| Title: |
French practical guidelines for the diagnosis and management of relapsing polychondritis |
| Authors: |
Arnaud, L.; Costedoat-Chalumeau, N.; Mathian, A.; Sailler, L.; Belot, A.; Dion, J.; Morel, N.; Moulis, G.; Bader-Meunier, B.; Bodaghi, B.; Bura Riviere, A.; Casadevall, M.; Fain, O.; Frances, C.; Hachulla, E.; Hamidou, M.; Karakoglou, C.; Lambert, M.; Lerebours, F.; Leroux, G.; Mariette, X.; Marquette, C.H.; Martin, T.; Mekinian, A.; Papo, T.; Piette, J.-C.; Puechal, X.; Richez, C.; Saraux, A.; Seve, P.; Tankere, F.; Terriou, L.; Varin, P. |
| Contributors: |
Service de rhumatologie Strasbourg; Centre Hospitalier Universitaire Strasbourg (CHU Strasbourg); Hôpitaux Universitaires de Strasbourg (HUS)-Hôpitaux Universitaires de Strasbourg (HUS)-Hôpital de Hautepierre Strasbourg; Hôpitaux Universitaires de Strasbourg (HUS); Service de médecine interne et centre de référence des maladies rares CHU Cochin; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin AP-HP; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP); Université Paris Cité (UPCité); Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)); Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM); CHU Pitié-Salpêtrière AP-HP; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU); Centre d'Immunologie et des Maladies Infectieuses (CIMI); Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS); Service Médecine interne CHU Toulouse; Pôle Inflammation, infection, immunologie et loco-moteur CHU Toulouse (Pôle I3LM Toulouse); Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse); Centre d'investigation clinique de Toulouse (CIC 1436); Université Toulouse III - Paul Sabatier (UT3); Communauté d'universités et établissements de Toulouse (Comue de Toulouse)-Communauté d'universités et établissements de Toulouse (Comue de Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Pôle Santé publique et médecine publique CHU Toulouse; Centre Hospitalier Lyon Sud CHU - HCL (CHLS); Hospices Civils de Lyon (HCL); Centre Hospitalier Annecy-Genevois Saint-Julien-en-Genevois; Centre Hospitalier Universitaire de Toulouse (CHU Toulouse); Imagine - Institut des maladies génétiques (IMAGINE - U1163); Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM); Service d'immuno-hématologie pédiatrique CHU Necker; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Necker - Enfants Malades AP-HP; Service de Médecine Vasculaire CHU Toulouse; Pôle Cardiovasculaire et Métabolique CHU Toulouse; Universitat de Girona = University of Girona (UdG); CHU Saint-Antoine AP-HP; CHU Tenon AP-HP; Centre Hospitalier Régional Universitaire CHU Lille (CHRU Lille); Centre Hospitalier Universitaire de Nantes = Nantes University Hospital (CHU Nantes); Centre de Recherche en Transplantation et Immunologie - Center for Research in Transplantation and Translational Immunology (U1064 Inserm - CR2TI); Institut National de la Santé et de la Recherche Médicale (INSERM)-Nantes Université - UFR de Médecine et des Techniques Médicales (Nantes Univ - UFR MEDECINE); Nantes Université - pôle Santé; Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Santé; Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ); Association AFPCA; Hôpital Claude Huriez Lille; Département Neurologie CHU Toulouse; Pôle Neurosciences CHU Toulouse; Centre de recherche en Immunologie des Infections virales et des maladies auto-immunes; Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Bicêtre AP-HP, Le Kremlin-Bicêtre; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Saclay; Centre Hospitalier Universitaire de Nice (CHU Nice) |
| Source: |
ISSN: 0248-8663. |
| Publisher Information: |
CCSD; Elsevier |
| Publication Year: |
2023 |
| Collection: |
Université Toulouse III - Paul Sabatier: HAL-UPS |
| Subject Terms: |
Myelodysplasia; Gestion; La polychondrite récidivante; Management; VEXAS; Relapsing polychondritis; Myélodysplasie; MESH: Adrenal Cortex Hormones / therapeutic use; MESH: Bone Diseases; MESH: Polychondritis; Relapsing* / therapy; MESH: Skin Diseases; Genetic; MESH: Humans; MESH: Immunosuppressive Agents / therapeutic use; MESH: Inflammation / complications; MESH: Male; MESH: Middle Aged; MESH: Myelodysplastic Syndromes* / complications; Relapsing* / diagnosis; Relapsing* / epidemiology; [SDV.IMM]Life Sciences [q-bio]/Immunology |
| Description: |
International audience ; Relapsing polychondritis is a rare systemic disease. It 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 myelodysplasia/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 50years of age) are detrimental to the prognosis of the disease. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| Relation: |
info:eu-repo/semantics/altIdentifier/pmid/37236870; PUBMED: 37236870 |
| DOI: |
10.1016/j.revmed.2023.05.005 |
| Availability: |
https://hal.science/hal-04890412; https://hal.science/hal-04890412v1/document; https://hal.science/hal-04890412v1/file/S024886632300591X.pdf; https://doi.org/10.1016/j.revmed.2023.05.005 |
| Rights: |
https://creativecommons.org/licenses/by-nc/4.0/ ; info:eu-repo/semantics/OpenAccess |
| Accession Number: |
edsbas.A72850B4 |
| Database: |
BASE |