ぶどう膜炎を契機に診断した再発性多発軟骨炎の1例

A case of relapsing polychondritis with concomitant with uveitis

要約　背景：再発性多発軟骨炎は，全身の軟骨組織に再発性の炎症をきたす難治性疾患である。ぶどう膜炎を契機に診断した再発性多発性軟骨炎の1例を報告する。

症例：患者は43歳，女性。3か月前からの頸部リンパ節腫脹，両耳の腫脹・疼痛，咽頭痛を認め，発熱を繰り返していた。2日前からの霧視があり近医眼科を受診し，両眼のぶどう膜炎を認め高知大学医学部附属病院眼科に紹介となった。初診時，眼所見として両眼に眼球結膜充血，豚脂様の角膜後面沈着物，前房内炎症，虹彩後癒着，黄斑浮腫を認め，全身所見としては両耳介の発赤・腫脹のほか，血液検査で炎症反応の上昇を認めた。耳介の超音波検査で耳介軟骨周囲の軟部組織の腫脹と血流がみられ，耳介生検の病理検査で軟骨周囲のリンパ球・好中球の浸潤および血管新生所見が認められたため，再発性多発軟骨炎と診断した。ステロイド点眼薬に加えステロイド結膜下注射を施行した。全身症状に対してはステロイド内服で加療し，ぶどう膜炎および耳介の炎症は改善した。その後ステロイドを漸減しているが，眼および耳介の炎症の再発はない。

結論：全身の炎症所見を伴う原因不明の再発性の眼炎症疾患は，鑑別疾患として再発性多発軟骨炎を考えることが重要である。

Abstract　Purpose：Relapsing polychondritis is a chronic systemic inflammatory disorder of the cartilaginous tissue. We report a case of relapsing polychondritis concomitant with uveitis.

Case：A 43-year-old woman developed cervical lymphadenopathy, swelling and pain in both the ears, sore throat and fever three months prior to her visit to our hospital. She was referred because of blurred vison in her both eyes. At the initial examination, she had conjunctival hyperemia, mutton-fat keratic precipitates, anterior uveitis, synechia iridis posterior, and macular edema in her both eyes. In addition, redness and swelling of both auricles were observed. Laboratory analysis revealed an elevated serum C-reactive protein level, erythrocyte sedimentation rate, and white blood cell count. Ultrasonography of the auricle showed swelling and blood flow in the soft tissue of the auricular cartilage and pathological examination of the auricular biopsy showed infiltration of lymphocytes and neutrophils. We diagnosed her condition as relapsing polychondritis. We started treatment with steroid eye drop and subconjunctival steroid injections for uveitis and oral steroids for systemic symptoms. Uveitis and auricular inflammation improved and there was no further recurrence of ocular inflammation with oral prednisolone maintenance therapy.

Conclusion：Ophthalmologists should be aware that relapsing polychondritis is a potential cause of recurrent ocular inflammatory disorders associated with systemic inflammation.