多様な眼合併症を呈した再発性多発軟骨炎の1例

A case of recurrent polychondritis with various ocular symptoms

要約　目的：再発性多発軟骨炎（RPC）は比較的稀な難治性疾患である。今回多様な眼合併症を呈した1例を経験したので報告する。

症例：79歳，男性。X−20日頃から右耳痛，X−10日頃から右耳介軟骨腫脹とめまいの症状があり，X日に精査のため内科に入院となった。さらに右聴力障害，左視力低下が出現しRPCが疑われ，X＋6日に前医眼科を受診した。矯正視力は右1.2，左0.4，両眼の前部強膜炎と左眼の虹彩炎を認めた。耳介軟骨の生検でRPCと確定診断され，X＋12日にプレドニゾロン（PSL）20mgの内服が開始された。X＋13日の診察時に両眼の後部強膜炎と，OCTで乳頭黄斑間の網膜内層に血流障害を疑う高輝度病変を認め，左視力は（0.1）に低下した。X＋16日からPSL 60mgに増量され徳島大学病院（当院）に転院し，X＋19日に当院眼科に初診となった。後部強膜炎は改善傾向であったが，左視力は（0.2），中心フリッカ値は15Hzと不良であった。X＋1か月後のMRI STIR法で左視神経が高信号を示し，左中心暗点と乳頭蒼白化を認めたことから，急性期に左視神経炎も合併していたと考えられた。

結論：RPCは短期間に増悪し，眼球から視神経に至る広範囲に病変をきたす可能性がある。耳介軟骨腫脹を含めた全身所見を確認し，早期に診断，治療を開始する必要がある。

Abstract　Purpose：Relapsing polychondritis（RPC）is a relatively rare and intractable disease. Here, we report a case of RPC that presented with various ocular complications.

Case：A 79-year-old man had pain in his right ear from day X−20, and swelling of the right auricular cartilage and dizziness from day X−10. On day X, he was admitted to the internal medicine department for investigation. Furthermore, RPC was suspected because of the appearrance of hearing loss in the right ear and decreased visual acuity in the left eye, so the patient visited an eye clinic on X＋6. The best corrected visual acuity was 1.2 on the right and 0.4 on the left, and anterior scleritis in both eyes and iritis in the left eye were observed. An auricular cartilage biopsy confirmed the diagnosis of RPC, and prednisolone（PSL） 20 mg/day was started on day X＋12. On day X＋13, posterior scleritis in both eyes was observed, and optical coherence tomography revealed a high-intensity lesion in the inner retinal layer between the papilla and macula, that was suspected to be a blood flow disorder. The visual acuity in the left eye decreased to 0.1. From day X＋16, the PSL dose was increased to 60 mg and the patient was transferred to our hospital. The patient first visited our clinic on day X＋19. Although the posterior scleritis was improving, the visual acuity of his left eye was 0.2 and the critical flicker frequency was 15 Hz. X＋1 month later, magnetic resonance imaging short tau inversion recovery showed high signal intensity in the left optic nerve. Moreover, a left central scotoma on the visual field testing and papillary pallor were observed, suggesting the presence of acute-phase left optic neuritis.

Conclusion：RPC, worsens over a short period of time and may cause lesions from the eyeball to the optic nerve. In addition to eye inflammation, systemic findings of RPC including auricular cartilage swelling must be confirmed, and early diagnosis and treatment are necessary.