Corticosteroid Therapy for a Patient with Relapsing Polychondritis Complicated by IgG4-Related Disease
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- Yamasue Mari
- Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine
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- Nureki Shin-ichi
- Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine
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- Matsumoto Hiroyuki
- Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine
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- Kan Takamasa
- Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine
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- Hashimoto Takehiro
- Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine
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- Ushijima Ryoichi
- Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine
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- Usagawa Yuko
- Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine
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- Kadota Jun-ichi
- Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine
抄録
Relapsing polychondritis (RP) is a rare systemic disorder characterized by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease characterized by the infiltration of IgG4-bearing plasma cells into systemic organs. Although 25% to 35% of patients with RP have a concurrent autoimmune disease, coexistence of RP and IgG4-RD is rare. We herein report a case of RP complicated by IgG4-RD. A 63-year-old man developed recurrent bilateral ear pain and swelling, recurrent blurred and decreased vision, and migratory multiple joint pain, sequentially within one year. Fourteen months after the first symptom, he experienced dry cough and dyspnea with exertion. A computed tomography (CT) scan detected interstitial pneumonia, swelling of bilateral submandibular glands, bilateral hilar and mediastinal lymphadenopathy, and several nodules in bilateral kidneys. His serum levels of IgG and IgG4 were elevated. The biopsy specimen of auricular cartilage showed infiltrations of inflammatory cells and fibrosis consistent with RP. The IgG4-positive cells were not observed in auricular cartilage. The patient met the diagnostic criteria of RP, including bilateral auricular chondritis, conjunctivitis, iritis and polyarthritis. The biopsy specimens of lung and kidney revealed the significant infiltrations of IgG4-positive plasma cells and fibrosis. We also diagnosed him as having IgG4-RD, affecting bilateral submandibular glands, hilar and mediastinal lymph nodes, lungs, and kidneys. Thus, RP preceded the onset of IgG4-RD. Corticosteroid therapy improved the symptoms and CT scan findings. In conclusion, RP and IgG4-RD do coexist; however, the pathogenesis of their coexistence is unknown.
収録刊行物
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- The Tohoku Journal of Experimental Medicine
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The Tohoku Journal of Experimental Medicine 239 (3), 223-230, 2016
東北ジャーナル刊行会