1,721,139 research outputs found
Systemic sclerosis and primary biliary cholangitis: An overlapping entity?
No full textSystemic sclerosis (SSc) is a complex autoimmune disease that may lead to skin and internal organ fibrosis. Based on skin involvement, two subsets of the disease are recognized (limited cutaneous SSc and diffuse cutaneous SSc). The new 2013 American College of Rheumatology/European League against Rheumatism classification criteria allow to identify SSc patients at the early stage of the disease that allows new research avenues. The aetiology of the disease is still unknown, but it has an important autoimmune basis and its association with other autoimmune diseases has been reproducibly reported. Among them, primary biliary cholangitis is considered the most common liver disease in SSc. The aim of this review is to provide an overview on recent findings about SSc associated to primary biliary cholangitis. Although the aetiology of the two diseases is still unknown, data suggest that these two disorders share the expression of fibrogenic cytokines, involved both in generation and function of T lymphocytes subpopulation (Th17 cells) and regulatory T lymphocytes. In addition, the relationships between SSc and primary biliary cholangitis may be closer as suggested by the presence of primary biliary cholangitis–specific antibodies in SSc patients and vice versa. Recent findings confirm a prevalence of overt primary biliary cholangitis in about 2% of SSc population, in particular in patients with limited cutaneous SSc and positive anticentromere antibodies. The prevalence increases if also patients with only primary biliary cholangitis–specific antibodies are considered. Data regarding SSc prevalence in primary biliary cholangitis patients have also been recently clarified. Altogether, stimulating results are moving the field forward regarding the relationships of these two autoimmune and fibrotic disorders that may belong to an overlapping entity
Sintomatologia gastrointestinale e qualità di vita dei pazienti affetti da Sclerosi Sistemica
No full textRazionale e scopo del lavoro La Sclerosi Sistemica SSc è una malattia autoimmune caratterizzata da fibrosi della cute e degli organi interni e da deficit angiogenico, causando l’alterazione della microcircolazione e quindi della irrorazione sanguigna di cute e organi Anche il tratto gastro intestinale risulta interessato da tale fenomeno, infatti causa una vasta sintomatologia che può coinvolgere esofago, stomaco e intestino Lo scopo dello studio è, quindi, la valutazione dell’interessamento gastro intestinale attraverso il questionario UCLA SCTC GIT 2 0 e del livello di aderenza alla dieta mediterranea misurato attraverso il Mediterranean Diet Score di Sofi et al. Materiali e metodi Nel periodo da gennaio ad aprile 2019 sono stati arruolati 18 pazienti affetti da SSc. Ai pazienti inclusi nello studio sono stati somministrati i seguenti questionari UCLA SCTC GIT 2 0 per la valutazione dell’interessamento gastro intestinalerange 0 3 Mediterranean Diet Score (M+DS range 0 -14) per la valutazione dell’aderenza alla dieta mediterranea, Health Assessment Questionniare (range 0-3) per la valutazione della disabilità ed SF 36 (range 0 100) per la valutazione della qualità di vita, inoltre sono stati raccolti anche i dati su peso, altezza per il calcolo dell’Indice di Massa Corporea. Risultati: 18 pazienti affetti da SSc sono stati inclusi nello studio e presentavano un IMC medio di 23.9-4.7 (M DS), risultando pertanto normopeso, solo una paziente presentava un IMC di 16.6 ( e 4 pazienti invece un IMC>25 quindi sovrappeso Per quanto concerne la dieta mediterranea, i nostri risultati mostrano buona aderenza a tale dieta con un valore di 9.78-2.24 (M DS) al MDS Per quanto concerne invece la qualità di vita misurata con SF 36 i due indici sommari mentale 36.32-11.35 e fisico 39.53-8.61 mostrano dei punteggi al di sotto del cut off 50 riportando quindi una qualità di vita generale compromessa inoltre i punteggi medi dell’HAQ mostrano anch’essi che i pazienti affetti da SSc hanno qualche difficoltà nello svolgere le attività di vita quotidiane a causa della malattia 0 52 0 53 M DS). Il coinvolgimento intestinale, misurato con il questionario UCLA GIT 2 0 riporta sintomi moderati 0.50-1.00 per quanto riguarda la maggior parte delle aree ( distensione addominale, funzione sociale e benessere emotivo), mentre un punteggio più basso 0.00-0.49 è stato riscontrato nelle restanti aree ( costipazione e incontinenza fecale) Pertanto, il punteggio totale del coinvolgimento gastro intestinale risulta moderato 0.42- 0.38 M DS
Recent advances steer the future of systemic sclerosis toward precision medicine
No full textRecent advances steer the future of systemic sclerosis toward precision medicine
Critical finger ischemia and myocardial fibrosis development after sudden interruption of sildenafil treatment in a systemic sclerosis patient
No full textSystemic sclerosis (SSc) is a connective tissue disease frequently associated with Raynaud’s Phenomenon (RP). Among possible pharmacological treatments, phosphodiesterase 5 inhibitors are considered in cases of severe non-responsive RP. We present the case of a male SSc patient wh presented with critical finger ischemia and concomitant appearance of myocardial fibrosis after sudden interruption of sildenafil treatment
The COVID-19 pandemic highlights the need for a psychological support in systemic sclerosis patients
No full textSystemic sclerosis (SSc) in a chronic autoimmune disease characterised by internal organs damage and skin fibrosis potentially leading to disability and reducing quality of life (QoL). In SSc, patients must face the fear and uncertainty induced by the disease chronic progression and the changes of the physical appearance leading to body image distress (1). The diminished life expectancy, the body pain, the gastrointestinal involvement and the reduced health-related QoL explains the higher risk of depression observed in SSc patients (2- 4). Depression is reported to be more frequent in women than in men, and is mainly due to decreased QoL, fatigue and reduced physical activity. However it does not impact patients survival (5, 6). Older age, diffuse cutaneous subset (dcSSc) and pulmonary arterial hypertension (PAH) are the major determinants of a lower health-related QoL when measured by the Short Form 36 (SF-36). Moreover, a strong association between disability and depressive symptoms is often reported (7-9). Illness-related uncertainty is one of the main stressors, influencing the behaviour and disease perception, thus leading to an excessive self-examination, ultimately increasing anxiety symptoms (2). Also anxiety is more frequent in SSc female than in male and unrelated to disease severity (10, 11). In the last years, numerous studies reported an impaired health-related QoL and an increased prevalence of psychiatric symptoms among SSc patients (5, 12, 13). In addition, data on psychosocial aspects as body image distress, sexual function and disability are increased (14), reinforcing the need to pay more attention to psychological aspects in SSc patients in routine clinical practice. In the last three years, the attention to patients mental health has globally grown and COVID-19 pandemic has increased the need for the evaluation of psychological symptoms, forcing clinicians to address the problem in every day practice, dealing therefore with an increasing number of patients characterised by psychiatric symptoms (15). Pre-existing medical conditions exposed patients with chronic diseases at major risk of COVID-19 infection and complications. For this reason, SSc patients are at greater risk for COVID19-related stress, boredom, grief, fear, depression and anxiety from the earliest phase of the pandemic (15, 16). In the management of patients with chronic diseases as SSc, the COVID-19 has quickly prompted the attention on psychological aspects, making the clinician aware that a new vision and care of SSc patients was mandatory. Recently, Henry et al. (17) evaluated anxiety and depression in SSc patients in the pre-COVID-19 and during the COVID-19 through March 2021 and assessed the trend of anxiety, depression, fear and loneliness across the pandemic era through March 2022 in patients enrolled in the Scleroderma Patient-centred Intervention Network (SPIN). A previous study focussed the attention on mental health of SSc patients in the early phase of pandemic and on its comparison with pre-COVID-19 status. The analysis of SSc patients from the SPIN Cohort evaluated anxiety and depression symptoms before and after the pandemic reporting an increase in anxiety but not in depression during the COVID-19 (18). Also other data from previous studies confirmed the attention on SSc patients mental health in the first phases of pandemic and on the different support Editorial The COVID-19 pandemic highlights the need for a psychological support in systemic sclerosis patients G. Lepri1 , M. Orlandi1 , S. Bellando-Randone1 , M. Matucci-Cerinic1,2, S. Guiducci1 1576 Clinical and Experimental Rheumatology 2023 COVID-19 and psychological support in SSc patients / G. Lepri et al. programmes conducted to aid patients with chronic disease in such a stressful era (18-21). The study by Henry et al. also assessed the trend of psychological symptoms when patients faced new challenges (17). In fact, at the beginning of pandemic fear and uncertainty of COVID infection were common feelings in patients with chronic diseases (treated with immunosuppressant) and in patients with SSc complications as interstitial lung disease. Afterwards, the attention was focused on other concerns, as vaccines safety, their efficacy, the presence of new variants of virus, the progressive less use of preventive restrictions as masks and social distancing and consequently the recovery of social relations. Therefore, the analysis of Henry et al. reflects not only the fear of the pandemic but also the feelings of patients in managing the everyday living with the virus and in the progressive recovery of social relationships after the pandemic era. The study showed that anxiety increased in April 2020, returned to pre-COVID-19 levels by mid-2020 and remained stable from this period until March 2022. In SSc patients, depression symptoms were stable in the first months of 2020 and then slightly decreased. Loneliness was stable during the study period and COVID-19 fear was high in the early phase and quickly decreased. The trend of psychological symptoms (depression, anxiety, fear and loneliness) was similar across time by subpopulations defined by age, sex, country and disease cutaneous subset and these results were in line with previous data (17, 20). More attention in detecting the differences of psychological symptoms trend in the different SSc sub-populations may be of paramount importance to identify patients at major risk to increase impaired mental health symptoms during stressful events. Some previous data suggested an association between increased fear and reported interference of breathing problems in daily activities, lower economic availability and greater pre-pandemic anxiety levels (22). The possibility to define determinants of mental health worsening in SSc may help to stratify patients, thus applying specific support and preventive programmes on different disease subsets. In the study by Henry et al. depression and anxiety trajectories were not influenced by age or disease subsets: this is not in agreement with previous data regarding mental health symptoms in SSc and disease features in the pre-pandemic era correlating depression to gender, older age, dcSSc and cardiac involvement (PAH), pain or gastrointestinal symptoms (3-5, 7-9). The relationship between anxiety symptoms and disease characteristics were not significant. Moreover, the trend of mental symptoms from pre-COVID-19 to pandemic era could be traced, comparing patients with to those without cardio-pulmonary involvement, knowing the high risk of COVID-19-related complications in patients with pre-existing lung disease. This analysis has not been performed by Henry et al. as well as by other authors, and the trend of psychological symptoms in patients with different organ involvement is still unknown. Another point to consider is the fact that the authors investigated psychological aspects of SSc patients until March 2022 when vaccines were widely available for frail patients with chronic diseases. For this reason, the interest could be focused on fear, on loneliness, on immunosuppressed patients as well as on uncertainty and anxiety to investigate if there may be any correlation to the ongoing therapy. In fact, this specific datum might clarify the therapeutic impact during a stressful period. It is well known that immunosuppressant drugs expose patients to a greater infectious risk, contributing to increase anxiety and/or fear levels, also affecting vaccine efficacy. For this reason, now other retrospective studies are warranted to identify determinants of psychological and psychiatric symptoms identifying the progression of the deterioration from the pre-COVID to the pandemic. This may help to understand what are the patient categories at greatest risk. Unfortunately, the COVID-19 is an extremely stressful model for some subjects including patients with chronic diseases. Therefore, the identification of a subpopulation which is more sensitive and is burdened by a major risk to develop or worsen either depression and anxiety or other metal symptoms, might help the clinician to identify in practice the frailest patients who need early a psychological support to prevent the onset of psychiatric symptoms. The identification of patients at major risk for psychological support suggests a multidisciplinary collaboration (psychologists and psychiatrists) to decide the best support programmes according to patients individual needs. References 1. WALLACE ZS, COOK C, FINKELSTEIN-FOX L et al.: The association of illness-related uncertainty with mental health in systemic autoimmune rheumatic diseases. J Rheumatol 2022; 49(9): 1058-66. https://doi.org/10.3899/jrheum.211084 2. NGUYEN C, RANQUE B, BAUBET T et al.: Clinical, functional and health-related quality of life correlates of clinically significant symptoms of anxiety and depression in patients with systemic sclerosis: a cross-sectional survey. PLoS One 2014; 9(2): e90484. https://doi.org/10.1371/journal.pone.0090484 3. BENRUD-LARSON LM, HAYTHORNTHWAITE JA, HEINBERG LJ et al.: The impact of pain and symptoms of depression in scleroderma. Pain 2002; 95: 267-75. https:// doi.org/10.1016/s0304-3959(01)00409-2 4. NIETERT PJ, MITCHELL HC, BOLSTER MB, CURRAN MY, TILLEY BC, SILVER RM: Correlates of depression, including overall and gastrointestinal functional status, among patients with systemic sclerosis. J Rheumatol 2005; 32: 51-7. 5. MARCH C, HUSCHER D, PREIS E et al.: Prevalence, risk factors and assessment of depressive symptoms in patients with systemic sclerosis. Arch Rheumatol 2019; 34(3): 253-61. https:// doi.org/10.5606/ArchRheumatol.2019.7111 6. BRAGAZZI NL, WATAD A, GIZUNTERMAN A et al.: The burden of depression in systemic sclerosis patients: a nationwide populationbased study. J Affect Disord 2019; 243: 427- 31. https://doi.org/10.1016/j.jad.2018.09.075 7. MORRISROE K, HUDSON M, BARON M et al.: Determinants of health-related quality of life in a multinational systemic sclerosis inception cohort. Clin Exp Rheumatol 2018; 36 (Suppl. 113): 53-60. 8. DANIELI E, AIRÒ P, BETTONI L, CINQUINI M, ANTONIOLI CM, CAVAZZANA I: Health-related quality of life measured by the Short Form 36 (SF-36) in systemic sclerosis: correlations with indexes of disease activity and severity, disability, and depressive symptoms. Clin Rheumatol 2005; 24(1): 48-54. https://doi.org/10.1007/s10067-004-0970-z 9. GEORGES C, CHASSANY O, TOLEDANO C et al.: Impact of pain in health related quality of life of patients with systemic sclerosis. Rheumatology (Oxford) 2006; 45(10): 1298-302. Clinical and Experimental Rheumatology 2023 1577 COVID-19 and psychological support in SSc patients / G. Lepri et al. https://doi.org/10.1093/rheumatology/kel189 10.OSTOJIC P, ZIVOJINOVIC S, REZA T, DAMJANOV N: Symptoms of depression and anxiety in Serbian patients with systemic sclerosis: impact of disease severity and socioeconomic factors. Mod Rheumatol 2010; 20(4): 353-7. https://doi.org/10.1007/s10165-010-0285-7 11.NGUYEN C, BÉREZNÉ A, BAUBET T et al.: Association of gender with clinical expression, quality of life, disability, and depression and anxiety in patients with systemic sclerosis. PLoS One 2011; 6(3): e17551. https://doi.org/10.1371/journal.pone.0017551 12.FRANTZ C, AVOUAC J, DISTLER O et al.: Impaired quality of life in systemic sclerosis and patient perception of the disease: A large international survey. Semin Arthritis Rheum 2016; 46(1): 115-23. https:// doi.org/10.1016/j.semarthrit.2016.02.005 13.LI L, CUI Y, CHEN S et al.: The impact of systemic sclerosis on health-related quality of life assessed by SF-36: A systematic review and meta-analysis. Int J Rheum Dis 2018; 21(11): 1884-93. https://doi.org/10.1111/1756-185X.13438 14.MALCARNE VL, FOX RS, MILLS SD, GHOLIZADEH S: Psychosocial aspects of systemic sclerosis. Curr Opin Rheumatol 2013; 25(6): 707-13. https:// doi.org/10.1097/01.bor.0000434666.47397.c2 15.WU T, JIA W, SHI H et al.: Prevalence of mental health problems during the COVID-19 pandemic: a systematic review and metaanalysis. J Affect Disord 2021; 281: 91-98. https://doi.org/10.1016/j.jad.2020.11.117 16.WHO Mental health considerations during COVID-19 outbreak. https://www.who.int/ docs/default-source/coronaviruse/mentalhealth-considerations.pdf 17.HENRY RS, KWAKKENBOS L, CARRIER ME et al.: Mental health symptoms in scleroderma during COVID-19: a Scleroderma Patient-centred Intervention Network (SPIN) cohort longitudinal study. Clin Exp Rheumatol 2023; 41(8): 1639-43. https:// doi.org/10.55563/clinexprheumatol/cca0xv 18.THOMBS BD, KWAKKENBOS L, HENRY RS et al.: Changes in mental health symptoms from pre-COVID-19 to COVID-19 among participants with systemic sclerosis from four countries: A Scleroderma Patient-centered Intervention Network (SPIN) Cohort study. J Psychosom Res 2020; 139: 110262. https:// doi.org/10.1016/j.jpsychores.2020.110262 19.THOMBS BD, KWAKKENBOS L, LEVIS B et al.: Effects of a multi-faceted education and support programme on anxiety symptoms among people with systemic sclerosis and anxiety during COVID-19 (SPIN-CHAT): a two-arm parallel, partially nested, randomised, controlled trial. Lancet Rheumatol 2021; 3(6): e427-e437. https:// doi.org/10.1016/S2665-9913(21)00060-6 20.HENRY RS, KWAKKENBOS L, CARRIER ME et al.: Mental health before and during the pandemic in people with systemic sclerosis. Lancet Rheumatol 2022; 4(2): e82-e85. https:// doi.org/10.1016/S2665-9913(21)00363-5 21.WURZ A, DUCHEK D, ELLIS K et al.: A qualitative interview study exploring the psychological health impacts of the SPINCHAT program among people with systemic sclerosis at the onset of COVID-19: perceptions of trial participants and research team members. Disabil Rehabil 2023; 1-13. https:// doi.org/10.1080/09638288.2023.2169775 22.WU Y, KWAKKENBOS L, HENRY RS et al.: Factors associated with fears due to COVID-19: A Scleroderma Patient-centered Intervention Network (SPIN) COVID-19 cohort study. J Psychosom Res 2021; 140: 110314. https:// doi.org/10.1016/j.jpsychores.2020.1103
Exploring the oral microbiome in rheumatic diseases, state of art and future prospective in personalized medicine with an ai approach
Full text linkThe oral microbiome is receiving growing interest from the scientific community, as the mouth is the gateway for numerous potential etiopathogenetic factors in different diseases. In addition, the progression of niches from the mouth to the gut, defined as “oral–gut microbiome axis”, affects several pathologies, as rheumatic diseases. Notably, rheumatic disorders (RDs) are conditions causing chronic, often intermittent pain affecting the joints or connective tissue. In this review, we examine evidence which supports a role for the oral microbiome in the etiology and progression of various RDs, including rheumatoid arthritis (RA), Sjogren’s syndrome (SS), and systemic lupus erythematosus (SLE). In addition, we address the most recent studies endorsing the oral microbiome as promising diagnostic biomarkers for RDs. Lastly, we introduce the concepts of artificial intelligence (AI), in particular, machine learning (ML) and their general application for understanding the link between oral microbiota and rheumatic diseases, speculating the application of a possible AI approach-based that can be applied to personalized medicine in the future
The Role of Adipokines between Genders in the Pathogenesis of Osteoarthritis
Full text linkOsteoarthritis (OA) is a chronic, progressive, degenerative joint disease characterized by joint pain, stiffness, and limited movement. It presents significant intra- and inter-individual variability-in particular, between genders. Recent research has increasingly focused on the role of adipokines-especially leptin, adiponectin, and resistin-in the development of OA. Adipokines, peptide hormones primarily secreted by adipose tissue, are involved in crucial physiological processes related to metabolism and immunity. They can also impact bone and cartilage turnover by interacting with joint cells such as osteoblasts, osteoclasts, chondrocytes, and mesenchymal stem cells, thereby linking inflammation with bone cartilage homeostasis. This review aims to elucidate the structure and functions of various adipokines, their serum and synovial levels, and their association with clinical presentation and radiographic progression in OA patients, with a focus on differences between sexes. A narrative literature review was conducted using three databases specifically analyzing sex differences. OA patients generally show elevated serum and synovial levels of leptin, chemerin, and visfatin, as well as high plasma levels of resistin and visfatin. In contrast, synovial levels of adiponectin and omentin are reduced in OA patients compared to healthy individuals, with an inverse relationship to disease severity, suggesting a potential protective role. Resistin and leptin were positively correlated with pain severity and radiographic progression, while adiponectin's role in OA remains controversial. Regarding sex differences, male OA patients exhibited higher serum levels of leptin, chemerin, and omentin compared to healthy controls, with a positive correlation to the BMI and estrogen levels, potentially explaining the sexual dimorphism observed in this condition. Studies on visfatin and lipocalin did not reveal significant differences in synovial or serum levels between the sexes. The role of resistin remains controversial. Adipokines influence the joint microenvironment and contribute to the progression of osteoarthritis (OA). However, the precise biological mechanisms are not yet fully understood due to the complex interactions between the metabolic, mechanical, and immune systems. Further research is needed to clarify their roles in OA and to identify targeted therapies for managing this degenerative disease
Bosentan fosters microvascular de-remodelling in systemic sclerosis
No full textBosentan, a dual endothelin receptor antagonist, may reduce blood pressure by blocking the vasoconstrictor effect of endothelin-1. In systemic sclerosis (SSc) nailfold videocapillaroscopy (NVC); allows diagnostic and follow-up of microvascular damage. Distinct NVC patterns have been identified for the evaluation of severity of SSc microvascular damage. The objective of this study is to evaluate the modification of the microvasculature under Bosentan therapy in SSc patients with pulmonary arterial hypertension (PAH). Nine patients with PAH related to SSc in New York Heart Association classes III-IV were treated with Bosentan 125 mg twice a day. NVC optical probe videocapillaroscopy equipped with 100x and 200x contact lenses and connected to image analyse software was performed before and after 12 months of Bosentan therapy to evaluate the modification of microvasculature. Nine PAH SSc patients treated with Iloprost were used as controls. Before Bosentan therapy, seven patients showed at NVC severe loss of capillaries with large avascular areas and vascular architectural disorganisation which are typically "late" SSc pattern. After 12 months of Bosentan, NVC pattern changed in seven patients from "late" into "active" SSc pattern. The disappearance of avascular areas and capillary haemorrhages was the most striking result. Two patients had an "active" SSc pattern, not modified by Bosentan treatment. These data show that Bosentan may improve NVC pattern in SSC and the presence of new capillaries suggests that it may favour angiogenesis. Bosentan may improve and stabilise the microvasculature in long-term treatment modulating the structural modifications detected by NVC
- …
