Impaired health-related quality of life in idiopathic inflammatory myopathies: a cross-sectional analysis from the COVAD-2 e-survey

Objectives To investigate health-related quality of life in patients with idiopathic inflammatory myopathies (IIMs) compared with those with non-IIM autoimmune rheumatic diseases (AIRDs), non-rheumatic autoimmune diseases (nrAIDs) and without autoimmune diseases (controls) using Patient-Reported Outcome Measurement Information System (PROMIS) instrument data obtained from the second COVID-19 vaccination in autoimmune disease (COVAD-2) e-survey database. Methods Demographics, diagnosis, comorbidities, disease activity, treatments and PROMIS instrument data were analysed. Primary outcomes were PROMIS Global Physical Health (GPH) and Global Mental Health (GMH) scores. Factors affecting GPH and GMH scores in IIMs were identified using multivariable regression analysis. Results We analysed responses from 1582 IIM, 4700 non-IIM AIRD and 545 nrAID patients and 3675 controls gathered through 23 May 2022. The median GPH scores were the lowest in IIM and non-IIM AIRD patients {13 [interquartile range (IQR) 10–15] IIMs vs 13 [11–15] non-IIM AIRDs vs 15 [13–17] nrAIDs vs 17 [15–18] controls, P < 0.001}. The median GMH scores in IIM patients were also significantly lower compared with those without autoimmune diseases [13 (IQR 10–15) IIMs vs 15 (13–17) controls, P < 0.001]. Inclusion body myositis, comorbidities, active disease and glucocorticoid use were the determinants of lower GPH scores, whereas overlap myositis, interstitial lung disease, depression, active disease, lower PROMIS Physical Function 10a and higher PROMIS Fatigue 4a scores were associated with lower GMH scores in IIM patients. Conclusion Both physical and mental health are significantly impaired in IIM patients, particularly in those with comorbidities and increased fatigue, emphasizing the importance of patient-reported experiences and optimized multidisciplinary care to enhance well-being in people with IIMs

Repositioning of the global epicentre of non-optimal cholesterol

High blood cholesterol is typically considered a feature of wealthy western countries1,2. However, dietary and behavioural determinants of blood cholesterol are changing rapidly throughout the world3 and countries are using lipid-lowering medications at varying rates. These changes can have distinct effects on the levels of high-density lipoprotein (HDL) cholesterol and non-HDL cholesterol, which have different effects on human health4,5. However, the trends of HDL and non-HDL cholesterol levels over time have not been previously reported in a global analysis. Here we pooled 1,127 population-based studies that measured blood lipids in 102.6 million individuals aged 18 years and older to estimate trends from 1980 to 2018 in mean total, non-HDL and HDL cholesterol levels for 200 countries. Globally, there was little change in total or non-HDL cholesterol from 1980 to 2018. This was a net effect of increases in low- and middle-income countries, especially in east and southeast Asia, and decreases in high-income western countries, especially those in northwestern Europe, and in central and eastern Europe. As a result, countries with the highest level of non-HDL cholesterol—which is a marker of cardiovascular risk—changed from those in western Europe such as Belgium, Finland, Greenland, Iceland, Norway, Sweden, Switzerland and Malta in 1980 to those in Asia and the Pacific, such as Tokelau, Malaysia, The Philippines and Thailand. In 2017, high non-HDL cholesterol was responsible for an estimated 3.9 million (95% credible interval 3.7 million–4.2 million) worldwide deaths, half of which occurred in east, southeast and south Asia. The global repositioning of lipid-related risk, with non-optimal cholesterol shifting from a distinct feature of high-income countries in northwestern Europe, north America and Australasia to one that affects countries in east and southeast Asia and Oceania should motivate the use of population-based policies and personal interventions to improve nutrition and enhance access to treatment throughout the world.</p

Reducing the environmental impact of surgery on a global scale: systematic review and co-prioritization with healthcare workers in 132 countries

Background Healthcare cannot achieve net-zero carbon without addressing operating theatres. The aim of this study was to prioritize feasible interventions to reduce the environmental impact of operating theatres. Methods This study adopted a four-phase Delphi consensus co-prioritization methodology. In phase 1, a systematic review of published interventions and global consultation of perioperative healthcare professionals were used to longlist interventions. In phase 2, iterative thematic analysis consolidated comparable interventions into a shortlist. In phase 3, the shortlist was co-prioritized based on patient and clinician views on acceptability, feasibility, and safety. In phase 4, ranked lists of interventions were presented by their relevance to high-income countries and low–middle-income countries. Results In phase 1, 43 interventions were identified, which had low uptake in practice according to 3042 professionals globally. In phase 2, a shortlist of 15 intervention domains was generated. In phase 3, interventions were deemed acceptable for more than 90 per cent of patients except for reducing general anaesthesia (84 per cent) and re-sterilization of ‘single-use’ consumables (86 per cent). In phase 4, the top three shortlisted interventions for high-income countries were: introducing recycling; reducing use of anaesthetic gases; and appropriate clinical waste processing. In phase 4, the top three shortlisted interventions for low–middle-income countries were: introducing reusable surgical devices; reducing use of consumables; and reducing the use of general anaesthesia. Conclusion This is a step toward environmentally sustainable operating environments with actionable interventions applicable to both high– and low–middle–income countries

A century of trends in adult human height

Being taller is associated with enhanced longevity, and higher education and earnings. We reanalysed 1472 population-based studies, with measurement of height on more than 18.6 million participants to estimate mean height for people born between 1896 and 1996 in 200 countries. The largest gain in adult height over the past century has occurred in South Korean women and Iranian men, who became 20.2 cm (95% credible interval 17.5-22.7) and 16.5 cm (13.3-19.7) taller, respectively. In contrast, there was little change in adult height in some sub-Saharan African countries and in South Asia over the century of analysis. The tallest people over these 100 years are men born in the Netherlands in the last quarter of 20th century, whose average heights surpassed 182.5 cm, and the shortest were women born in Guatemala in 1896 (140.3 cm; 135.8-144.8). The height differential between the tallest and shortest populations was 19-20 cm a century ago, and has remained the same for women and increased for men a century later despite substantial changes in the ranking of countries

Repositioning of the global epicentre of non-optimal cholesterol

High blood cholesterol is typically considered a feature of wealthy western countries1,2. However, dietary and behavioural determinants of blood cholesterol are changing rapidly throughout the world3 and countries are using lipid-lowering medications at varying rates. These changes can have distinct effects on the levels of high-density lipoprotein (HDL) cholesterol and non-HDL cholesterol, which have different effects on human health4,5. However, the trends of HDL and non-HDL cholesterol levels over time have not been previously reported in a global analysis. Here we pooled 1,127 population-based studies that measured blood lipids in 102.6 million individuals aged 18 years and older to estimate trends from 1980 to 2018 in mean total, non-HDL and HDL cholesterol levels for 200 countries. Globally, there was little change in total or non-HDL cholesterol from 1980 to 2018. This was a net effect of increases in low- and middle-income countries, especially in east and southeast Asia, and decreases in high-income western countries, especially those in northwestern Europe, and in central and eastern Europe. As a result, countries with the highest level of non-HDL cholesterol�which is a marker of cardiovascular risk�changed from those in western Europe such as Belgium, Finland, Greenland, Iceland, Norway, Sweden, Switzerland and Malta in 1980 to those in Asia and the Pacific, such as Tokelau, Malaysia, The Philippines and Thailand. In 2017, high non-HDL cholesterol was responsible for an estimated 3.9 million (95 credible interval 3.7 million�4.2 million) worldwide deaths, half of which occurred in east, southeast and south Asia. The global repositioning of lipid-related risk, with non-optimal cholesterol shifting from a distinct feature of high-income countries in northwestern Europe, north America and Australasia to one that affects countries in east and southeast Asia and Oceania should motivate the use of population-based policies and personal interventions to improve nutrition and enhance access to treatment throughout the world. © 2020, The Author(s), under exclusive licence to Springer Nature Limited

A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a mysterious disorder characterized by periodically recurrent fevers, oropharyngeal inflammation, and adenitis, which mainly affects children, though in very recent times, it has been also recognized in adulthood. We enrolled 115 unrelated pediatric and adult patients with history of periodic fevers who fulfilled the current diagnostic criteria for PFAPA syndrome in three Italian referral centers and highlighted differences between children and adults. Eighty-five children and 30 adults were evaluated: the frequency of flares was significantly higher in pediatric cases, while febrile attack duration was significantly longer in adults. Clockwork periodicity of fever and recurrent pharyngitis were more frequently observed in childhood, but no differences were identified for aphthosis and cervical adenopathy. Conversely, joint symptoms, myalgia, headache, fatigue, ocular signs, and rashes were more common in adults. The simultaneous occurrence of two or three cardinal PFAPA signs did not show any statistical difference between the groups, while the occurrence of only one cardinal manifestation was more frequent in adults. Corticosteroids were effective in 98.82&nbsp;% of children and 88.2&nbsp;% of adults. Tonsillectomy was rarely performed, resulting effective in only two patients. Our data illustrate the clinical overlap between pediatric and adult cases of PFAPA syndrome. Adults are characterized by a wider repertoire of inflammatory signs, suggesting that onset in adulthood might leave the disease misdiagnosed. Clinicians, not only pediatricians, should take into account this clinical entity in every patient of whatever age suffering from recurrent fevers of unknown origin

Is There A Role For Tnf-α Antagonists In The Treatment Of Ssc? Eustar Expert Consensus Development Using The Delphi Technique

Objective: To obtain experiences and expert opinion on treatment of SSc patients with TNF-α antagonists. Methods: An investigation was carried out among the EUSTAR centres into their expertise on use of TNF-α antagonists. Assessment forms on the frequency of TNF-α inhibitor use were distributed to EULAR Scleroderma Trials and Research Group (EUSTAR) centres. Afterwards, a three round Delphi exercise was performed to obtain expert consensus on the use of TNF-α inhibitors in SSc. Results: Seventy-nine centres returned information on use of TNF-α antagonists in SSc patients. A total of 65 patients were treated with TNF-a inhibitors in 14 different centres. Forty-eight of the 65 patients treated with TNF-α inhibitors improved. Improvement was mainly seen in patients with arthritis, whereas the effects on fibrosis varied. In the first round of the subsequent Delphi approach, 71 out of 79 experts stated that they would use TNF-α antagonists in SSc. Arthritis was suggested as an indication for TNFa antagonists by 75% of the experts. However, after the third stage of the Delphi exercise, the acceptance for the off-label use of TNF-a antagonists decreased and 59% recommended that TNF-α antagonists should not be used or only used in clinical trials in SSc patients, while 38% of the experts suggested the use of TNF-a antagonists for arthritis associated with SSc. Conclusions: Most of the experts do not recommend the routine use of TNF-a antagonists in systemic sclerosis. Arthritis might be a potential indication in SSc, although controlled clinical trials with TNF-α antagonists are needed before general recommendations can be given. © Copyright Clinical and Experimental Rheumatology 2011.292 SUPPL. 65S40S45Keyser, F.D., Mielants, H., Veys, E.M., Current use of biologicals for the treatment of spondyloarthropathies (2001) Expert Opin Pharmacother, 2, pp. 85-93Olsen, N.J., Stein, C.M., New drugs for rheumatoid arthritis (2004) N Engl J Med, 350, pp. 2167-2179Chua, C.C., Chua, B.H., Tumor necrosis factor-alpha induces mRNA for collagenase and TIMP in human skin fibroblasts (1990) Connect Tissue Res, 25, pp. 161-170Mauviel, A., Daireaux, M., Redini, F., Galera, P., Loyau, G., Pujol, J.P., Tumor necrosis factor inhibits collagen and fibronectin synthesis in human dermal fibroblasts (1988) FEBS Lett, 236, pp. 47-52Liu, J.Y., Brass, D.M., Hoyle, G.W., Brody, A.R., TNF-alpha receptor knockout mice are protected from the fibroproliferative effects of inhaled asbestos fibers (1998) Am J Pathol, 153, pp. 1839-1847Piguet, P.F., Vesin, C., Treatment by human recombinant soluble TNF receptor of pulmonary fibrosis induced by bleomycin or silica in mice (1994) Eur Respir J, 7, pp. 515-518Khan, S.B., Cook, H.T., Bhangal, G., Smith, J., Tam, F.W., Pusey, C.D., Antibody blockade of TNF-alpha reduces inflammation and scarring in experimental crescentic glomerulonephritis (2005) Kidney Int, 67, pp. 1812-1820Distler, J.H., Schett, G., Gay, S., Distler, O., The controversial role of tumor necrosis factor alpha in fibrotic diseases (2008) Arthritis Rheum, 58, pp. 2228-2235Varga, J., Abraham, D., Systemic sclerosis: A prototypic multisystem fibrotic disorder (2007) J Clin Invest, 117, pp. 557-567Gabrielli, A., Avvedimento, E.V., Krieg, T., Scleroderma (2009) N Engl J Med, 360, pp. 1989-2003Bosello, S., De Santis, M., Tolusso, B., Zoli, A., Ferraccioli, G., Tumor necrosis factor-alpha inhibitor therapy in erosive polyarthritis secondary to systemic sclerosis (2005) Ann Intern Med, 143, pp. 918-920Antoniou, K.M., Mamoulaki, M., Malagari, K., Infliximab therapy in pulmonary fibrosis associated with collagen vascular disease (2007) Clin Exp Rheumatol, 25, pp. 23-28Bargagli, E., Galeazzi, M., Bellisai, F., Volterrani, L., Rottoli, P., Infliximab treatment in a patient with systemic sclerosis associated with lung fibrosis and pulmonary hypertension (2008) Respiration, 75, pp. 346-349Denton, C.P., Engelhart, M., Tvede, N., An open-label pilot study of infliximab therapy in diffuse cutaneous systemic sclerosis (2009) Ann Rheum Dis, 68, pp. 1433-1439Ellman, M.H., Macdonald, P.A., Katz, R.S., Open label use of etanercept in eight scleroderma patients (2003) Ann Rheum Dis, 62 (SUPPL. I), p. 229Lam, G.K., Hummers, L.K., Woods, A., Wigley, F.M., Efficacy and safety of etanercept in the treatment of scleroderma-associated joint disease (2007) J Rheumatol, 34, pp. 1636-1637. , JulAlexis, A.F., Strober, B.E., Off-label dermatologic uses of anti-TNF-α therapies (2005) J Cutan Med Surg, 9, pp. 296-302Avouac, J., Walker, U., Tyndall, A., Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: Results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database (2010) J Rheumatol, 37, pp. 1488-1501Khanna, D., Agrawal, H., Clements, P.J., Infliximab may be effective in the treatment of steroid-resistant eosinophilic fasciitis: Report of three cases (2010) Rheumatology, 49, pp. 1184-1188. , OxfordTzaribachev, N., Holzer, U., Schedel, J., Maier, V., Klein, R., Kuemmerle-Deschner, J., Infliximab effective in steroid-dependent juvenile eosinophilic fasciitis (2008) Rheumatology, 47, pp. 930-932. , OxfordAllanore, Y., Devos-Francois, G., Caramella, C., Boumler, P., Jounieaux, V., Kahan, A., Fatal exacerbation of fibrosing alveolitis associated with systemic sclerosis in a patient treated with adalimumab (2006) Ann Rheum Dis, 65, pp. 834-835Marie, I., Lahaxe, L., Levesque, H., Hellot, P., Pulmonary actinomycosis in a patient with diffuse systemic sclerosis treated with infliximab (2008) QJM, 101, pp. 419-421Ostor, A.J., Crisp, A.J., Somerville, M.F., Scott, D.G., Fatal exacerbation of rheumatoid arthritis associated fibrosing alveolitis in patients given infliximab (2004) BMJ, 329, p. 1266Amjadi, S., Maranian, P., Furst, D.E., Course of the modified Rodnan skin thickness score in systemic sclerosis clinical trials: Analysis of three large multicentre, double-blind, randomized controlled trials (2009) Arthritis Rheum, 60, pp. 2490-2498Denton, C.P., Merkel, P.A., Furst, D.E., Recombinant human anti-transforming growth factor beta1 antibody therapy in systemic sclerosis: A multicenter, randomized, placebo-controlled phase I/II trial of CAT-192 (2007) Arthritis Rheum, 56, pp. 323-333Kaldas, M., Khanna, P.P., Furst, D.E., Sensitivity to change of the modified Rodnan skin score in diffuse systemic sclerosis-assessment of individual body sites in two large randomized controlled trials (2009) Rheumatology, 48, pp. 1143-1146. , OxfordPostlethwaite, A.E., Wong, W.K., Clements, P., A multicenter, randomized, double-blind, placebo-controlled trial of oral type I collagen treatment in patients with diffuse cutaneous systemic sclerosis: I. Oral type I collagen does not improve skin in all patients, but may improve skin in late-phase disease (2008) Arthritis Rheum, 58, pp. 1810-1822Walker, U.A., Tyndall, A., Czirjak, L., Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database (2007) Ann Rheum Dis, 66, pp. 754-763Linstone, H., Turoff, M., (2002) The Delphi Method. Techniques and Applications, , New Jersey Institute of Technology, California, USAJones, J., Hunter, D., Consensus methods for medical and health services research (1995) BMJ, 311, pp. 376-380Generini, S., Steiner, G., Miniati, I., Anti-hnRNP and other autoantibodies in systemic sclerosis with joint involvement (2009) Rheumatology, 48, pp. 920-925. , OxfordMatucci-Cerinic, M., Allanore, Y., Czirjak, L., The challenge of early systemic sclerosis for the EULAR Scleroderma Trial and Research group (EUSTAR) community. It is time to cut the Gordian knot and develop a prevention or rescue strategy (2009) Ann Rheum Dis, 68, pp. 1377-138