Relapsing polychondritis: A 2016 update on clinical features, diagnostic tools, treatment and biological drug use

Best Pract Res Clin Rheumatol. 2016 Apr;30(2):316-333. doi: 10.1016/j.berh.2016.08.001. Epub 2016 Oct 1.

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.

Keywords: Biologics; Chondritis; Myelodysplastic syndrome; Relapsing polychondritis.

Publication types

  • Review

MeSH terms

  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / pathology
  • Azathioprine / therapeutic use
  • Biological Products / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Humans
  • Methotrexate / therapeutic use
  • Mycophenolic Acid / therapeutic use
  • Polychondritis, Relapsing / diagnosis*
  • Polychondritis, Relapsing / drug therapy*
  • Polychondritis, Relapsing / pathology*

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Biological Products
  • Cyclophosphamide
  • Mycophenolic Acid
  • Azathioprine
  • Methotrexate