946 research outputs found
Vitamin D Deficiency in a Cohort of Patients with Systemic Scleroderma from the South of Spain Reply
No full textIs There a Place for Chimeric Antigen Receptor–T Cells in the Treatment of Chronic Autoimmune Rheumatic Diseases?
No full textInternational audienceArticle type : Review Is there a place for CART cells in the treatment of chronic autoimmune rheumatic diseases? Cindy Orvain (PhD) (1), Morgane Boulch (PhD) (2), Philippe Bousso (PhD) (2), Yannick Allanore (MD, PhD) (1, 3, 4), Jérôme Avouac (MD, PhD) (1, 3, 4
Scleroderma renal crisis: Case reports and update on critical issues
No full textTo date, scleroderma renal crisis (SRC) remains a life-threatening complication in patients affected by systemic sclerosis (SSc), with high morbidity and mortality. In the last few years, some studies have tried to more precisely identify predictors of SRC and clarify the role of previous drug exposure-in particular, angiotensin-converting enzyme (ACE) inhibitors and corticosteroids-in patients with SSc presenting other well-known risk factors for SRC. Different from the findings of previous reports, more recent findings suggest that the presence of chronic kidney disease, systemic arterial hypertension, and proteinuria might all be predictors of SRC. Moreover, because about 40 to 50% of SRC cases can present signs of microangiopathy, a recent study has proposed SSc thrombotic microangiopathy (SSc-TMA) as a clinically and pathophysiologically different entity from narrowly defined SRC. Even though such clear distinction may not always be applicable/feasible in clinical practice, it highlights that complement pathway dysregulation may play a key pathogenetic role in SRC presenting as TMA. Thus, plasma exchange may be considered in severe refractory cases. Nevertheless, ACE inhibitors and prompt achievement of blood pressure control (to rapidly improve ongoing renal ischemia) remain to date the cornerstone of SRC treatment. Here, we report the cases of three SSc patients with SRC followed at our rheumatology units. While describing these patients' risk factors, clinical presentation, and therapy, we aim to discuss the state of the art in SRC and highlight critical issues
Supplementary_data – Supplemental material for Comparison of the clinical phenotype of systemic sclerosis patients in Iran and France in two university centers
No full textSupplemental material, Supplementary_data for Comparison of the clinical phenotype of systemic sclerosis patients in Iran and France in two university centers by Yannick Allanore, Farhad Gharibdoost, Ahmad Reza Jamshidi, Ali Javinani, Jérôme Avouac, Elnaz Rastkar, Sadid Hooshmandi and Hoda Kavosi in Journal of Scleroderma and Related Disorders</p
Systemic sclerosis: Recent insight in clinical management
No full textSystemic sclerosis (SSc) is a connective tissue disease characterized by diffuse microangiopathy and immune dysregulation which ultimately result in widespread fibrosis of skin and internal organs. Although the 2013 EULAR/ACR criteria have allowed to improve the sensitivity for SSc diagnosis, it has recently come to light that the traditional subclassification into limited and diffuse cutaneous forms does not appear to fully capture the different phenotypes of the scleroderma spectrum. In this regard, a recent large cluster analysis-based study and other ongoing projects are trying to achieve a better stratification of SSc patients, as the disease course remains largely unpredictable to date. Recent preclinical studies and randomized controlled trials have yielded encouraging results with new drugs targeting inflammatory/immunological and fibrotic pathways. One of the main unmet needs in SSc remains the early identification of patients at high mortality risk, for whom aggressiveness of therapies ought to be determined and weighed against disease prognosis. Furthermore, lung and cardiac transplantation may also be taken into account in some carefully selected patients. Though the prognosis of SSc remains poor, an optimized stratification of patients along with the recent and ongoing advances in therapies could greatly impact the natural course of the disease in the near future. Moreover, it is envisioned that there will be an increasing need in the future to further develop combination therapies to better fight against this complex disease. In this review we discussed new insights into organ involvements and therapeutic options
sj-pdf-2-jso-10.1177_23971983231209807 – Supplemental material for Deficiency and altered phenotype of mucosal-associated invariant T cells in systemic sclerosis
No full textSupplemental material, sj-pdf-2-jso-10.1177_23971983231209807 for Deficiency and altered phenotype of mucosal-associated invariant T cells in systemic sclerosis by Manon Lesturgie-Talarek, Virginie Gonzalez, Lucie Beaudoin, Camelia Frantz, Noémie Sénot, Zouriatou Gouda, Camille Rousseau, Jérôme Avouac, Agnès Lehuen and Yannick Allanore in Journal of Scleroderma and Related Disorders</p
BA21570_Khanna_Online_supplementary_data – Supplemental material for Patient perception of disease burden in diffuse cutaneous systemic sclerosis
No full textSupplemental material, BA21570_Khanna_Online_supplementary_data for Patient perception of disease burden in diffuse cutaneous systemic sclerosis by Dinesh Khanna, Yannick Allanore, Christopher P Denton, Marco Matucci-Cerinic, Janet Pope, Barbara Hinzmann, Siobhan Davies, Janethe de Oliveira Pena and Oliver Distler in Journal of Scleroderma and Related Disorders</p
sj-pdf-1-jso-10.1177_23971983231209807 – Supplemental material for Deficiency and altered phenotype of mucosal-associated invariant T cells in systemic sclerosis
No full textSupplemental material, sj-pdf-1-jso-10.1177_23971983231209807 for Deficiency and altered phenotype of mucosal-associated invariant T cells in systemic sclerosis by Manon Lesturgie-Talarek, Virginie Gonzalez, Lucie Beaudoin, Camelia Frantz, Noémie Sénot, Zouriatou Gouda, Camille Rousseau, Jérôme Avouac, Agnès Lehuen and Yannick Allanore in Journal of Scleroderma and Related Disorders</p
sj-pdf-1-jso-10.1177_23971983241235708 – Supplemental material for Attitudes and barriers to pulmonary arterial hypertension screening in systemic sclerosis patients: A survey of UK-based rheumatologists
No full textSupplemental material, sj-pdf-1-jso-10.1177_23971983241235708 for Attitudes and barriers to pulmonary arterial hypertension screening in systemic sclerosis patients: A survey of UK-based rheumatologists by María Paula Álvarez-Hernández, Yannick Allanore, Ivo Andrade, Maya H Buch, Gerry Coghlan, Francesco Del Galdo, Christopher P Denton, Dinesh Khanna, David G Kiely, John D Pauling, Sheila Ramjug and Michael Hughes in Journal of Scleroderma and Related Disorders</p
Use of biologics and other novel therapies for the treatment of systemic sclerosis
No full textINTRODUCTION Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by vasculopathy, inflammation and fibrosis. These three main disease-determining pathways are the target of the currently available treatments used to possibly modify the progression of disease-related manifestations, although this synergy has not been fully applied on SSc joint, skin or lung involvement yet. Areas covered: we describe the current status of SSc treatment/therapy performing a literature search in MEDLINE/Pubmed and Thomson Reuter's Web of Science for articles published until March 2016. Moreover, ongoing registered clinical trials (RCTs) on SSc were searched through clinicaltrials.gov website. Expert commentary: presently, promising drugs are under evaluation to target the different pathogenic pathways of systemic sclerosis: Tocilizumab and Abatacept for skin and lung fibrosis; Riociguat and Selexipag are approved for pulmonary arterial hypertension but promising anti-fibrotic effects are now being studied. Finally, several anti-fibrotic molecules are currently involved in RCTs, such as Nintedanib, IVA-337, Terguride
- …
