French protocol for diagnosis and management of Cogan's syndrome.

Cogan's syndrome Diagnosis Diagnostic Management Prise en charge Syndrome de Cogan Traitement Treatment

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:
24 Oct 2024
Historique:
received: 19 06 2024
accepted: 26 09 2024
medline: 26 10 2024
pubmed: 26 10 2024
entrez: 25 10 2024
Statut: aheadofprint

Résumé

Cogan's syndrome is a condition of unknown origin, classified as a systemic vasculitis. It is characterised by a predilection for the cornea and the inner ear. It mainly affects Caucasian individuals with a sex-ratio close to one. Ophthalmological and cochleo-vestibular involvement are the most common manifestations of the disease. The most frequent ophthalmological type of involvement is non-syphilitic interstitial keratitis. Cochleo-vestibular manifestations are similar to those of Meniere's syndrome. The disease progresses in ocular and ear-nose-throat (ENT) flares, which may occur simultaneously or in isolation. Association with other autoimmune diseases, particularly other forms of vasculitis such as polyarteritis nodosa or Takayasu's arteritis, is possible. Ocular involvement, as well as cochleo-vestibular involvement, can be inaugural and initially isolated. Onset is often abrupt. The characteristic involvement is "non-syphilitic" interstitial keratitis. It is usually bilateral from the outset or becomes so during the course of the disease. It presents as a red, painful eye, possibly associated with decreased visual acuity. Cochleo-vestibular involvement is usually bilateral from the outset. It is characterised by the sudden onset of continuous rotational vertigo associated with tinnitus, rapidly progressive sensorineural deafness. Approximately 30-70% of patients present with systemic manifestations. Deterioration in general status with fever may be present. Laboratory evidence of inflammatory syndrome is associated in 75% of cases. Cogan's syndrome is a presumed autoimmune type of vasculitis, although no specific autoantibodies have been identified. Ocular involvement is usually associated with a good prognosis, with total visual acuity recovery in the majority of cases. In contrast, cochleo-vestibular involvement can be severe and irreversible. Therapeutic management of Cogan's syndrome, given its rarity, lacks consensus since no prospective randomised studies have been conducted to date. Corticosteroid therapy is the first-line treatment. Combination with anti-TNF therapy should be promptly discussed.

Identifiants

pubmed: 39455380
pii: S0248-8663(24)00778-1
doi: 10.1016/j.revmed.2024.09.007
pii:
doi:

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

Copyright © 2024 The Authors. Published by Elsevier Masson SAS.. All rights reserved.

Auteurs

Laurent Arnaud (L)

INSERM UMRS-1109, Department of Rheumatology, National Reference Center for Autoimmune diseases (RESO), Strasbourg-Hautepierre University Hospital, Strasbourg, France.

Alexandra Audemard-Verger (A)

Department of Internal Medicine, Tours University Hospital, Tours, France.

Alexandre Belot (A)

Department of Paediatric Nephrology, Rheumatology, Dermatology, Reference Centre for Rheumatic, AutoImmune and Systemic Diseases in Children (RAISE), Femme-Mère-Enfant Hospital, Hospices Civils of Lyon, Bron, France.

Boris Bienvenu (B)

Department of Internal Medicine, Saint-Joseph Hospital, Marseille, France.

Carole Burillon (C)

Department of Ophthalmology, Édouard-Herriot University Hospital, Hospices Civils of Lyon, Lyon, France.

François Chasset (F)

Department of Dermatology and Allergology, Tenon Hospital, Faculty of Medicine, Sorbonne University, Paris, France.

Florence Chaudot (F)

Service de médecine interne, hôpital Saint-Antoine, AP-HP, Sorbonne université, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.

Raphael Darbon (R)

French Vasculitis Association, Paris, France.

Anastasia Delmotte (A)

Service de médecine interne, hôpital Saint-Antoine, AP-HP, Sorbonne université, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.

Mikael Ebbo (M)

Department of Internal Medicine, Marseille University Hospital, Marseille, France.

Olivier Espitia (O)

INSERM UMR1087/CNRS UMR 6291, Team III Vascular & Pulmonary diseases, Department of Internal and Vascular Medicine, institut du thorax, CHU of Nantes, Nantes université, F-44000 Nantes, France.

Anne-Laure Fauchais (AL)

Department of Internal Medicine, Dupuytren University Hospital, Limoges, France.

Alexis F Guedon (AF)

Department of Internal Medicine, Saint-Antoine Hospital, Paris, France.

Eric Hachulla (E)

INSERM, Department of Internal Medicine and Clinical Immunology, Reference Centre for Auto-immune Systemic Rare Diseases of North and North-West of France (CeRAINO), Lille University Hospital, Lille University, Lille, France.

Jérôme Hadjadj (J)

Service de médecine interne, hôpital Saint-Antoine, AP-HP, Sorbonne université, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.

Charlotte Hautefort (C)

Department of Ear, Nose, Throat, Lariboisière Hospital, université Paris Cité, Paris, France.

Vincent Jachiet (V)

Service de médecine interne, hôpital Saint-Antoine, AP-HP, Sorbonne université, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.

Elisabeth Mamelle (E)

Department of Ear, Nose, Throat, Rothschild Hospital, Paris, France.

Mickael Martin (M)

Department of Internal Medicine, Poitiers University Hospital, Poitiers, France.

Marc Muraine (M)

Department of Ophthalmology, Rouen University Hospital, Rouen, France.

Thomas Papo (T)

Department of Internal Medicine, Bichat Hospital, Paris, France.

Jacques Pouchot (J)

Department of Internal Medicine, Georges-Pompidou European Hospital, Paris, France.

Grégory Pugnet (G)

Department of Internal Medicine, Toulouse University Hospital, Toulouse, France.

Pascal Seve (P)

Department of Internal Medicine, La Croix-Rousse Hospital, Hospices Civils of Lyon, Lyon, France.

Thierry Zenone (T)

Department of Internal Medicine, Valence Hospital Centre, Valence, France.

Arsène Mekinian (A)

Service de médecine interne, hôpital Saint-Antoine, AP-HP, Sorbonne université, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France. Electronic address: arsene.mekinian@aphp.fr.

Classifications MeSH