French practical guidelines for the diagnosis and management of relapsing polychondritis.


Journal

La Revue de medecine interne
ISSN: 1768-3122
Titre abrégé: Rev Med Interne
Pays: France
ID NLM: 8101383

Informations de publication

Date de publication:
Jun 2023
Historique:
received: 02 05 2023
accepted: 12 05 2023
medline: 5 6 2023
pubmed: 27 5 2023
entrez: 26 5 2023
Statut: ppublish

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.

Identifiants

pubmed: 37236870
pii: S0248-8663(23)00591-X
doi: 10.1016/j.revmed.2023.05.005
pii:
doi:

Substances chimiques

Immunosuppressive Agents 0
Adrenal Cortex Hormones 0

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

282-294

Investigateurs

B Bader-Meunier (B)
B Bodaghi (B)
A Bura Riviere (A)
M Casadevall (M)
O Fain (O)
C Frances (C)
E Hachulla (E)
M Hamidou (M)
C Karakoglou (C)
M Lambert (M)
F Lerebours (F)
G Leroux (G)
X Mariette (X)
C H Marquette (CH)
T Martin (T)
A Mekinian (A)
T Papo (T)
J-C Piette (JC)
X Puechal (X)
C Richez (C)
A Saraux (A)
P Seve (P)
F Tankere (F)
L Terriou (L)
P Varin (P)

Informations de copyright

Copyright © 2023 The Authors. Published by Elsevier Masson SAS.. All rights reserved.

Auteurs

L Arnaud (L)

Service de Rhumatologie, Hôpitaux Universitaires de Strasbourg, Centre National de Référence des Maladies Auto-Immunes Est Sud-Ouest (RESO), Strasbourg, France. Electronic address: Laurent.arnaud@chru-strasbourg.fr.

N Costedoat-Chalumeau (N)

Department of Internal Medicine, Île-de-France Rare Autoimmune and Systemic Diseases Reference Centre, hôpital Cochin, Paris, France; University of Paris Cité, Paris, France; Inserm U1153, Centre de recherche en épidémiologie et statistiques Sorbonne Paris Cité (CRESS), Paris, France.

A Mathian (A)

Service de médecine interne 2, Institut E3M, Inserm UMRS, Centre d'immunologie et des maladies infectieuses (CIMI-Paris)groupement hospitalier Pitié-Salpêtrière, Centre de référence du lupus, syndrome des anticorps antiphospholipides et autres maladies auto-immunes rares, Assistance publique-Hôpitaux de Paris, Paris, France.

L Sailler (L)

Internal Medicine Department URM Pavilion C.I.C. 1436 - module plurithématique adulte, hôpital Purpan, CHU de Toulouse, Toulouse, France.

A Belot (A)

Department of Paediatric Nephrology-Rheumatology-Dermatology, Mère-enfant Hospital, hospices civils de Lyon, Lyon, France.

J Dion (J)

Department of Internal Medicine, Île-de-France Rare Autoimmune and Systemic Diseases Reference Centre, hôpital Cochin, Paris, France; University of Paris Cité, Paris, France; Inserm U1153, Centre de recherche en épidémiologie et statistiques Sorbonne Paris Cité (CRESS), Paris, France.

N Morel (N)

Department of Internal Medicine, Île-de-France Rare Autoimmune and Systemic Diseases Reference Centre, hôpital Cochin, Paris, France; University of Paris Cité, Paris, France; Inserm U1153, Centre de recherche en épidémiologie et statistiques Sorbonne Paris Cité (CRESS), Paris, France.

G Moulis (G)

Internal Medicine Department URM Pavilion C.I.C. 1436 - module plurithématique adulte, hôpital Purpan, CHU de Toulouse, Toulouse, France.

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