The effect of the collateral cerebrovascular circulation on tolerance to carotid artery cross-clamping and on early outcome after carotid endarterectomy.

OBJECTIVE: The Circle of Willis (CoW) serves as the primary source of contralateral blood supply in patients who undergo carotid artery cross-clamping (CC) for carotid endarterectomy (CEA). It has been suggested that the CoW's anatomy influences CEA outcomes. The aim of this study was to evaluate associations between the cerebral collateral circulation, a positive awake test for intraoperative neurologic deficit after carotid CC, and postoperative adverse neurologic events. METHODS: A systematic review was conducted searching MEDLINE, Cochrane, and Web of Science databases for studies that assessed the cerebral circulation, including CoW variations, using neuroimaging techniques in patients who underwent carotid CC. For the metanalytical incidence, the statistical technique used was weight averaging. Otherwise, descriptive analysis was used due to the excessive heterogeneity of the studies. RESULTS: Eight publications, seven cohort and one case-controlled study, involving 1313 patients who underwent carotid artery CC under loco-regional anesthesia, were included in the systematic review. The incidence of positive awake test in the cohort studies ranged from 4.4% to 19.7%. Carotid artery CC resulted in positive awake test in 5% to 91% of patients with alterations in the anterior portion and in 27% to 74% with alterations in the posterior portion of the CoW. A positive awake test in patients with contralateral carotid stenosis or occlusion ranged from 5.8% to 45.7%. Contralateral carotid stenosis >70% or occlusion were associated with a positive awake test (P < .001). Patients with incomplete CoW did not have statistically significant correlation with intraoperative neurological deficits after CC. Data were insufficient to evaluate the effect of the collateral circulation on early outcome after CEA. CONCLUSIONS: In this systematic review, contralateral carotid artery stenosis or occlusion, but not CoW abnormalities, were associated with a positive awake test after carotid artery CC. Further research is needed to evaluate which specific CoW anomaly predicts neurologic deficit after CC and to confirm association between a positive awake test and clinical outcome after CEA.

Patients' unmet needs and treatment preferences concerning digital ulcers in systemic sclerosis.

INTRODUCTION: Digital ulcers (DUs) significantly impact on quality of life and function in patients with systemic sclerosis (SSc). The aim of our survey was to explore patients' perspectives and their unmet needs concerning SSc-DUs. MATERIALS: SSc patients were invited through international patient associations and social media to participate in an online survey. RESULTS: 358 responses were obtained from 34 countries: US (65.6%), UK (11.5%) and Canada (4.5%). Recurrent DUs are common: >10 DUs (46.1%), 5-10 DUs (21.5%), 1-5 DUs (28.5%), 1 DU (3.9%). Fingertip DUs were most frequent (84.9%), followed by those overlying the interphalangeal joints (50.8%). The impact of DUs in patients is broad, from broad-ranging emotional impacts to impact on activities of daily living, and personal relationships. Half (51.7%) of respondents reported that they received wound/ulcer care, most often provided by non-specialist wound care clinics (63.8%). There was significant variation in local (wound) DU care, in particular the use of debridement and pain management. DU-related education was only provided to one-third of patients. One-quarter (24.6%) were 'very satisfied' or 'satisfied' that the provided DU treatment(s) relieved their DU symptoms. Pain, limited hand function, and ulcer duration/chronicity were the main reasons for patients to consider changing DU treatment. CONCLUSIONS: Our data show that there is a large variation in DU treatment between countries. Patient access to specialist wound-care services is limited and only a small proportion of patients had their DU needs met. Moreover, patient education is often neglected. Evidence-based treatment pathways are urgently needed for DU management.

A multi-national survey to identify clinicians' perspectives concerning Proton Pump inhibitors in patients with systemic sclerosis.

OBJECTIVES: Proton Pump Inhibitors (PPIs) are widely used in SSc for gastroesophageal reflux disease (GERD). However, there is little evidence to support their empirical use and long-term safety has been questioned. Our objective was to better describe clinicians' attitudes toward PPIs prescription and use in SSc patients. METHODS: Clinicians involved in the care of SSc patients were invited through international physician networks and social media to participate in an online survey. RESULTS: Responses from 227 clinicians from 36 countries were evaluable. The majority 'agreed' (41.4 %) or 'strongly agreed' (45.4 %) that GERD is a major cause of morbidity in SSc. Lifestyle modifications are seldom (16 %) considered effective. Only half 'agreed' (43 %) or 'strongly agreed' (11 %) there is solid evidence supporting PPIs efficacy in SSc. The most common reasons for PPIs prescription were symptomatic GERD unresponsive to lifestyle modification (95 %), objective evidence of GERD (82 %), and hoarseness or respiratory symptoms (71 %). There are variable concerns about PPIs long-term safety in SSc. The three highest (mean) reasons (0-10, here 10 is 'very concerned') were: small intestinal bacterial overgrowth (5.5), osteoporosis (5.4), and drug interactions (5.2). There are significant differences in attitudes towards surgery for refractory GERD, and concerns about potential complications. PPIs may have a putative role for disease modification (e.g., ILD and calcinosis), and the role of immunosuppression is uncertain for GI (gastrointestinal) disease in SSc. CONCLUSION: PPIs are frequently prescribed in SSc. Side effects are a recognized concern, especially regarding long-term therapy. There is significant variation in attitudes towards surgical intervention. Future research and practical treatment recommendation for PPIs in SSc are urgently needed.

Subacute Neurotoxicity as an Adverse Reaction to Ceftazidime: A Case Report.

Neurotoxicity associated with cephalosporins is an increasingly recognized complication, although among cephalosporins, ceftazidime is rarely reported for such an adverse reaction. Moreover, subacute, rather than acute, presentation of neurotoxicity associated with cephalosporins is rare. A 77-year-old female patient with stage 4 chronic renal disease was admitted due to cellulitis in her right lower limb, multiorgan dysfunction complicated by oliguric acute kidney injury, and a need for hemodialysis via a central venous catheter. On the 13th day after admission, she became febrile, and bacteremia associated with a central venous catheter was identified, which prompted the initiation of empirical antibiotic therapy with vancomycin and ceftazidime. After 13 days of antibiotic therapy with vancomycin and ceftazidime, the patient became confused, with temporal-spatial disorientation and myoclonus, especially in the upper limbs, with worsening renal function. Ceftazidime was discontinued, and the patient's condition improved with complete remission of symptoms on the 8th day after symptom onset. Neurotoxicity associated with ceftazidime is a rare but probably underdiagnosed adverse reaction. It is more frequent in elderly patients with previous neurological dysfunction and end-stage kidney disease and/or acute kidney injury, and it usually manifests soon after starting treatment. Early identification and monitoring of risk factors and symptoms should lead the physician to a rapid withdrawal of the offending drug.

A Multinational Survey Investigating the Unmet Needs and Patient Perspectives Concerning Proton Pump Inhibitors in Systemic Sclerosis.

OBJECTIVE: Proton pump inhibitors (PPIs) are widely prescribed to treat gastroesophageal reflux disease (GERD) in Systemic Sclerosis (SSc). However, not all patients adequately respond to the treatment, and there are frequent concerns about the safety of long-term use of PPIs. Our aim was to identify the main problems/complaints of SSc patients on PPIs, as well as understand their unmet needs. METHODS: SSc patients treated with PPIs were invited through international patient associations and social media to participate in an online survey. RESULTS: We gathered 301 valid responses from 14 countries (United Kingdom 19.3% and United States 70.4%). Multiple PPIs use (two: 30% and three: 21% in series) was common. The majority (89%) reported improvement in gastrointestinal symptoms from receiving PPIs. Side effects attributed to receiving PPIs were uncommon (19%); however, most (79%) were potentially concerned. Around half (58%) had received lifestyle information, and most (85%) had searched online for information about PPIs. Only in the minority (12%) had a surgical approach been discussed; however, half (46%) indicated that they would be willing to undergo surgery to resolve their GERD symptoms but had important concerns. CONCLUSION: Despite the frequent use of PPIs in patients with SSc, there is significant heterogeneity in prescription, and combination therapy (PPIs plus other medication for acid reflux) is not uncommon (approximately 40%). Patients have significant concerns about PPIs side effects. Education about PPIs is often neglected, and patients very frequently use online sources to obtain information on drug treatment. A surgical approach is infrequently discussed, and patients fear this potential therapeutic approach.

Effects of Dietary Methionine Restriction on Cognition in Mice.

Dietary restriction of the essential amino acid, methionine, has been shown to induce unique metabolic protection. The peripheral benefits of methionine restriction (MR) are well established and include improvements in metabolic, energy, inflammatory, and lifespan parameters in preclinical models. These benefits all occur despite MR increasing energy intake, making MR an attractive dietary intervention for the prevention or reversal of many metabolic and chronic conditions. New and emerging evidence suggests that MR also benefits the brain and promotes cognitive health. Despite widespread interest in MR over the past few decades, many findings are limited in scope, and gaps remain in our understanding of its comprehensive effects on the brain and cognition. This review details the current literature investigating the impact of MR on cognition in various mouse models, highlights some of the key mechanisms responsible for its cognitive benefits, and identifies gaps that should be addressed in MR research moving forward. Overall findings indicate that in animal models, MR is associated with protection against obesity-, age-, and Alzheimer's disease-induced impairments in learning and memory that depend on different brain regions, including the prefrontal cortex, amygdala, and hippocampus. These benefits are likely mediated by increases in fibroblast growth factor 21, alterations in methionine metabolism pathways, reductions in neuroinflammation and central oxidative stress, and potentially alterations in the gut microbiome, mitochondrial function, and synaptic plasticity.

Immune-mediated skin lesions related to biological disease-modifying antirheumatic drugs: a 22-year experience of a tertiary center.

INTRODUCTION: Immune-mediated skin lesions (IMSL) can be very disabling leading to treatment discontinuation. Although these lesions have rarely been previously described, the true incidence is unknown. OBJECTIVE: To explore the cumulative incidence, management and outcomes of IMSL related to bDMARD in a large cohort of patients with chronic inflammatory rheumatic diseases. To explore possible associations and risk factors for IMSL development. METHODS: A retrospective single-center study of patients with rheumatoid arthritis (RA), spondylarthritis (SpA) and psoriatic arthritis (PsA) that had been treated with at least one bDMARD for at least 6 months was conducted. IMSL related to bDMARD characteristics and outcomes were collected. RESULTS: A total of 989 patients with RA, SpA and PsA were included. Twenty-seven patients (2.7%) presented IMSL potentially related to bDMARD, being psoriasis the most common IMSL (n=12, 44.4%), followed by drug-induced lupus erythematosus (n=6), alopecia areata (n=3) and leukocytoclastic vasculitis (n=2). IMSL led to withdrawal of bDMARD in 18 of the 27 patients (66.7%). Patients with IMSL had younger age at diagnosis (p=0.038), longer disease duration (p=0.018), longer duration of bDMARD treatment (p=0.008), and higher number of previous bDMARDs (p < 0.001) than patients without IMSL. In the group of patients with IMSL there was a significantly higher percentage of patients treated with adalimumab (p < 0.001). In multivariate regression model, the number of previous bDMARDs (OR 2.13, 95%CI 1.47-3.10, p < 0.001) and treatment with adalimumab (OR 4.60, 95%CI 1.96-10.80 , p < 0.001) were statistically significant predictive factors for IMSL development. CONCLUSION: In our study, IMSL related to bDMARDs had an estimated cumulative incidence of 2.7%. Younger age at diagnosis, longer disease duration, longer duration of bDMARD treatment, higher number of previous bDMARDs and treatment with adalimumab were independently associated with an increased risk of IMSL development.

The idiopathic inflammatory myopathies module of the Rheumatic Diseases Portuguese Register.

AIMS: To characterise the idiopathic inflammatory myopathies (IIM) module of the Rheumatic Diseases Portuguese Register (Reuma.pt/myositis) and the patients in its cohort. METHODS: Reuma.pt is a web-based system with standardised patient files gathered in a registry. This was a multicentre open cohort study, including patients registered in Reuma.pt/myositis up to January 2022. RESULTS: Reuma.pt/myositis was designed to record all relevant data in clinical practice and includes disease-specific diagnosis and classification criteria, clinical manifestations, immunological data, and disease activity scores. Two hundred eighty patients were included, 71.4% female, 89.4% Caucasian, with a median age at diagnosis and disease duration of 48.9 (33.6-59.3) and 5.3 (3.0-9.8) years. Patients were classified as having definite (N=57/118, 48.3%), likely (N=23/118, 19.5%), or possible (N=2/118, 1.7%) IIM by 2017 EULAR/ACR criteria. The most common disease subtypes were dermatomyositis (DM, N=122/280, 43.6%), polymyositis (N=59/280, 21.1%), and myositis in overlap syndromes (N=41/280, 14.6%). The most common symptoms were proximal muscle weakness (N=180/215, 83.7%) and arthralgia (N=127/249, 52.9%), and the most common clinical signs were Gottron's sign (N=75/184, 40.8%) and heliotrope rash (N=101/252, 40.1%). Organ involvement included lung (N=78/230, 33.9%) and heart (N=11/229, 4.8%) involvements. Most patients expressed myositis-specific (MSA, N=158/242, 65.3%) or myositis-associated (MAA, 112/242, 46.3%) antibodies. The most frequent were anti-SSA/SSB (N=70/231, 30.3%), anti-Jo1 (N=56/236, 23.7%), and anti-Mi2 (N=31/212, 14.6%). Most patients had a myopathic pattern on electromyogram (N=101/138, 73.2%), muscle oedema in magnetic resonance (N=33/62, 53.2%), and high CK (N=154/200, 55.0%) and aldolase levels (N=74/135, 54.8%). Cancer was found in 11/127 patients (8.7%), most commonly breast cancer (N=3/11, 27.3%). Most patients with cancer-associated myositis had DM (N=8/11, 72.7%) and expressed MSA (N=6/11) and/or MAA (N=3/11). The most used drugs were glucocorticoids (N=201/280, 71.8%), methotrexate (N=117/280, 41.8%), hydroxychloroquine (N=87/280, 31.1%), azathioprine (N=85/280, 30.4%), and mycophenolate mofetil (N=56/280, 20.0%). At the last follow-up, there was a median MMT8 of 150 (142-150), modified DAS skin of 0 (0-1), global VAS of 10 (0-50) mm, and HAQ of 0.125 (0.000-1.125). CONCLUSIONS: Reuma.pt/myositis adequately captures the main features of inflammatory myopathies' patients, depicting, in this first report, a heterogeneous population with frequent muscle, joint, skin, and lung involvements.

Severe infections in Portuguese patients with rheumatoid arthritis under biologic treatment - a multicenter, nationwide study (SIPPRA-B Study).

INTRODUCTION: Despite years of experience with biological disease modifying anti-rheumatic drugs (bDMARD) in rheumatoid arthritis (RA), little is known about differences in infectious risk among bDMARDs. The aim of this study was to assess the incidence and type of infections in RA patients on bDMARDs and to determine possible predictors. METHODS: A retrospective multicenter cohort study that included patients registered in the Rheumatic Diseases Portuguese Registry (Reuma.pt) with RA, and exposed to at least one bDMARD until April 2021. RA patients under bDMARD and with at least one episode of severe infection (SI), defined as infection that requires hospitalization, use of parenteral antibiotics or that resulted in death, were compared to patients with no report of SI. Demographic and clinical data at baseline and at the time of each SI were collected to establish comparisons between different groups of bDMARDs. Comparisons between different bDMARDs were assessed and logistic regression was performed to identify predictors of SI. RESULTS: We included 3394 patients, 2833 (83.5%) female, with a mean age at RA diagnosis of 45.5±13.7 years. SI was diagnosed in 142 of the 3394 patients evaluated (4.2%), totaling 151 episodes of SI. At baseline, patients with SI had a significantly higher proportion of prior orthopedic surgery, asthma, interstitial lung disease, chronic kidney disease and corticosteroid use, higher mean age and longer median disease duration at first bDMARD. Nine patients died (6.0%). Ninety-two SI (60.9%) occurred with the first bDMARD, the majority leading to discontinuation of the bDMARD within 6 months (n=75, 49.7%), while 65 (43.0%) restarted the same bDMARD and 11 (7.3%) switched to another bDMARD (6 of them to a different mechanism of action). In the multivariate analysis, we found that chronic kidney disease, asthma, infliximab, corticosteroid use, interstitial lung disease, previous orthopedic surgery, higher Health Assessment Questionnaire and DAS284V-ESR are independent predictors of SI. CONCLUSION: This study described the incidence and types of SI among Portuguese RA patients on biologics, identifying several predictors of SI, both globally and with different bDMARDs. Physicians should be aware of the real-word infectious risk in RA patients on bDMARDs when making treatment decisions.

The impact of antinuclear antibodies seroconversion induced by anti-tumor necrosis factor α agents on the clinical outcomes in rheumatic patients.

INTRODUCTION: Anti-tumor necrosis factor α (anti-TNFα) agents can potentially induce the anti-nuclear antibodies (ANA) development over time. Evidence of the real impact of these autoantibodies on clinical response to treatment in rheumatic patients is still scarce. OBJECTIVES: To explore the impact of ANA seroconversion induced by anti-TNFα therapy on clinical outcomes in biologic-naïve patients with Rheumatoid arthritis (RA), axial spondylarthritis (axSpA) and psoriatic arthritis (PsA). METHODS: An observational retrospective cohort study enrolling biologic-naïve patients with RA, axSpA and PsA who started their first anti-TNFα agent was conducted for 24 months(M). Sociodemographic data, laboratory findings, disease activity and physical function scores were collected at baseline, 12M and 24M. To examine the differences between the groups with and without ANA seroconversion, independent samples t-tests, Mann-Whitney U-tests and chi-square tests were performed. Linear and logistic regression models were used to assess the effects of ANA seroconversion on the clinical response to treatment. RESULTS: A total of 432 patients with RA (N=185), axSpA (N=171) and PsA (N=66) were included. ANA seroconversion rate at 24M was 34.6%, 64.3% and 63.6% for RA, axSpA and PsA, respectively. Regarding sociodemographic and clinical data in RA and PsA patients, no statistically significant differences between groups with and without ANA seroconversion were found. In axSpA patients, ANA seroconversion was more frequent in patients with higher body mass index (p=0.017) and significantly less frequent in patients treated with etanercept (p=0.01). Regarding disease activity, DAS28 for RA patients and ASDAS-CRP for axSpA patients were significantly higher in ANA seroconversion group at 12M (p=0.017 and p=0.009, respectively). For PsA patients, CDAI was significantly higher in ANA seroconversion group at 24M (p=0.043). Overall switching rate of biologic disease-modifying antirheumatic drugs (bDMARD) was significantly higher in the ANA seroconversion group over time (p=0.025). For RA patients, ANA seroconversion predicted DAS28 (ß=-0.21, 95%CI[-1.86;-0.18], p=0.017) at 12M. CONCLUSIONS: ANA seroconversion induced by anti-TNFα agents could interfere in clinical response of patients with rheumatic diseases. The presence of these autoantibodies can be considered as a potential predictor of poor treatment response and higher need for bDMARD switching over time.

When a Hypersomnolent Hemodialyzed Patient Actually Has a Rare Stroke: A Case of Artery of Percheron Infarction With Paradoxical Embolism.

INTRODUCTION: Artery of Percheron (AOP) is an uncommon anatomic variant of the arterial supply of the medial thalami. Owing to variable clinical presentation, challenging imaging diagnosis, and its rarity, it is difficult to diagnose AOP infarctions. We present a clinical case of a unique presentation of AOP infarction associated with paradoxical embolism and highlight the atypical clinical manifestations and challenging diagnosis of this stroke syndrome. CASE REPORT: A 58-year-old White female with chronic renal insufficiency on hemodialysis was admitted to our center with a 10-hour course of hypersomnolence and right-sided ataxia. She had normal body temperature, blood pressure, peripheral oxygen saturation, and heart rate and scored 11 points in the Glasgow Coma Scale and 12 points in National Institutes of Health Stroke Scale. Initial brain computerized tomography scan, electrocardiogram, and thoracic radiography were normal; transcranial Doppler ultrasound showed >50% stenosis at the P2 segment of the right posterior cerebral artery, and transthoracic echocardiogram, a patent foramen ovale and thrombus adherent to the hemodialysis catheter. On day 3, she underwent brain magnetic resonance that showed acute ischemic lesions at the paramedian thalami and the superior cerebral peduncles. AOP infarction due to a paradoxical embolism from a patent foramen ovale with a right atrial thrombus was the final diagnosis. CONCLUSIONS: AOP infarctions are a rare type of stroke with elusive clinical presentations and frequently, initial imaging assessment is normal. Early recognition is crucial, and a high index of suspicion is needed to suspect this diagnosis.

Tuberculous Osteomyelitis of The Pubic Symphysis - a Case Report of a Rare Entity Mimicking Spondyloarthritis.

Tuberculosis (TB) osteomyelitis of the pubic symphysis is an extremely rare diagnosis. Axial spondyloarthritis (SpA) is characterized by inflammatory back pain and enthesitis, and involvement of pubic symphysis is very unusual at presentation. A 36-year-old female patient with a history of inflammatory back and pubic pain was referred to Rheumatology. She had a pelvic magnetic resonance imaging (MRI) suggestive of osteitis pubis. She was started on etoricoxib 90mg/day as axial spondyloarthritis was suspected, with no improvement. Pelvic MRI was repeated and showed osteomyelitis of the iliopubic branches. An ultrasound-guided biopsy was performed, and culture was positive for Mycobacterium tuberculosis. Further imaging studies revealed small cavitations and several centrilobular micronodules with a tree-in-bud pattern in the upper lung lobes and in the upper segment of the lower left lobe. She was started on anti-tuberculous treatment for 1 year and had a good clinical and radiological response. TB osteomyelitis of the pubic symphysis is a rare entity and has seldom been reported. However, this is the first case, to our knowledge, where the clinical picture mimicked an itself unusual presentation of SpA.

Predictors of cardiac involvement in idiopathic inflammatory myopathies.

Objectives: Idiopathic inflammatory myopathies (IIM) are a group of rare disorders that can affect the heart. This work aimed to find predictors of cardiac involvement in IIM. Methods: Multicenter, open cohort study, including patients registered in the IIM module of the Rheumatic Diseases Portuguese Register (Reuma.pt/Myositis) until January 2022. Patients without cardiac involvement information were excluded. Myo(peri)carditis, dilated cardiomyopathy, conduction abnormalities, and/or premature coronary artery disease were considered. Results: 230 patients were included, 163 (70.9%) of whom were females. Thirteen patients (5.7%) had cardiac involvement. Compared with IIM patients without cardiac involvement, these patients had a lower bilateral manual muscle testing score (MMT) at the peak of muscle weakness [108.0 ± 55.0 vs 147.5 ± 22.0, p=0.008] and more frequently had oesophageal [6/12 (50.0%) vs 33/207 (15.9%), p=0.009] and lung [10/13 (76.9%) vs 68/216 (31.5%), p=0.001] involvements. Anti-SRP antibodies were more commonly identified in patients with cardiac involvement [3/11 (27.3%) vs 9/174 (5.2%), p=0.026]. In the multivariate analysis, positivity for anti-SRP antibodies (OR 104.3, 95% CI: 2.5-4277.8, p=0.014) was a predictor of cardiac involvement, regardless of sex, ethnicity, age at diagnosis, and lung involvement. Sensitivity analysis confirmed these results. Conclusion: Anti-SRP antibodies were predictors of cardiac involvement in our cohort of IIM patients, irrespective of demographical characteristics and lung involvement. We suggest considering frequent screening for heart involvement in anti-SRP-positive IIM patients.

Oculomotor nerve palsy, an unusual onset of polyarteritis nodosa.

Introduction: Cranial nerve involvement in polyarteritis nodosa(PAN) is underrecognized and rarely reported. The aim of this article is to review the available literature and present an example of oculomotor nerve palsy in the course of PAN. Material and methods: Evaluation of texts describing the analyzed problem using the terms "polyarteritis nodosa", "nerve", "oculomotor", "cranial nerve" and "cranial neuropathy" for searching the PubMed database was done. Only full-text articles in English language with titles and abstracts were included in the analysis. As a guideline for the analysis of articles, the methodology described in the Principles of Individual Patient Data systematic reviews (PRISMA-IPD) was used. Results: After screening articles only 16 reported cases of PAN with cranial neuropathy were included in the analysis. In 10 the cranial neuropathy was reported as the initial manifestation of PAN with optic nerve involvement as the most frequent (62.5%); among these cases the oculomotor nerve was involved in 3 cases. Treatment with glucocorticosteroids and cyclophosphamide was the most common. Conclusions: Although cranial neuropathy, especially oculomotor nerve palsy is a rare first neurological manifestation of PAN, this clinical problem should be considered in the differential diagnosis.Especially patients with peripheral neuropathy, general symptoms, skin lesions and hepatitis B virus infection should be evaluated for cranial nerve involvement in the course of vasculitis.In the case of unclear involvement of the cranial nerves, PAN should also be considered in the differential diagnosis as the cause of symptoms and the first manifestation of the disease.

FRAX 10-year fracture risk in rheumatoid arthritis assessed with and without bone mineral density - are we treating our patients under bDMARDs?

OBJECTIVE: This study aimed to identify the rheumatoid arthritis (RA) patients under biological therapy who have FRAX® scores classified as high fracture risk and to evaluate if they are receiving treatment for osteoporosis (OP). The authors also investigated the intra-individual agreement between FRAX® fracture risk calculated with and without bone mineral density (BMD). METHODS: A single-center retrospective cohort study was performed in a total of 303 patients with RA under biologics. Demographic and clinical data were collected using Rheumatic Diseases Portuguese Register (Reuma.pt), complemented with data from the hospital clinical records. FRAX scores with and without BMD were calculated. The Kendall's Tau coefficient was used to assess the agreement between FRAX risk categories. Correlations were evaluated by the Spearman test. Comparisons of distributions from independent variables used the Mann-Whitney test. RESULTS: When FRAX® score was calculated without BMD (n=303), 25% patients were categorized as high fracture risk. Among them, only 54% were receiving OP treatment. FRAX® assessment with BMD (n=231) identified 33% patients with high fracture risk, 52% in treatment for OP. Thirty patients (21%) previously classified as low fracture risk using FRAX® without BMD were recategorized as high risk (𝜏=0.570, p.

Outcome of Uveitis in Juvenile Idiopathic Arthritis and Spondyloarthritis Patients - A 5-Year Follow-Up Study.

OBJECTIVE: Uveitis is a frequent complication of juvenile idiopathic arthritis (JIA) and spondyloarthritis (SpA). The aim of this study is to evaluate the prevalence and risk factors for complications associated with uveitis in patients with JIA and SpA. METHODS: A longitudinal, monocentric cohort study that included patients diagnosed with JIA and SpA who developed uveitis. Demographic, laboratory, and clinical data were collected including complications of uveitis, HLA-B27, antinuclear antibodies, erythrocyte sedimentation rate, C-reactive protein, visual acuity and DMARD treatment. Comparison between groups (complicated versus uncomplicated uveitis) was evaluated using chi-square, t test and Mann-Whitney U test. Logistic regression was performed to determine predictors of complications. RESULTS: A total of 270 patients were evaluated, of which 37 patients (13.7%) had uveitis and were included in this study. Twenty patients were female (54.1%), aged 11.9±8.7 years at diagnosis of SpA/JIA and 15.3±9.9 years at diagnosis of uveitis. Twenty-seven patients (73.0%) had a diagnosis of JIA (23 with oligoarticular disease) and in 12 patients (32.4%) uveitis was the first manifestation. Fifteen (40.5%) patients exhibited complications during follow-up period. Eleven patients (29.7%) underwent ophthalmologic surgery. Complications were significantly higher in patients with JIA (51.9% vs 10.0% in SpA, p=0.03), as was the need for surgery (40.7% vs 0%, p=0.02). Complications in JIA were significantly more frequent in patients who had uveitis as the initial presentation (50.0% vs 7.7%, p=0.03); no significant differences were found between the groups in the other variables studied. Univariate logistic regression analysis showed that uveitis as the first manifestation of JIA (OR 12.0, confidence interval 95% 1.21-118.89, p=0.03) is a significant predictor of complications. CONCLUSION: We found higher rates of complications and need for ophthalmologic surgery in patients with JIA-associated uveitis. The initial presentation of JIA as uveitis is significantly associated with the occurrence of uveitis complications, so it is essential that there is a collaboration between ophthalmologist and rheumatologist in the diagnosis and treatment of these patients.

Cumulative dose and length of methotrexate treatment were not shown to be predictors of hepatic fibrosis by elastography - a monocentric cohort study.

OBJECTIVE: Methotrexate is used in several inflammatory diseases, such as rheumatoid arthritis (RA), spondyloarthritis (SpA) or inflammatory bowel disease (IBD). There has been some controversy regarding methotrexate liver toxicity, especially since the use of newer techniques. We aim to evaluate the prevalence of liver injury in methotrexate-treated patients with inflammatory diseases. METHODS: We performed a cross-sectional study where consecutive patients diagnosed with RA, SpA or IBD, treated with methotrexate, were submitted to liver elastography. The cutoff for fibrosis was ≥7.1 kPa. Comparisons between groups were evaluated using chi-square, t test and Mann-Whitney U test. Correlations were made between continuous variables using Spearman correlation. Logistic regression was performed to determine predictors of fibrosis. RESULTS: A total of 101 patients were included, 60 (59.4%) females, aged 46.2±12.6 years. Eleven patients (10.9%) had fibrosis, with a median score of 4.8 (4.1-5.9) kPa. Patients with fibrosis had higher rates of daily alcohol consumption (63.6% vs 31.1%, p=0.045). Methotrexate exposure time (OR 1.001, 95% CI 0.999-1.003, p=0.549) and cumulative dose (OR 1.000, 95% CI 1000-1000, p=0.629) were shown not to be predictors of fibrosis, unlike alcohol (OR 3.875, 95% CI 1.049-14.319, p=0.042). In multivariate logistic regression analysis, methotrexate cumulative and exposure times were not predictors of significant fibrosis, even when adjusted for alcohol consumption. CONCLUSIONS: In this study, we found that fibrosis detected on hepatic elastography was not associated with methotrexate, unlike alcohol. Therefore, it is of paramount importance to redefine risk factors for liver toxicity in patients with inflammatory diseases under treatment with methotrexate.