Proceedings of the 2019 Canadian Inflammatory Myopathy Study Symposium: Clinical Trial Readiness in Myositis.

The Canadian Inflammatory Myopathy Study (CIMS) is a multicenter prospective cohort recruiting in 8 centers across Canada. One of the aims of CIMS is to conduct and participate in clinical trials in autoimmune inflammatory myopathies (AIM). Conducting clinical trials in rare diseases such as AIM presents challenges. During this symposium, experts in the field presented different solutions to successfully conduct clinical trials in AIM, including the importance of collaboration and careful trial design, as well as training and mentoring of young investigators

Scleromyositis: A distinct novel entity within the systemic sclerosis and autoimmune myositis spectrum. Implications for care and pathogenesis

Systemic sclerosis and autoimmune myositis are both associated with decreased quality of life and increased mortality. Their prognosis and management largely depend on the disease subgroups. Indeed, systemic sclerosis is a heterogeneous disease, the two predominant forms of the disease being limited and diffuse scleroderma. Autoimmune myositis is also a heterogeneous group of myopathies that classically encompass necrotizing myopathy, antisynthetase syndrome, dermatomyositis and inclusion body myositis. Recent data revealed that an additional disease subset, denominated “scleromyositis”, should be recognized within both the systemic sclerosis and the autoimmune myositis spectrum. We performed an in-depth review of the literature with the aim of better delineating scleromyositis. Our review highlights that this concept is supported by recent clinical, serological and histopathological findings that have important implications for patient management and understanding of the disease pathophysiology. As compared with other subsets of systemic sclerosis and autoimmune myositis, scleromyositis patients can present with a characteristic pattern of muscle involvement (i.e. distribution of muscle weakness) along with multisystemic involvement, and some of these extra-muscular complications are associated with poor prognosis. Several autoantibodies have been specifically associated with scleromyositis, but they are not currently integrated in diagnostic and classification criteria for systemic sclerosis and autoimmune myositis. Finally, striking vasculopathic lesions at muscle biopsy have been shown to be hallmarks of scleromyositis, providing a strong anatomopathological substratum for the concept of scleromyositis. These findings bring new insights into the pathogenesis of scleromyositis and help to diagnose this condition, in patients with subtle SSc features and/or no autoantibodies (i.e. “seronegative” scleromyositis). No guidelines are available for the management of these patients, but recent data are showing the way towards a new therapeutic approach dedicated to these patients

Response to: Comment on “Systematic retrospective study on 64 patients anti-Mi2 dermatomyositis: A classic skin rash with a necrotizing myositis and high risk of malignancy”

International audienc

Nano-positioning and tubulin conformation contribute to axonal transport regulation of mitochondria along microtubules

Correct spatiotemporal distribution of organelles and vesicles is crucial for healthy cell functioning and is regulated by intracellular transport mechanisms. Controlled transport of bulky mitochondria is especially important in polarized cells such as neurons that rely on these organelles to locally produce energy and buffer calcium. Mitochondrial transport requires and depends on microtubules that fill much of the available axonal space. How mitochondrial transport is affected by their position within the microtubule bundles is not known. Here, we found that anterograde transport, driven by kinesin motors, is susceptible to the molecular conformation of tubulin in neurons both in vitro and in vivo. Anterograde velocities negatively correlate with the density of elongated tubulin dimers like guanosine triphosphate (GTP)-tubulin. The impact of the tubulin conformation depends primarily on where a mitochondrion is positioned, either within or at the rim of microtubule bundle. Increasing elongated tubulin levels lowers the number of motile anterograde mitochondria within the microtubule bundle and increases anterograde transport speed at the microtubule bundle rim. We demonstrate that the increased kinesin velocity and density on microtubules consisting of elongated dimers add to the increased mitochondrial dynamics. Our work indicates that the molecular conformation of tubulin contributes to the regulation of mitochondrial motility and as such to the local distribution of mitochondria along axons

e-courses for masters: online fundamental semester for master on climate – related sciencedisciplines

International audienceThe Climate Graduate School of the Institut Pierre-Simon Laplace (CGS-IPSL) iscurrently developing a series of seven courses online for Master students. On these sevencourses of 3 ECTS each, three have a strong focus on the ocean: Dynamics of the Ocean andAtmosphere; Contemporary Biogeochemical Cycles; Study of Paleo-climates. Each course is ledby a pair of university teachers specialists in their field accompanied by a pedagogical andgraphic team in order to design all resources specifically adapted to graduate students andonline education. Indeed, our first goal is to open these courses as self-paced learning underthe IPSL Learning Management System (Moodle) to students who will be joining CGS-IPSLmasters without having all the prerequisites so that they can update their academicbackground. These courses could also be open to second year CGS IPSL Master students whoaimed at acquiring credits on a secondary theme not necessarily developed in their master (e.g.on biogeochemistry for students registered in an ocean – climate master). Finally all the e-resources developed will be made available at least to the educational community of the CGS-IPSL that include several Universities in and around Paris (Sorbonne Universite, Paris Saclay,Universite Versailles Saint Quentin, Université Paris Est Créteil...) in order to reuse theseresources for higher education either on-line or face-to-face courses. The courses will beavailable in both French and English so that international students can have access to these e-resources

e-courses for masters: online fundamental semester for master on climate – related sciencedisciplines

International audienceThe Climate Graduate School of the Institut Pierre-Simon Laplace (CGS-IPSL) iscurrently developing a series of seven courses online for Master students. On these sevencourses of 3 ECTS each, three have a strong focus on the ocean: Dynamics of the Ocean andAtmosphere; Contemporary Biogeochemical Cycles; Study of Paleo-climates. Each course is ledby a pair of university teachers specialists in their field accompanied by a pedagogical andgraphic team in order to design all resources specifically adapted to graduate students andonline education. Indeed, our first goal is to open these courses as self-paced learning underthe IPSL Learning Management System (Moodle) to students who will be joining CGS-IPSLmasters without having all the prerequisites so that they can update their academicbackground. These courses could also be open to second year CGS IPSL Master students whoaimed at acquiring credits on a secondary theme not necessarily developed in their master (e.g.on biogeochemistry for students registered in an ocean – climate master). Finally all the e-resources developed will be made available at least to the educational community of the CGS-IPSL that include several Universities in and around Paris (Sorbonne Universite, Paris Saclay,Universite Versailles Saint Quentin, Université Paris Est Créteil...) in order to reuse theseresources for higher education either on-line or face-to-face courses. The courses will beavailable in both French and English so that international students can have access to these e-resources

e-courses for masters: online fundamental semester for master on climate – related sciencedisciplines

International audienceThe Climate Graduate School of the Institut Pierre-Simon Laplace (CGS-IPSL) iscurrently developing a series of seven courses online for Master students. On these sevencourses of 3 ECTS each, three have a strong focus on the ocean: Dynamics of the Ocean andAtmosphere; Contemporary Biogeochemical Cycles; Study of Paleo-climates. Each course is ledby a pair of university teachers specialists in their field accompanied by a pedagogical andgraphic team in order to design all resources specifically adapted to graduate students andonline education. Indeed, our first goal is to open these courses as self-paced learning underthe IPSL Learning Management System (Moodle) to students who will be joining CGS-IPSLmasters without having all the prerequisites so that they can update their academicbackground. These courses could also be open to second year CGS IPSL Master students whoaimed at acquiring credits on a secondary theme not necessarily developed in their master (e.g.on biogeochemistry for students registered in an ocean – climate master). Finally all the e-resources developed will be made available at least to the educational community of the CGS-IPSL that include several Universities in and around Paris (Sorbonne Universite, Paris Saclay,Universite Versailles Saint Quentin, Université Paris Est Créteil...) in order to reuse theseresources for higher education either on-line or face-to-face courses. The courses will beavailable in both French and English so that international students can have access to these e-resources