Short description
The webinar is focused on the French practical guidelines for the diagnosis and management of relapsing Polychondritis (RP). RP usually begins in middle-aged individuals. This diagnosis is mainly suggested in the presence of chondritis, i.e. inflammatory flares on the cartilage, in particular of the ears, nose or respiratory tract, and more rarely in the presence of other manifestations. The formal diagnosis of relapsing polychondritis cannot be established with certainty before the onset of chondritis, which can sometimes occur several years after the first signs. No laboratory test is specific of relapsing polychondritis, the diagnosis is usually based on clinical evidence and the elimination of differential diagnoses. Relapsing polychondritis is a long-lasting and often unpredictable disease, evolving in the form of relapses interspersed with periods of remission that can be very prolonged. Its management is not codified and depends on the nature of the patient’s symptoms and association or not with myelo-dysplasia/vacuoles, E1 enzyme, X linked, autoinflammatory, somatic (VEXAS). Some minor forms can be treated with non-steroidal anti-inflammatory drugs, or a short course of corticosteroids with possibly a background treatment of colchicine. However, the treatment strategy is often based on the lowest possible dosage of corticosteroids combined with background treatment with conventional immunosuppressants (e.g. methotrexate, azathioprine, mycophenolate mofetil, rarely cyclophosphamide) or targeted therapies. Specific strategies are required if relapsing polychondritis is associated with myelodysplasia/VEXAS. Forms limited to the cartilage of the nose or ears have a good prognosis. Involvement of the cartilage of the respiratory tract, cardiovascular involvement, and association with myelodysplasia/VEXAS (more frequent in men over 50 years of age) are detrimental to the prognosis of the disease.
Speaker
Prof. Laurent Arnaud (MD, PhD) is full Professor of Rheumatology at Strasbourg University & Academic Hospitals (France). He obtained his medical degree and a PhD in immunology from Paris University. His main clinical interests focus on the care of patients with autoimmune diseases such as Systemic Lupus Erythematosus (SLE) and relapsing polychondritis (RP). He is leading a research group focusing on innovative strategies to study the epidemiology of autoimmune diseases, with a particular interest for the use of novel diagnostic and therapeutic digital strategies based on big data and Artificial Intelligence. With his team at the French National Reference Center for Rare Autoimmune Diseases of Strasbourg (CRMR RESO), he has published more than 200 articles in the field of rare diseases. Professor Arnaud is currently the President of the European Lupus Society (SLEuro) and Disease Coordinator for Lupus and relapsing polychondritis of the ERN ReCONNET. He is the curator of a twitter account (@Lupusreference) followed by more than 15.5k people.
More info about RP can be found here:
reconnet.ern-net.eu/disease-rp/
The registration to the ERN ReCONNET Newsletter can be done following this link:
reconnet.ern-net.eu/event-and-media-newsletter/