ステロイド反応性を契機に診断に至った再発性多発軟骨炎例

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  • 橘 茜
    東京都立病院機構東京都立墨東病院耳鼻咽喉科
  • 横西 久幸
    東京都立病院機構東京都立墨東病院耳鼻咽喉科
  • 馬場 美雪
    東京都立病院機構東京都立墨東病院耳鼻咽喉科

書誌事項

タイトル別名
  • A Case of Relapsing Polychondritis Diagnosed by Its Steroid Responsiveness
  • ステロイド ハンノウセイ オ ケイキ ニ シンダン ニ イタッタ サイハツセイ タハツ ナンコツエンレイ

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説明

<p>Relapsing polychondritis is a rare, refractory disease characterized by chronic inflammation of the cartilage; involvement of the airways is often a poor prognostic factor.</p><p>In this report, we describe a case of relapsing polychondritis that was diagnosed by the presence of intractable subglottic stenosis. The patient, a 67-year-old woman, presented to our department with dyspnea. She was diagnosed as having subglottic laryngitis based on the presence of subglottic stenosis and laryngeal swelling, and was admitted to the hospital. The subglottic stenosis improved slightly after treatment with intravenous antibiotics and high doses of a steroid, and the patient was discharged from the hospital. However, after discharge, the subglottic stenosis worsened. The inflammatory response markers remained mildly elevated. The patient was initiated on treatment with an oral steroid on an outpatient basis, and this treatment led to a marked improvement of her symptoms. Thus, the subglottic stenosis was steroid-responsive. The patient also had a tender saddle nose. Based on the above, and the presence of inflammatory findings in the nasal cartilage and laryngeal tracheal cartilage, we made the diagnosis of recurrent polychondritis according to the diagnostic criteria of Michet et al. Subsequent steroid treatment resulted in improvement of both the nasal cartilage tenderness and tinnitus, fulfilling the diagnostic criteria proposed by Damiani et al.</p><p>Because of the diverse clinical manifestations of recurrent polychondritis, a definitive diagnosis often takes time. Another reason is the lack of a highly specific laboratory test. Patients with airway involvement often require early intervention, but in this case, we were able to make the diagnosis based on the clinical findings and steroid responsiveness of the lesions, in the absence of a pathological diagnosis. We hope that this case report contributes to increasing awareness about this disease and timely therapeutic intervention in the future.</p>

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