Clinical analysis of 15 patients with relapsing auricular polychondritis

Eur Arch Otorhinolaryngol. 2014 Mar;271(3):473-6. doi: 10.1007/s00405-013-2422-3. Epub 2013 Mar 17.

Abstract

Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology. The disease is characterized by episodic inflammation and destruction of cartilaginous and connective tissue structures, including the ear, eye, nose, larynx, trachea, bronchi, joints, skin, heart valves, and aorta. As the symptoms of RP are diverse and complex, it is easily misdiagnosed. The aim of this paper was to improve the understanding of the clinical features of RP, thereby facilitating its early diagnosis. Fifteen patients with RP were analyzed retrospectively and the relevant literature reviewed. The number of patients presenting with auricular chondritis was 13, while two presented with polyarthritis. Among them, the treatment of 2 RP patients with respiratory tract involvement failed and 1 patient died. Eleven patients with RP (73 %) were initially misdiagnosed. RP involves cartilage and connective tissue. The prognosis for patients with respiratory tract involvement is poor. RP causes episodic and progressive inflammation of cartilage throughout the body and is associated with a variety of clinical manifestations. Early diagnosis of RP depends on a thorough understanding of its clinical features.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Arthritis / pathology
  • Arthritis / physiopathology
  • Child
  • Cohort Studies
  • Diagnostic Errors*
  • Ear Cartilage / pathology
  • Ear Cartilage / physiopathology*
  • Female
  • Hearing Loss, Sensorineural / physiopathology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Labyrinth Diseases / physiopathology
  • Laryngeal Cartilages / pathology
  • Laryngeal Cartilages / physiopathology*
  • Male
  • Middle Aged
  • Polychondritis, Relapsing / diagnosis*
  • Polychondritis, Relapsing / drug therapy
  • Polychondritis, Relapsing / physiopathology
  • Prognosis
  • Retrospective Studies
  • Trachea / pathology
  • Trachea / physiopathology*
  • Young Adult

Substances

  • Immunosuppressive Agents