Hypoperfusive and hypertensive ocular manifestations in Takayasu arteritis

Takayasu arteritis is a relatively rare inflammatory arteritis that can be associated with ocular manifestations. We report four patients with proven Takayasu arteritis; two patients manifested hypoperfusive ocular manifestations of ocular ischemic syndrome and anterior ischemic optic neuropathy whilst two others had exudative retinal detachment and papilledema as a result of severe hypertension. The ischemic ocular manifestations were a result of hypoperfusion of the ocular structures due to occlusive arteritis of the aortic arch and its branches. The exudative retinal detachment and papilledema were manifestations of severe hypertension due to renal arterial involvement. Patients with Takayasu arteritis should be referred for ophthalmic assessment and screening for hypoperfusive and hypertensive manifestations

Mental healthcare for young and adolescent LGBTQ+ individuals in the Indian subcontinent

The coronavirus disease (COVID-19) pandemic has led to a significant change in the way healthcare is dispensed. During the pandemic, healthcare inequities were experienced by various sections of society, based on gender, ethnicity, and socioeconomic status. The LGBTQ individuals were also affected by this inequity. There is a lack of information on this topic especially in the developing countries. Hence this issue requires further exploration and understanding. Previous literature briefly explored the mental, physical, and emotional turmoil faced by the LGBTQ community on a regular basis. They feared rejection by family and friends, bullying, physical assault, and religious biases. These issues prevented them from publicly speaking about their sexual orientation thereby making it difficult to collect reliable data. Although they require medical and psychological treatment, they are afraid to ask for help and access healthcare and mental health services. Being mindful of these difficulties, this article explores the various underlying causes of the mental health problems faced by LGBTQ individuals, especially, in the Indian subcontinent. The article also examines the status of healthcare services available to Indian sexual minorities and provides recommendations about possible remedial measures to ensure the well-being of LGBTQ individuals

Coalition for Health and Gender Equity (CHANGE)—a protocol for a global cross-sectional survey of health and gender equity in rheumatology

Lay Summary: What does this mean for patients? The CHANGE Study, led by a team of rheumatology professionals worldwide, is working to make health care more equal for everyone. We are focusing on challenges faced by rheumatologists, such as fair pay and career opportunities. To understand these issues better, the team is gathering information through a global survey of rheumatology professionals. The goal is to find out why there are differences and come up with solutions. Ultimately, the aim is to create a fair and inclusive environment in rheumatology, ensuring that everyone has the same chances to grow in their careers, regardless of their gender. The findings of the study will help to create better guidelines, promoting fairness and equality for health-care professionals in rheumatology

How immunological profle drives clinical phenotype of primary Sjögren’s syndrome at diagnosis: analysis of 10,500 patients (Sjögren Big Data Project)

To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjögren´s syndrome (SjS).METHODS:The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres shared a harmonised data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfillment of the 2002 classification criteria. Immunological tests were carried out using standard commercial assays.RESULTS:By January 2018, the participant centres had included 10,500 valid patients from 22 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti- La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementaemia and especially cryoglo-bulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p<0.001) in the organ-by-organ ESS- DAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains).CONCLUSIONS:We confirm the strong influence of immunological markers on the phenotype of primary SjS at diagnosis in the largest multi-ethnic international cohort ever analysed, with a greater influence for cryoglobulinaemic-related markers in comparison with Ro/La autoantibodies and ANA. Immunological patterns play a central role in the phenotypic expression of the disease already at the time of diagnosis, and may guide physicians to design a specific personalised management during the follow-up of patients with primary SjS.Fil: Brito Zerón, Pilar. Hospital Sanitas CIMA; España. Universidad de Barcelona; EspañaFil: Acar Denizli, Nihan. Mimar Sinan Fine Arts University; TurquíaFil: Ng, Wan Fai. University of Newcastle; Reino UnidoFil: Zeher, Margit. University of Debrecen; HungríaFil: Rasmussen, Astrid. Oklahoma Medical Research Foundation; Estados UnidosFil: Mandl, Thomas. Lund University; SueciaFil: Seror, Raphaele. Université Paris Sud; FranciaFil: Xiaolin, Li. Anhui Provincial Hospital; ChinaFil: Baldini, Chiara. Università degli Studi di Pisa; ItaliaFil: Gottenberg, Jaques. Université de Strasbourg; Francia. Centre National de la Recherche Scientifique; FranciaFil: Danda, Debashish. Christian Medical College & Hospital; IndiaFil: Quartuccio, Luca. University Hospital “Santa María della Misericordia”; ItaliaFil: Priori, Roberta. Università degli Studi di Roma "La Sapienza"; ItaliaFil: Hernandez Molina, Gabriela. Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán; MéxicoFil: Armagan, Berkan. Hacettepe University. Faculty of Medicine.Department of Internal Medicine; TurquíaFil: Kruize, Aike. University Medical Center Utrecht; Países BajosFil: Kwok, Seung Ki. The Catholic University of Korea; Corea del SurFil: Kvarnström, Marika. Karolinska University Hospital.Department of Medicine.Unit of Rheumatology. Karolinska Institutet ; SueciaFil: Praprotnik, Sonja. University Medical Centre; EsloveniaFil: Sene, Damien. Université Paris Diderot - Paris 7; FranciaFil: Bartoloni, Elena. Università di Perugia; ItaliaFil: Solans, R.. Hospital Vall d’Hebron; ItaliaFil: Rischmueller, M.. University of Western Australia; AustraliaFil: Suzuki, Y.. Kanazawa University Hospital; JapónFil: Isenberg, D. A.. University College London; Estados UnidosFil: Valim, V.. Federal University of Espírito Santo; BrasilFil: Wiland, P.. Wroclaw Medical Hospital; PoloniaFil: Nordmark, G.. Uppsala Universitet; SueciaFil: Fraile, G.. Hospital Ramón y Cajal; EspañaFil: Retamozo, Maria Soledad. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Hospital Privado Centro Medico de Córdoba; Argentina; Argentina. Instituto Universitario de Ciencias Biomédicas de Córdoba; Argentin

Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjogren's syndrome

Objective: To characterize the systemic phenotype of primary Sjögren’s syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren’s syndrome from the five continents. Results: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P &lt; 0.001) compared with females, as did patients diagnosed at &lt;35 years (6.7 vs 5.6 in patients diagnosed at &gt;65 years, P &lt; 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P &lt; 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P &lt; 0.001). Conclusion: The systemic phenotype of primary Sjögren’s syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.

Basic science232. Certolizumab pegol prevents pro-inflammatory alterations in endothelial cell function

Background: Cardiovascular disease is a major comorbidity of rheumatoid arthritis (RA) and a leading cause of death. Chronic systemic inflammation involving tumour necrosis factor alpha (TNF) could contribute to endothelial activation and atherogenesis. A number of anti-TNF therapies are in current use for the treatment of RA, including certolizumab pegol (CZP), (Cimzia ®; UCB, Belgium). Anti-TNF therapy has been associated with reduced clinical cardiovascular disease risk and ameliorated vascular function in RA patients. However, the specific effects of TNF inhibitors on endothelial cell function are largely unknown. Our aim was to investigate the mechanisms underpinning CZP effects on TNF-activated human endothelial cells. Methods: Human aortic endothelial cells (HAoECs) were cultured in vitro and exposed to a) TNF alone, b) TNF plus CZP, or c) neither agent. Microarray analysis was used to examine the transcriptional profile of cells treated for 6 hrs and quantitative polymerase chain reaction (qPCR) analysed gene expression at 1, 3, 6 and 24 hrs. NF-κB localization and IκB degradation were investigated using immunocytochemistry, high content analysis and western blotting. Flow cytometry was conducted to detect microparticle release from HAoECs. Results: Transcriptional profiling revealed that while TNF alone had strong effects on endothelial gene expression, TNF and CZP in combination produced a global gene expression pattern similar to untreated control. The two most highly up-regulated genes in response to TNF treatment were adhesion molecules E-selectin and VCAM-1 (q 0.2 compared to control; p > 0.05 compared to TNF alone). The NF-κB pathway was confirmed as a downstream target of TNF-induced HAoEC activation, via nuclear translocation of NF-κB and degradation of IκB, effects which were abolished by treatment with CZP. In addition, flow cytometry detected an increased production of endothelial microparticles in TNF-activated HAoECs, which was prevented by treatment with CZP. Conclusions: We have found at a cellular level that a clinically available TNF inhibitor, CZP reduces the expression of adhesion molecule expression, and prevents TNF-induced activation of the NF-κB pathway. Furthermore, CZP prevents the production of microparticles by activated endothelial cells. This could be central to the prevention of inflammatory environments underlying these conditions and measurement of microparticles has potential as a novel prognostic marker for future cardiovascular events in this patient group. Disclosure statement: Y.A. received a research grant from UCB. I.B. received a research grant from UCB. S.H. received a research grant from UCB. All other authors have declared no conflicts of interes

Are Indian patients with juvenile-onset ankylosing spondylitis taller than reference population ?

Background & objectives: Paucity of growth retardation has been observed by us in patients with juvenile-onset ankylosing spondylitis (JAS) in a tertiary care health centre in south India. We, therefore, undertook this pilot study to assess and compare anthropometry of patients with JAS who were 15 yr and older with that of adult onset ankylosing spondylitis (AAS) and matching Indian reference population. Methods: Consecutive male patients (December 2009- October 2012) with JAS and AAS fulfilling Modified New York Criteria were selected after applying inclusion and exclusion criteria. Demography and anthropometry were noted. Height of both patient groups as well as their parents and siblings were compared with that of the reference population. Mid-parental height and delta height were derived. Those with delta height of >8.5 cm were compared with the remaining. Multivariate logistic regression was done for variables that were found to be significant by chi-square in bivariate analysis. Similar analysis was done for BMI also. Results: There was no significant difference in anthropometric variables between JAS and AAS groups. Twenty eight of the 30 (93.33%) JAS patients were taller as compared to the reference population. Twenty six (86.67%) AAS patients were taller than the reference population. The mean heights of JAS (170.67 ± 6.94 cm) and AAS (168.2 ± 5.94 cm) patients were significantly higher than the reference value of 163.11 cm; both p0 <0.001. Logistic regression revealed that tallness in JAS was associated positively with hypermobility (OR=23.46,95%CI 1.2-447.2, p0 =0.036). No significant association was detected for height in AAS and for BMI in both JAS and AAS groups. Interpretation & conclusions: No growth retardation was seen in patients with JAS in our study. Majority of patients with JAS and AAS were taller than reference population. The difference between mean height of JAS and AAS was not significant. Larger studies involving different populations are required to confirm these findings

A descriptive pilot study of mitochondrial mutations & clinical phenotype in fibromyalgia syndrome

Background & objectives: Fibromyalgia syndrome (FMS) is one of the most common chronic pain conditions of unknown aetiology. Mitochondrial dysfunction has been reported in FMS with some studies reporting the presence of mitochondrial mutation namely A3243G, which also causes mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes. This pilot study was conducted to assess this mutation and also detect large deletions in mitochondrial DNA (mtDNA) in patients with FMS. Methods: Thirty female patients with FMS participated and 30 matched controls were included. Genomic DNA was subjected to polymerase chain reaction (PCR) amplification using specific primers followed by restriction digestion with Apa I enzyme to detect the specific A3243G mtDNA mutation. Long-range PCR was done in two sets to detect the large deletions in the mtDNA. Biochemical parameters including thyroid-stimulating hormone and vitamin D levels were also looked at. Results: None of the patients were found to carry the common mutation or large deletions. Low vitamin D level was a common finding. Hypothyroidism was found in a few patients. Interpretation & conclusions: Although the common mutation or large mtDNA deletions were not detected in blood mtDNA in the FMS patients, mutations in the muscle and sequence variation in mtDNA remained a possibility. Future studies in both blood and muscle tissue including mtDNA sequencing are warranted in such patients to determine if a subset of FMS patients have mitochondrial myopathy

Management of patients with fibromyalgia using biofeedback: A randomized control trial

Objective: Fibromyalgia syndrome (FMS) is a chronic rheumatological condition which could be characterized by generalized pain and fatigue. Cognitive and behavioral therapy has been found to be a suitable technique in the management of FMS. This study intends to evaluate the efficacy of electromyography (EMG) biofeedback to reduce pain in patients with FMS. Materials and Methods: A randomized controlled trial involving two groups of FMS patients, one receiving EMG biofeedback and the other a sham biofeedback, was carried out. The assessment tools included in the study were fibromyalgia impact questionnaire (FIQ), visual analogue scale (VAS), six-minute walk test (SMWT) and number of tender points; and tenderness of each tender point was done for both the groups. Statistics: A Student′s ′t′ test was used to study the test for significance. Results: After using biofeedback, the mean VAS scores and the mean number of tender points were found to be 3 out of 10 and 6 out of 18 respectively. Subjective analysis from both groups showed improvement in physical and psychological realms. Statistical significance (P < 0.001) was observed for decrease in pain and number of tender points. ANOVA analysis revealed a significant decrease in VAS, FIQ and tender points within the groups. Conclusion: Biofeedback as a treatment modality reduces pain in patients with FMS, along with improvements in FIQ, SMWT and the number of tender points