title: Relapsing polychondritis: A 2016 update on clinical features, diagnostic tools, treatment and biological drug use.
abstract: Relapsing polychondritis (RP) is a very rare autoimmune disease characterised by a relapsing inflammation of the cartilaginous tissues (joints, ears, nose, intervertebral discs, larynx, trachea and cartilaginous bronchi), which may progress to long-lasting atrophy and/or deformity of the cartilages. Non-cartilaginous tissues may also be affected, such as the eyes, heart, aorta, inner ear and skin. RP has a long and unpredictable course. Because no randomised therapeutic trials are available, the treatment of RP remains mainly empirical. Minor forms of the disease can be treated with non-steroidal anti-inflammatory drugs, whereas more severe forms are treated with systemic corticosteroids. Life-threatening diseases and corticosteroid-dependent or resistant diseases are an indication for immunosuppressant therapy such as methotrexate, azathioprine, mycophenolate mofetil and cyclophosphamide. Biologics could be given as second-line treatment in patients with an active disease despite the use of steroids and immunosuppressive drugs. Although the biologics represent new potential treatment for RP, very scarce information is available to draw any firm conclusion on their use in RP.
authors.affiliation: Assistance Publique-Hôpitaux de Paris (AP-HP), Groupement Hospitalier Pitié-Salpêtrière (GHPS), French National Reference Center for Rare Systemic Auto-immune Diseases, Service de Médecine interne 2, Paris, France; Inserm, U1135, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Paris, France; Université Pierre et Marie Curie, Univ Paris 06, Paris, France. Electronic address: alexis.mathian@psl.aphp.fr.
authors.forename: Alexis
authors.initials: A
authors.lastname: Mathian
authors.affiliation: AP-HP, GHPS, Département d'Immunologie, Paris, France; Inserm, U1135, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Paris, France; Université Pierre et Marie Curie, Univ Paris 06, Paris, France.
authors.forename: Makoto
authors.initials: M
authors.lastname: Miyara
authors.affiliation: Assistance Publique-Hôpitaux de Paris (AP-HP), Groupement Hospitalier Pitié-Salpêtrière (GHPS), French National Reference Center for Rare Systemic Auto-immune Diseases, Service de Médecine interne 2, Paris, France; Inserm, U1135, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Paris, France; Université Pierre et Marie Curie, Univ Paris 06, Paris, France.
authors.forename: Fleur
authors.initials: F
authors.lastname: Cohen-Aubart
authors.affiliation: Assistance Publique-Hôpitaux de Paris (AP-HP), Groupement Hospitalier Pitié-Salpêtrière (GHPS), French National Reference Center for Rare Systemic Auto-immune Diseases, Service de Médecine interne 2, Paris, France; Inserm, U1135, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Paris, France; Université Pierre et Marie Curie, Univ Paris 06, Paris, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.affiliation: Assistance Publique-Hôpitaux de Paris (AP-HP), Groupement Hospitalier Pitié-Salpêtrière (GHPS), French National Reference Center for Rare Systemic Auto-immune Diseases, Service de Médecine interne 2, Paris, France.
authors.forename: Miguel
authors.initials: M
authors.lastname: Hie
authors.affiliation: Assistance Publique-Hôpitaux de Paris (AP-HP), Groupement Hospitalier Pitié-Salpêtrière (GHPS), French National Reference Center for Rare Systemic Auto-immune Diseases, Service de Médecine interne 2, Paris, France; Université Pierre et Marie Curie, Univ Paris 06, Paris, France.
authors.forename: Micheline
authors.initials: M
authors.lastname: Pha
authors.affiliation: Université Pierre et Marie Curie, Univ Paris 06, Paris, France; AP-HP, GHPS, Service de Radiologie, Paris, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Grenier
authors.affiliation: Assistance Publique-Hôpitaux de Paris (AP-HP), Groupement Hospitalier Pitié-Salpêtrière (GHPS), French National Reference Center for Rare Systemic Auto-immune Diseases, Service de Médecine interne 2, Paris, France; Inserm, U1135, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Paris, France; Université Pierre et Marie Curie, Univ Paris 06, Paris, France.
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D001379:Azathioprine / Q000627:therapeutic use; D001688:Biological Products / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D006801:Humans; D008727:Methotrexate / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000627:therapeutic use; D011081:Polychondritis, Relapsing / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D001688:Biological Products; D003520:Cyclophosphamide; D009173:Mycophenolic Acid; D001379:Azathioprine; D008727:Methotrexate
keywords: Biologics; Chondritis; Myelodysplastic syndrome; Relapsing polychondritis