Article Dans Une Revue La Revue de Médecine Interne Année : 2023

French practical guidelines for the diagnosis and management of relapsing polychondritis

1 Service de rhumatologie [Strasbourg]
2 CHU Strasbourg - Centre Hospitalier Universitaire [Strasbourg]
3 Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
4 UPCité - Université Paris Cité
5 CRESS (U1153 / UMR_A_1125 / UMR_S_1153) - Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité
6 CHU Pitié-Salpêtrière [AP-HP]
7 CIMI - Centre d'Immunologie et des Maladies Infectieuses
8 Service Médecine interne [CHU Toulouse]
9 CIC 1436 - Centre d'investigation clinique de Toulouse
10 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
11 Centre Hospitalier Annecy-Genevois [Saint-Julien-en-Genevois]
12 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
13 IMAGINE - U1163 - Imagine - Institut des maladies génétiques
14 Service d'immuno-hématologie pédiatrique [CHU Necker]
15 Service de Médecine Vasculaire [CHU Toulouse]
16 UdG - Universitat de Girona = University of Girona
17 CHU Saint-Antoine [AP-HP]
18 CHU Tenon [AP-HP]
19 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
20 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
21 U1064 Inserm - CR2TI - Centre de Recherche en Transplantation et Immunologie - Center for Research in Transplantation and Translational Immunology
22 Association AFPCA
23 Hôpital Claude Huriez [Lille]
24 Département Neurologie [CHU Toulouse]
25 Centre de recherche en Immunologie des Infections virales et des maladies auto-immunes
26 CHU Nice - Centre Hospitalier Universitaire de Nice
A. Belot
C. Frances
  • Fonction : Auteur
A. Mekinian
T. Papo
  • Fonction : Auteur
J.-C. Piette
  • Fonction : Auteur
X. Puechal
  • Fonction : Auteur
C. Richez
  • Fonction : Auteur
A. Saraux
  • Fonction : Auteur
P. Seve
  • Fonction : Auteur
F. Tankere
  • Fonction : Auteur
L. Terriou
  • Fonction : Auteur
P. Varin
  • Fonction : Auteur

Résumé

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.

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hal-04890412 , version 1 (09-07-2025)

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L. Arnaud, N. Costedoat-Chalumeau, A. Mathian, L. Sailler, A. Belot, et al.. French practical guidelines for the diagnosis and management of relapsing polychondritis. La Revue de Médecine Interne, 2023, 44 (6), pp.282-294. ⟨10.1016/j.revmed.2023.05.005⟩. ⟨hal-04890412⟩
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