A Clinical Study of 9 Cases of Relapsing Polychondritis

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  • Nagano Hiromi
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Miyamoto Yumi
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Jimura Tomohiro
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Iuchi Hiroyuki
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Umakoshi Mizuo
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Makise Takao
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Kawabata Masaki
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Miyashita Keiichi
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Ohori Junichirou
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Miyanohara Ikuyo
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences
  • Kurono Yuichi
    Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences

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Other Title
  • 再発性多発軟骨炎9症例の臨床検討
  • サイハツセイ タハツ ナンコツエン 9 ショウレイ ノ リンショウ ケントウ

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Abstract

<p>　Relapsing polychondritis is a rare and potentially fatal autoimmune disease of unknown etiology, characterized by inflammation and destruction of different cartilaginous structures, including the ear, nose, larynx, trachea, bronchi, peripheral joints, eyes, heart and skin, with a high risk of misdiagnosis.</p><p></p><p>　Data of nine patients with this condition were analyzed. The average age at diagnosis of the nine patients (four male and five female) was 56.4 years (33-84 years). The interval from onset to diagnosis varied from three months to two years. Auricular chondritis was detected in six patients, polyarthritis in three patients, nasal chondritis in four patients, inflammation of the eyes in two patients, laryngotracheal chondritis in six patients, and labyrinthine dysfunction in two patients.</p><p></p><p>　Underlying autoimmune diseases included Behçet's disease, polymyalgia rheumatica, and Vogt-Koyanagi-Harada disease. No biopsy findings are pathognomonic for the diagnosis of RP. Histopathological examination of an involved cartilage can be a valuable tool when accurate diagnosis proves difficult. However, steroid treatment, as in patients with autoimmune diseases can affect the results of histopathological examination and modify the clinical manifestations.</p><p></p><p>　Until date, therapy for RP is still empiric, due to the lack of standardized guidelines for the treatment, and depends on the basis of the disease activity and severity of organ involvement. Mild forms, such as auricular chondritis alone, are treated with NSAIDs and low-dose corticosteroids. Laryngotracheal complications require high-dose corticosteroids and immunosuppressants. As for convalescence, eight cases survived, and one died of other diseases.</p>

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