An adult with cystathionine beta-synthase deficiency, camptodactyly-arthropathy-coxa vara-pericarditis syndrome, and deafness: A case report.

Massive sequencing platforms allow the identification of complex clinical phenotypes involving more than one autosomal recessive disorder. In this study, we report on an adult patient, born to a related couple (third degree cousins), referred for genetic evaluation due to ectopia lentis, deafness and previous diagnosis of juvenile idiopathic arthritis. He was biochemically diagnosed as having Classic Homocystinuria (HCU); Sanger sequencing of the CBS gene showed the genotype NM_000071.2(CBS):c.[833T>C];[833T>C], compatible with the diagnosis of pyridoxine-responsive HCU. As he also had symptoms not usually associated with HCU, exome sequencing was performed. In addition to the variants found in the Sanger sequencing, the following variants were identified: NM_001256317.1(TMPRSS3):c.[413C>A];[413C>A]; and the NM_005807.6(PRG4):c.[3756dup]:[3756dup], confirming the diagnosis of autosomal recessive nonsyndromic deafness and Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome (CACP), respectively. Genomic analysis allowed the refinement of the diagnosis of a complex case and improvement of the patient's treatment.

Comparative study of two laboratory techniques for the detection of HLA-B27 in patients with axial spondyloarthritis: a cross-sectional analysis.

BACKGROUND: The diagnostic and prognostic relevance of Human Leukocyte Antigen B-27 (HLA-B27) in Axial Spondyloarthritis (AxSpA) is undeniable, with 70% of Ankylosing Spondylitis (AS) patients carrying the B27 gene, contrasted with a mere 4.35% in the general population. Flow cytometry (FC) and Polymerase Chain Reaction (PCR) have emerged as the predominant techniques for routine HLA-B27 typing. While various studies have compared these methods, none have catered to the unique characteristics of the Brazilian demographic. Therefore, this research aims to compare FC and PCR in a Brazilian cohort diagnosed with AxSpA. METHODS: An analytical cross-sectional study was undertaken involving 62 AxSpA outpatients from a Brazilian University Hospital. Both FC and PCR-SSP assays were utilized to ascertain HLA-B27 typing. The outcomes (either confirming or refuting the allele's presence) underwent rigorous scrutiny. Agreement between the methodologies was assessed using the kappa statistic. A p-value of < 0.05 was deemed statistically significant. RESULTS: Of the participants, 90.3% (n = 56) were HLA-B27 positive according to FC, while 79% (n = 49) were identified as positive using the PCR method. FC exhibited a sensitivity rate of 98% paired with a specificity of 38.5%. The Positive Predictive Value for FC stood at 85.7%, and the Negative Predictive Value was 83.5%. Consequently, the overall accuracy of the FC method was gauged at 85.5%. A kappa coefficient of κ = 0.454 was derived. CONCLUSIONS: FC demonstrated noteworthy sensitivity and satisfactory accuracy in HLA-B27 detection, albeit with a reduced specificity when contrasted with PCR-SSP. Nevertheless, given its cost-effectiveness and streamlined operation relative to PCR, FC remains a pragmatic option for preliminary screening in clinical practice, especially in low-income regions. To optimize resource allocation, we advocate for a refined algorithm that initiates by assessing the relevance of HLA-B27 typing based on Choosing Wisely recommendations. It then leans on FC, and, if results are negative yet clinical suspicion persists, advances to PCR. This approach aims to balance diagnostic accuracy and financial prudence, particularly in regions contending with escalating medical costs.

Patients with longstanding pPatients with longstanding psoriatic arthritis can achieve DAPSA remission or low disease activity and it correlates to better functional outcomes: results from a Latin-American real-life cohort.

BACKGROUND: Patients with psoriatic arthritis (PsA) experience reduced physical function and impaired quality of life. Better patient-reported functional outcomes are found when lower disease activity is achieved. OBJECTIVES: To evaluate the variation of physical function by HAQ-DI over time in PsA patients treated with standard therapy in a real-life setting: to verify predictors of achieving a minimum clinically important difference (MCID) in function by HAQ-DI (ΔHAQ-DI ≤ - 0.35) and to measure the impact of achieving REM/LDA on long-term function by HAQ-DI. METHODS: This is a longitudinal analysis of a real-life retrospective cohort. Data from PsA patients with at least 4 years of follow-up in the PsA clinic from 2011 to 2019 were extracted from electronic medical records. The variations of physical function by HAQ-DI and disease activity by DAPSA over time were calculated. A multivariate hierarchical regression model was applied to verify predictors of MCID in HAQ-DI. A comparison of HAQ-DI variation between patients with DAPSA REM, LDA, moderate and high disease activity was made using the generalized estimating equation model (GEE), adjusted by Bonferroni test. The Spearman correlation method was applied to verify the correlation of ΔDAPSA and ΔHAQ-DI over time. Statistical analysis was performed in SPSS program version 21.0. RESULTS: Seventy-three patients were included in the analysis. Physical function measured by HAQ-DI was determined by PsA disease activity measured by DAPSA (p < 0.000). A moderate and statistically significant correlation between ΔDAPSA and ΔHAQ-DI was observed (rs = 0.60; p < 0.001). Only patients in DAPSA REM demonstrated a constant decline in HAQ-DI scores during the follow-up. White ethnicity and older age at baseline were predictors for not achieving MCID in HAQ-DI [RR 0.33 (0.16-0.6795% CI p = 0.002) and RR 0.96 (0.93-0.9895% CI p < 0.000), respectively, while higher scores of HAQ-DI at baseline were predictors of achieving MCID [RR 1.71 (1.12-2.6095%CI p = 0.013)]. CONCLUSIONS: In PsA, patients who maintained DAPSA REM/LDA over time had better long-term functional outcomes. Higher HAQ-DI scores at baseline, non-white ethnicity and younger age were predictors for achieving a clinical meaningful improvement of HAQ-DI.

To be or not to B27 positive: implications for the phenotypes of axial spondyloarthritis outcomes. Data from a large multiracial cohort from the Brazilian Registry of Spondyloarthritis.

BACKGROUND: There is a remarkable variability in the frequency of HLA-B27 positivity in patients with spondyloarthritis (SpA), which may be associated with different clinical presentations worldwide. However, there is a lack of data considering ethnicity and sex on the evaluation of the main clinical and prognostic outcomes in mixed-race populations. The aim of this study was to evaluate the frequency of HLA-B27 and its correlation with disease parameters in a large population of patients from the Brazilian Registry of Spondyloarthritis (RBE). METHODS: The RBE is a multicenter, observational, prospective cohort that enrolled patients with SpA from 46 centers representing all five geographic regions of Brazil. The inclusion criteria were as follow: (1) diagnosis of axSpA by an expert rheumatologist; (2) age ≥18 years; (3) classification according to ASAS axial. The following data were collected via a standardized protocol: demographic data, disease parameters and treatment historical. RESULTS: A total of 1096 patients were included, with 73.4% HLA-B27 positivity and a mean age of 44.4 (±13.2) years. Positive HLA-B27 was significantly associated with male sex, earlier age at disease onset and diagnosis, uveitis, and family history of SpA. Conversely, negative HLA-B27 was associated with psoriasis, higher peripheral involvement and disease activity, worse quality of life and mobility. CONCLUSIONS: Our data showed that HLA-B27 positivity was associated with a classic axSpA pattern quite similar to that of Caucasian axSpA patients around the world. Furthermore, its absence was associated with peripheral manifestations and worse outcomes, suggesting a relevant phenotypic difference in a highly miscegenated population.

Challenges in the Management of Psoriatic Arthritis in Latin America: A Systematic Review.

PURPOSE: In 2020, the International League of Associations for Rheumatology published recommendations for managing psoriatic arthritis (PsA), aiming to adapt the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis and the European Alliance of Associations for Rheumatology recommendations to low-income countries. At that time, the paucity of clinical studies examining the management of patients with PsA in Latin America was remarked on by the international working group. Therefore, the primary objective of this systematic literature review was to investigate the main challenges in managing PsA in Latin America as described in recent publications. METHODS: A systematic literature review of trials reporting at least one challenge/difficulty in the management of PsA in Latin America was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. References published in the PubMed, EMBASE, and LILACS (Latin American and Caribbean Health Sciences Literature) databases between 1980 and February 2023 were included. The selection of references was conducted independently by 2 researchers in the Rayyan Qatar Computing Research Institute program. Two other reviewers independently extracted data. All challenges were noted and categorized into domains. Data analysis was descriptive. FINDINGS: The search strategy yielded 2085 references, with 21 studies included in the final analysis. Most studies were conducted in Brazil (66.6%; n = 14) and were observational studies (100%; N = 21). Difficulties faced by PsA patients and physicians included the high incidence of opportunistic infections (described in 42.8% of the publications; n = 9), followed by nonadherence to therapy, discordance between patients and physicians regarding remission rates, low drug persistence, limited access to disease-modifying antirheumatic drugs, issues related to the storage of biologic drugs, elevated cost of biologic drugs, limited access to medical care, diagnostic delay, and the individual- and country-level impact of socioeconomic factors on work- and health-related outcomes. IMPLICATIONS: Challenges in the management of PsA in Latin America extend beyond the care of opportunistic infections, encompassing several other socioeconomic factors. More research is needed to better understand the peculiarities of treating PsA in Latin America to improve patient care. PROSPERO identifier: CRD42021228297.

Ultrasonographic and resistance index evaluation of nails in psoriatic arthritis, psoriasis, and control groups: a cross-sectional study.

BACKGROUND: Nail psoriasis occurs frequently in patients with psoriatic disease, it can lead to functional impairment, pain, discomfort, decreased quality of life and can also be a predictor for the development of arthritis. Early recognition of this condition can provide early and effective treatment and prevent structural impairment. This study aims to identify nail ultrasonographic characteristics in three groups: psoriasis (PsO), psoriatic arthritis (PsA) and controls patients, to determine if the ultrasonography (US) can identify early signs of nail psoriatic impairment or local inflammation. We conducted nail US to determine nail matrix resistance index (NMRI), nail bed resistance index (NBRI), and power Doppler (PD) and grayscale (GS) parameters in these 3 groups. METHODS: Single-center, cross-sectional study. GS, PD, and spectral doppler images of bilateral 2nd and 3rd fingernails were acquired from 35 PsO, 31 PsA, and 35 controls patients. An US equipment with an 18 MHz linear transducer for GS and 8.0 MHz for PD was used. PD, NMRI, NBRI, nail plate thickness (NPT), nail bed thickness (NBT), nail matrix thickness (NMT), and morphostructural characteristics of the trilaminar structure (TS) were evaluated in saved images, blind. RESULTS: Mean NMRI and NBRI did not differ between groups. Linear regression analysis detected no relationships between PsO or PsA and NMRI or NBRI. Nail PD grade did not differ between groups. Type I and IV TS changes were more frequent in PsO; types II and III changes were more frequent in PsA (p < 0.001). NPT was greater in PsA and PsO groups than controls: PsA 0.73 ± 0.14 mm, PsO 0.72 ± 0.15 mm, Controls 0.67 ± 0.10 mm (p = 0.001). CONCLUSION: Echographic TS characteristics of the nail plate and NPT evaluated by GS are useful and can distinguish PsO and PsA nails from controls. NMRI, NBRI, and US nail microcirculation parameters could not distinguish psoriatic nails. TRIAL REGISTRATION: 72762317.4.0000.5327 (Certificate of Presentation of Ethical Appreciation - CAAE - Plataforma Brasil) Avaiable in  https://plataformabrasil.saude.gov.br/login.jsf .

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 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.

Cross-cultural validation and psychometric properties of the Brazilian Portuguese version of the Psoriasis Epidemiology Screening Tool (PEST-bp).

Although the Psoriasis Epidemiology Screening Tool (PEST) questionnaire is a useful tool for screening patients for psoriatic arthritis (PsA), it has not been validated in Portuguese. Our aim was to perform a cross-cultural validation of the PEST for Brazilian Portuguese, as well as to analyse its psychometric properties and to test the association of PEST scores with patient clinical characteristics. The first step was the translation and cross-cultural adaptation of the PEST questionnaire into Brazilian Portuguese, which included forward translation, synthesis, back translation, consolidation with an expert panel and cognitive debriefing with pilot testing. The second step was validation and psychometric testing, in which 124 dermatology patients with no previous PsA diagnosis completed the new adapted questionnaire (PEST-bp). Patients were initially assessed by a dermatologist for clinical characteristics, then they answered the Dermatology Life Quality Index (DLQI) and PEST-bp questionnaires. Afterwards, a rheumatologist, blind to former tests, evaluated the presence of PsA according to CASPAR criteria. The PEST-bp proved to be 0.81 accurate (95% CI: 0.73-0.88) in the ROC curve, indicating a cutoff score ≥ 3 as suggestive of PsA (sensitivity = 84.6%, specificity = 63.3%). The assessment of internal consistency, via the Cronbach test, presented a coefficient of 0.72 (acceptable). A higher PEST-bp score was positively associated with lower quality of life scores and with male patients. In conclusion, the PEST-bp questionnaire proved to be suitable as a screening tool for PsA in patients with psoriasis. Elevated PEST-bp scores are associated with decreased patient quality of life.

Prevalence of Psoriatic Arthritis Patients Achieving Minimal Disease Activity in Real-world Studies and Randomized Clinical Trials: Systematic Review with Metaanalysis.

OBJECTIVE: To estimate the frequency of patients with psoriatic arthritis (PsA) achieving minimal disease activity (MDA) status in real-world studies and randomized controlled trials (RCT). METHODS: A systematic literature search for 2009-2017 was performed in PubMed, Embase, Cochrane Library, and LILACS. Study selection and data extraction were performed by 2 independent researchers. Random-effects single-arm metaanalyses were performed and heterogeneity was assessed using I2. RESULTS: A total of 405 records were identified and 45 studies were analyzed: 39 (86.7%) observational studies and 6 (13.3%) RCT; they included 12,469 patients. The overall prevalence of MDA in cross-sectional studies was 35% (95% CI 30%-41%, I2 = 94%), varying from 17% (95% CI 7%-34%) in patients taking synthetic disease-modifying antirheumatic drugs (DMARD) to 57% (95% CI 41%-71%) in those taking biological DMARD. Prevalence of MDA in cohort studies increased with longer followup time, ranging from 25% (95% CI 15%-40%) with 3- to 4-month followup to 42% (95% CI 38%-45%) with > 24-month followup. Patients with PsA receiving biological DMARD in a real-world context and RCT had similar prevalence of MDA at 6-month followup: 30% (95% CI 21%-41%, I2 = 85%) versus 32% (95% CI 26%-39%, I2 = 79%), respectively. CONCLUSION: Patients with PsA included in real-world studies had similar prevalence of MDA compared to those in controlled clinical trials. This finding suggests that MDA is a useful treatment target for PsA in the real-world setting.

Determinants of sleep impairment in psoriatic arthritis: An observational study with 396 patients from 14 countries.

OBJECTIVE: Sleep quality is diminished in patients with psoriatic arthritis (PsA) and close to 40% of PsA patients consider sleep difficulties a priority domain. This work analyzes determinants of impaired sleep in patients with PsA. METHODS: This was a cross-sectional analysis of an observational study (ReFlap, NCT NCT03119805), which included adult patients with definite PsA with≥2 years disease duration from 14 countries. Sleep was assessed using the patient self-reported evaluation of sleep on a 0-10 numerical scale, included in the Psoriatic Arthritis Impact of Disease questionnaire (PSAID-12). A score≥4 was considered as sleep impairment. Demographic and clinical variables associated to sleep impairment were assessed through univariate analysis and Poisson regression modeling leading to prevalence ratio (PR) [95% confidence interval]. RESULTS: A total of 396 patients were analyzed: mean age 51.9±12.6 years, 51% were females, 59.7% were receiving biologic therapy, 53.3% had 1-5% of body surface area affected by psoriasis; 23.7% were in remission and 36.9% in low disease activity according to the Disease Activity in Psoriatic Arthritis (DAPSA) score. Median (25th-75th) patient's self-evaluation of sleep difficulties was 2 (0-6), 157 (39.6%) had sleep impairment. In the Poisson regression model, self-reported levels of anxiety (PR: 1.05 [1.02-1.08], P=0.003) and pain (PR: 1.06 [1.04-1.09], P<0.001) were independently associated to sleep impairment. CONCLUSIONS: In this multicentric study, sleep impairment was present in 40% of PsA patients; pain and anxiety were associated to sleep impairment whereas inflammation was not. Impact on sleep appears multifactorial in PsA.

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.

Correlation of enthesitis indices with disease activity and function in axial and peripheral spondyloarthritis: a cross-sectional study comparing MASES, SPARCC and LEI.

BACKGROUND: The presence of enthesitis is associated with higher disease activity, more disability and incapacity to work and a poorer quality of life in spondyloarthritis (SpA). There is currently no consensus on which clinical score should be used to assess enthesitis in SpA. The objective of the present work was to compare the correlation of three enthesitis indices (MASES, SPARCC and LEI) with measures of disease activity and function in a heterogeneous population of patients with axial and peripheral SpA. METHODS: A cross-sectional study was conducted in three Brazilian public university hospitals; patients fulfilling ASAS classification criteria for peripheral or axial SpA were recruited and measures of disease activity and function were collected and correlated to three enthesitis indices: MASES, SPARCC and LEI using Spearman's Correlation index. ROC curves were used to determine if the the enthesitis indices were useful to discriminate patients with active disease from those with inactive disease. RESULTS: Two hundred four patients were included, 71.1% (N = 145) fulfilled ASAS criteria for axial SpA and 28.9% (N = 59) for peripheral SpA. In axial SpA, MASES performed better than LEI (p = 0.018) and equal to SPARCC (p = 0.212) regarding correlation with disease activity (BASDAI) and function (BASFI). In peripheral SpA, only MASES had a weak but statistical significant correlation with DAS28-ESR (rs 0.310 p = 0.05) and MASES had better correlation with functional measures (HAQ) than SPARCC (p = 0.034). CONCLUSION: In this sample composed of SpA patients with high coexistence of axial and peripheral features, MASES showed statistical significant correlation with measures of disease activity and function in both axial and peripheral SpA.

Patients with longstanding psoriatic arthritis can achieve DAPSA remission or low disease activity and it correlates to better functional outcomes: results from a Latin-American real-life cohort

Abstract Background Patients with psoriatic arthritis (PsA) experience reduced physical function and impaired quality of life. Better patient-reported functional outcomes are found when lower disease activity is achieved. Objectives To evaluate the variation of physical function by HAQ-DI over time in PsA patients treated with standard therapy in a real-life setting: to verify predictors of achieving a minimum clinically important difference (MCID) in function by HAQ-DI (ΔHAQ-DI ≤ − 0.35) and to measure the impact of achieving REM/LDA on long-term function by HAQ-DI. Methods This is a longitudinal analysis of a real-life retrospective cohort. Data from PsA patients with at least 4 years of follow-up in the PsA clinic from 2011 to 2019 were extracted from electronic medical records. The variations of physical function by HAQ-DI and disease activity by DAPSA over time were calculated. A multivariate hierarchical regression model was applied to verify predictors of MCID in HAQ-DI. A comparison of HAQ-DI variation between patients with DAPSA REM, LDA, moderate and high disease activity was made using the generalized estimating equation model (GEE), adjusted by Bonferroni test. The Spearman correlation method was applied to verify the correlation of ΔDAPSA and ΔHAQ-DI over time. Statistical analysis was performed in SPSS program version 21.0. Results Seventy-three patients were included in the analysis. Physical function measured by HAQ-DI was determined by PsA disease activity measured by DAPSA (p < 0.000). A moderate and statistically significant correlation between ΔDAPSA and ΔHAQ-DI was observed (rs = 0.60; p < 0.001). Only patients in DAPSA REM demonstrated a constant decline in HAQ-DI scores during the follow-up. White ethnicity and older age at baseline were predictors for not achieving MCID in HAQ-DI [RR 0.33 (0.16-0.6795% CI p = 0.002) and RR 0.96 (0.93-0.9895% CI p < 0.000), respectively, while higher scores of HAQ-DI at baseline were predictors of achieving MCID [RR 1.71 (1.12-2.6095%CI p = 0.013)]. Conclusion In PsA, patients who maintained DAPSA REM/LDA over time had better long-term functional outcomes. Higher HAQ-DI scores at baseline, non-white ethnicity and younger age were predictors for achieving a clinical meaningful improvement of HAQ-DI.

Treating psoriatic arthritis to target: discordance between physicians and patients' assessment, non-adherence, and restricted access to drugs precluded therapy escalation in a real-world cohort.

The treat-to-target strategy (T2T) was associated with better outcomes in psoriatic arthritis (PsA) compared to standard care in clinical trials. This study aimed to analyze factors precluding treatment optimization in a T2T strategy conducted in a real-world cohort of PsA patients. A retrospective cross-sectional study nested in a cohort was conducted. Medical records of patients ≥ 18 years old, fulfilling CASPAR criteria and with at least one visit in the PsA clinic, were reviewed. Demographic data, current medication, and minimal disease activity (MDA) criteria were recorded. Reasons for the non-escalation of therapy in patients who were not classified as MDA were reported as absolute and relative frequencies. In the 8-month period, 131 visits (corresponding to 74 patients) were conducted. The MDA criteria were available in 113 visits (86.3%) and patients were classified as MDA in 31.0% of the visits (N = 35/113). Although in 69.0% of the visits patients were not in MDA, (N = 78/113), therapy was adjusted in only 42.3% (N = 33/78). Reasons precluding treatment escalation in non-MDA subjects were physician's impression of remission (57.7%, N = 26), non-adherence to previous prescription (17.8%, N = 8), restricted access to drugs (17.8%, N = 8), adverse events (11.1%, N = 5), poor understanding of medication instructions (6.7%, N = 3), patient's refusal to escalate therapy (4.4%, N = 2), and recent change in therapy (2.2%, N = 1). Discordance between the physician's clinical evaluation and the MDA criteria, non-adherence to prescription, and poor access to drugs were the main factors precluding escalation of therapy in a T2T strategy in a real-world PsA cohort.

The effects of cultural background on patient-perceived impact of psoriatic arthritis - a qualitative study conducted in Brazil and France.

BACKGROUND: In psoriatic arthritis (PsA) almost all qualitative studies have been performed in European populations. This work aimed to evaluate the impact of PsA in Brazilian and French subjects, as well as to explore cultural differences in the experience of disease and to recognize domains important for patients living with PsA outside Europe. METHODS: A qualitative study was conducted in two university hospitals in Brazil and France; outpatients fulfilling Classification Criteria for PsA participated in individual interviews regarding the impact of PsA; interviews were conducted in the local language. The sample size was defined by saturation; interviews were recorded and transcribed and content analysis was performed. RESULTS: Fifteen patients were interviewed in Brazil and 13 in France. Mean disease duration was 16.5 ± 12.5 years (range: 8 months to 47 years) and 14.4 ± 8.4 years (range 12 months to 29 years) for Brazilian and French subjects, respectively. A broad impact was perceived: 67 codes emerged from the interviews and were grouped in 41 categories. Although 2/3 of categories were common to both nationalities, some important health domains from the perspective of PsA patients from a non-European background were brought to light including sexual dysfunction, emotional impact of psoriasis and impact of prejudice on social and professional life. CONCLUSIONS: This study highlights the importance of assessing the impact of PsA on a national level, emphasizing the common cross-cultural aspects but also revealing domains of interest for patients with PsA living outside Europe which merit further study.

Ambulatório conjunto de dermatologia e reumatologia para tratamento de psoríase e artrite psoriásica: experiência de um hospital terciário do sul do Brasil / Combined dermatology and rheumatology outpatient clinic for the management of psoriasis and psoriatic arthritis: a Southern Brazilian experience

Introdução: Diversas diretrizes enfatizam as vantagens do manejo multidisciplinar no cuidado ao paciente com psoríase (PSO) e artrite psoriásica (PSA). O diagnóstico precoce de PSA se relaciona com melhores desfechos em 5 anos. No entanto, o diagnóstico precoce de PSA ainda é um desafio. Métodos: Estudo transversal retrospectivo de pacientes com diagnóstico prévio ou suspeito de PSO e/ou PSA atendidos em ambulatório conjunto de dermatologia e reumatologia do sul do Brasil de janeiro de 2013 a janeiro de 2017. Resultados: Entre os 55 pacientes previamente diagnosticados com PSO, 30,9% (n = 17) foram diagnosticados com PSA. Alterações do tratamento foram feitas em 58,5% (n = 48) dos pacientes, principalmente devido ao mau controle da pele e das articulações. Os imunobiológicos foram a classe de medicamentos mais comumente iniciada, correspondendo a 35,4% (n = 17) das modificações terapêuticas. O metotrexato foi o segundo medicamento mais comumente iniciado (18,8%, n = 9) ou com modificação da dose ou via de administração (20,8%, n = 10), totalizando 39,6% (n = 19) de modificações terapêuticas. Houve um aumento na proporção de pacientes em uso de tratamento sistêmico (79,3%, n = 65). Conclusão: Este estudo reforça a importância da abordagem multidisciplinar no diagnóstico precoce da PSA e demonstra que a abordagem conjunta entre dermatologia e reumatologia é possível de ser realizada no Brasil, com resultados semelhantes aos reportados na literatura internacional. (AU)

Introduction: Several guidelines emphasize the advantages of multidisciplinary management of patients with psoriasis (PSO) or psoriatic arthritis (PSA). Early diagnosis of PSA is associated with better outcomes in 5 years. However, early diagnosis of PSA remains a challenge. Methods: We conducted a retrospective cross-sectional study of patients with previous or suspected diagnosis of PSO and/or PSA treated at a combined dermatology and rheumatology outpatient clinic in southern Brazil from January 2013 to January 2017. Results: Of 55 patients previously diagnosed with PSO, 30.9% (n = 17) were diagnosed with PSA. Changes in treatment were made in 58.5% (n = 48) of patients, mainly due to poor control of cutaneous and articular symptoms. Immunobiological agents were the most commonly prescribed class of drugs, corresponding to 35.4% (n = 17) of changes in medical therapy. Methotrexate was the second most commonly prescribed drug (18.8%; n = 9) and the second drug to undergo most changes in dose or route of administration (20.8%; n = 10), accounting for 39.6% (n = 19) of changes in medical therapy. There was an increase in the number of patients undergoing systemic therapies (79.3%; n = 65). Conclusions: This study reinforces the importance of a multidisciplinary approach in the early diagnosis of PSA and demonstrates that a collaborative approach between dermatology and rheumatology is feasible in Brazil, with outcomes similar to those reported in the international literature. (AU)

Ultrasonographic and resistance index evaluation of nails in psoriatic arthritis, psoriasis, and control groups: a cross-sectional study

Abstract Background: Nail psoriasis occurs frequently in patients with psoriatic disease, it can lead to functional impairment, pain, discomfort, decreased quality of life and can also be a predictor for the development of arthritis. Early recognition of this condition can provide early and effective treatment and prevent structural impairment. This study aims to identify nail ultrasonographic characteristics in three groups: psoriasis (PsO), psoriatic arthritis (PsA) and controls patients, to determine if the ultrasonography (US) can identify early signs of nail psoriatic impairment or local inflammation. We conducted nail US to determine nail matrix resistance index (NMRI), nail bed resistance index (NBRI), and power Doppler (PD) and grayscale (GS) parameters in these 3 groups. Methods: Single-center, cross-sectional study. GS, PD, and spectral doppler images of bilateral 2nd and 3rd fingernails were acquired from 35 PsO, 31 PsA, and 35 controls patients. An US equipment with an 18 MHz linear transducer for GS and 8.0 MHz for PD was used. PD, NMRI, NBRI, nail plate thickness (NPT), nail bed thickness (NBT), nail matrix thickness (NMT), and morphostructural characteristics of the trilaminar structure (TS) were evaluated in saved images, blind. Results: Mean NMRI and NBRI did not differ between groups. Linear regression analysis detected no relationships between PsO or PsA and NMRI or NBRI. Nail PD grade did not differ between groups. Type I and IV TS changes were more frequent in PsO; types II and III changes were more frequent in PsA (p < 0.001). NPT was greater in PsA and PsO groups than controls: PsA 0.73 ± 0.14 mm, PsO 0.72 ± 0.15 mm, Controls 0.67 ± 0.10mm (p = 0.001). Conclusion: Echographic TS characteristics of the nail plate and NPT evaluated by GS are useful and can distinguish PsO and PsA nails from controls. NMRI, NBRI, and US nail microcirculation parameters could not distinguish psoriatic nails. Trial registration: 72762317.4.0000.5327 (Certificate of Presentation of Ethical Appreciation - CAAE -Plataforma Brasil) Avaiable in https://plataformabrasil.saude.gov.br/login.jsf

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)

Is ultrasound a better target than clinical disease activity scores in rheumatoid arthritis with fibromyalgia? A case-control study.

OBJECTIVES: Our goal is to study the correlations among gray-scale seven-joint ultrasound score (GS-US7), power Doppler seven-joint ultrasound score (PD-US7), disease activity score-28 joints (DAS28), simplified disease activity index (SDAI) and clinical disease activity index (CDAI) in patients with and without fibromyalgia (FM). METHODS: A matched case-control study included all patients consecutively seen in the Rheumatoid Arthritis (RA) Clinic. Participants were allocated into one of two groups: RA with FM and RA without FM. Ultrasound (US) and clinical scoring were blinded for the presence of FM. Medians and proportions were compared by Mann-Whitney's test and McNemar's test, respectively. Spearman's rank correlation coefficients (rs) were calculated among clinical and US scores and differences were tested by r-to-z transformation test. RESULTS: Seventy-two women were included, out of 247 RA patients, mostly white, with median (IQR) age of 57.5 (49.3-66.8) years, with RA symptoms for 13.0 (6.0-19.0) years and FM symptoms for 6.0 (2.0-15.0) years. Disease-modifying antirheumatic drugs, non-steroidal anti-inflammatory drugs and prednisone use was comparable between groups. Objective activity parameters were not different between groups. RA patients with FM had greater DAS28, SDAI and CDAI but similar GS-US7 and PD-US7. GS-US7 correlated with DAS28, SDAI and CDAI in patients with and without FM (rs = 0.36-0.57), while PD-US7 correlated with clinical scores only in patients without FM (rs = 0.35-0.38). CONCLUSION: To our knowledge, this is the first study to demonstrate that ultrasound synovitis scores are not affected by FM in RA patients. PD-US7 performed better than GS-US7 in long-standing RA patients with DAS28, SDAI or CDAI allegedly overestimated due to FM. Since sonographic synovitis predicts erosion better than swollen joint count, C-reactive protein and erythrocyte sedimentation rate, US should be considered a promising treatment target in RA patients with FM.