse fatigue, and lower HRQoL scores on all instruments used. The estimated risk of HRQoL impairment was eight times higher in the presence of MSK pain, which was an independent predictive factor. With a NAPSI greater than 30, the probability of pain was greater than 80%. Conclusion MSK pain is frequent among children with Pso, related to the severity of skin and nail disease, and negatively affects HRQoL. The typically used complementary exams might not detect the inflammatory process caused by Pso.(AU)

Translation and validation of the Indian Takayasu clinical activity score (ITAS2010) for the Brazilian Portuguese language.

BACKGROUND: The Indian Takayasu Clinical Activity Score (ITAS2010) was developed in 2010 as an assessment tool for disease activity in patients with Takayasu arteritis (TA). It has since been widely used in different studies and in clinical practice for the management of patients with TA. The present study aims to translate the ITAS2010 into Brazilian Portuguese language and to validate it for use in clinical practice in Brazil. METHODS: For this cross-sectional study, the ITAS2010 was translated in accordance with the guidelines described by Beaton et al. and then applied with 27 patients with TA on three assessments by two rheumatologists working independently. To measure interrater agreement, the assessments were performed on the same day within approximately 1 hour. One of the rheumatologists performed a second evaluation of patients with TA within 7 to 14 days to measure intrarater agreement. RESULTS: The correlation coefficient for the ITAS2010 score between the two raters was high (r = 0.916; p < 0.0001), as well as the intraclass correlation coefficient (ICC) [0.918 with a 95% confidence interval (95CI): 0.828-0.962]. The correlation coefficient and the ICC for intrarater agreement were moderate for ITAS2010 (r = 0.633; p < 0.0001 and ICC = 0.594; 95CI: 0.292-0.790). The ITAS2010 at baseline was compared with the physician's global assessment (PGA) and with Kerr's criteria for detecting disease activity in TA. Higher ITAS2010 scores were observed in patients with active and grumbling/persistent disease than in those presenting inactive disease according to the PGA [1.5 (0.0-3.0) vs. 0.0 (0.0-0.0); p = 0.0025]. Patients with active disease according to the Kerr's criteria had also higher ITAS2010 scores than those considered in remission [3.0 (3.0-7.0) vs. 0.0 (0.0-0.0); p = 0.0068]. CONCLUSIONS: The Brazilian Portuguese version of the ITAS2010 is a valid and reproducible tool for the assessment of disease activity in TA and it is an additional tool for the routine evaluation of Brazilian patients with TA.

Benzbromarone in the treatment of gout.

BACKGROUND: Benzbromarone is a uricosuric drug that has been used in the treatment of gout over the last 30 years. Due to its potent inhibition of the dominant apical (luminal) urate exchanger in the human proximal tubule URAT1, it reduces the urate reabsorption, diminishing serum urate levels and therefore preventing gout flares. Through several clinical trials, Benzbromarone has been proved effective and safe, inclusive in patients with chronic kidney disease and as combination therapy with allopurinol. Due to hepatotoxicity reports, it was withdrawn from the European market by the manufacturer, however many authors have questioned the product's withdrawal due to a lack of clinical evidence in order to support its hepatotoxicity. Benzbromarone is still available in several European countries, New Zealand, Brazil and several other countries. Despite the product's marketing over more than 20 years after the first hepatotoxicity reports, we have found only five reports in our literature search, and no prospective or retrospective study correlating hepatotoxicity with benzbromarone use. SHORT CONCLUSION: Benzbromarone is a safe and effective molecule for the treatment of gout. However, due to in vitro and in vivo data related to hepatotoxicity, it is prudent to prescribe it with some caution, especially for patients with an already known liver condition.

Practical Guidance on Biosimilars, With a Focus on Latin America: What Do Rheumatologists Need to Know?

BACKGROUND/HISTORICAL PERSPECTIVE: Availability of biologic disease-modifying antirheumatic drugs (bDMARDs) has improved clinical outcomes in rheumatoid arthritis, but it also increased the cost of treatment. Biosimilars, the regulated copies of biologic products, have a potential to reduce health care costs and expand access to treatment. However, because of a complex development process, biosimilars can be considered only those noninnovator biologics with satisfactory supporting evidence (ranging from structural to clinical), as outlined in the recommendations by the World Health Organization (WHO). In Latin America, a heterogeneous regulatory landscape and nonconsistent approval practices for biosimilars create decision-making challenges for practicing rheumatologists. SUMMARY OF LITERATURE: Most Latin American countries either have adopted or are in the process of adopting guidelines for the approval of biosimilars. However, among several marketed bDMARDs in the region, currently there are only 2 products that could be considered true biosimilars, based on the WHO criteria. The rest can be considered only intended copies, whose safety and efficacy are not fully established. One such product had to be withdrawn from the market because of safety concerns. CONCLUSIONS AND FUTURE DIRECTIONS: Practicing rheumatologists in Latin America need to understand the regulatory situation for biosimilars in their countries. When considering bDMARDs that are not innovator products, clinicians should use only those that have been approved according to the WHO recommendations. For clarification, local health authorities or professional associations should be contacted.

Benzbromarone in the treatment of gout

Abstract Background Benzbromarone is a uricosuric drug that has been used in the treatment of gout over the last 30 years. Due to its potent inhibition of the dominant apical (luminal) urate exchanger in the human proximal tubule URAT1, it reduces the urate reabsorption, diminishing serum urate levels and therefore preventing gout flares. Main body of the abstract Through several clinical trials, Benzbromarone has been proved effective and safe, inclusive in patients with chronic kidney disease and as combination therapy with allopurinol. Due to hepatotoxicity reports, it was withdrawn from the European market by the manufacturer, however many authors have questioned the product's withdrawal due to a lack of clinical evidence in order to support its hepatotoxicity. Benzbromarone is still available in several European countries, New Zealand, Brazil and several other countries. Despite the product's marketing over more than 20 years after the first hepatotoxicity reports, we have found only five reports in our literature search, and no prospective or retrospective study correlating hepatotoxicity with benzbromarone use. Short conclusion Benzbromarone is a safe and effective molecule for the treatment of gout. However, due to in vitro and in vivo data related to hepatotoxicity, it is prudent to prescribe it with some caution, especially for patients with an already known liver condition.

Translation and validation of the Indian Takayasu clinical activity score (ITAS2010) for the Brazilian Portuguese language

Abstract Background: The Indian Takayasu Clinical Activity Score (ITAS2010) was developed in 2010 as an assessment tool for disease activity in patients with Takayasu arteritis (TA). It has since been widely used in different studies and in clinical practice for the management of patients with TA. The present study aims to translate the ITAS2010 into Brazilian Portuguese language and to validate it for use in clinical practice in Brazil. Methods: For this cross-sectional study, the ITAS2010 was translated in accordance with the guidelines described by Beaton et al. and then applied with 27 patients with TA on three assessments by two rheumatologists working independently. To measure interrater agreement, the assessments were performed on the same day within approximately 1 hour. One of the rheumatologists performed a second evaluation of patients with TA within 7 to 14 days to measure intrarater agreement. Results: The correlation coefficient for the ITAS2010 score between the two raters was high (r =0.916; p < 0.0001), as well as the intraclass correlation coefficient (ICC) [0.918 with a 95% confidence interval (95CI): 0.828-0.962]. The correlation coefficient and the ICC for intrarater agreement were moderate for ITAS2010 (r =0.633; p < 0.0001 and ICC = 0.594; 95CI: 0.292-0.790). The ITAS2010 at baseline was compared with the physician's global assessment (PGA) and with Kerr's criteria for detecting disease activity in TA. Higher ITAS2010 scores were observed in patients with active and grumbling/persistent disease than in those presenting inactive disease according to the PGA [1.5 (0.0-3.0) vs. 0.0 (0.0-0.0); p = 0.0025]. Patients with active disease according to the Kerr's criteria had also higher ITAS2010 scores than those considered in remission [3.0 (3.0-7.0) vs. 0.0 (0.0-0.0); p = 0.0068]. Conclusions: The Brazilian Portuguese version of the ITAS2010 is a valid and reproducible tool for the assessment of disease activity in TA and it is an additional tool for the routine evaluation of Brazilian patients with TA.