[Relapsing polychondritis revealed by ENT symptoms: clinical characteristics in three patients].
Relapsing polychondritis (RP) is a recurrent, chronic and rare disease of unknown etiology, considered as a systemic vasculitis. RP is characterized by inflammation of cartilaginous structures of the ears, nose, respiratory tract and joints. RP is likely initiated by ENT symptoms. Etiology is unknown but the association with HLA-DR4 and the occurrence of antibodies to type-II collagen suggest that an immunologic mechanism is involved in its pathogenesis. Diagnosis is difficult requiring identification of elastic cartilaginous injuries. Delay before diagnosis is usually important after the first attack. Neither serum investigation nor histological confirmation are necessary to establish the RP diagnosis, and ENT symptoms are generally sufficient to achieve the diagnosis. Prognosis is linked to laryngeal, tracheal and cardiovascular involvements. An association with myelodysplasia is acknowledged. Based on these three cases and data in the literature, we review classical diagnostic criteria (McAdam), prognosis and therapeutic outcome.
Malard
O
O
Service d'O.R.L. et de chirurgie cervico-faciale, CHU Hôtel-Dieu, 1, place A. Ricordeau, BP 1005, 44093 Nantes Cedex 01, France.
Hamidou
M
M
Toquet
C
C
Bailleuil
S
S
Bordure
Ph
P
Beauvillain De Montreuil
C
C
D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001299:Audiometry; D002357:Cartilage Diseases / Q000209:etiology; D004425:Ear Cartilage; D004427:Ear Diseases / Q000209:etiology; D005260:Female; D003639:Hearing Loss, Sudden / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007817:Laryngeal Cartilages; D007818:Laryngeal Diseases / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010038:Otorhinolaryngologic Diseases / Q000209:etiology*; D010951:Plasma Exchange; D011081:Polychondritis, Relapsing / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy; D011379:Prognosis
D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents