On-line First

J Rheum Dis

Published online March 10, 2025

© Korean College of Rheumatology

Resolution of long-lasting laryngeal manifestations using glucocorticoids and tumor necrosis factor-α inhibitor therapy in a patient with mouth and genital ulcers with inflamed cartilage syndrome

Tomohiro Suzuki, M.D.1 , Toshihiko Komai, M.D., Ph.D.1 , Misaki Koyama, M.D.2 , Kento Koda, M.D.2 , Rumi Ueha, M.D., Ph.D.2,3 , Hirofumi Shoda, M.D., Ph.D.1 , Keishi Fujio, M.D., Ph.D.1

1Department of Allergy and Rheumatology, Graduate School of Medicine, 2Department of Otolaryngology and Head and Neck Surgery, The University of Tokyo, 3Swallowing Center, The University of Tokyo Hospital, Tokyo, Japan

Correspondence to : Toshihiko Komai, https://orcid.org/0000-0001-5890-3606
Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyoku, Tokyo 113-8655, Japan. E-mail: KOMAIT-INT@h.u-tokyo.ac.jp

Received: December 9, 2024; Revised: February 12, 2025; Accepted: March 1, 2025

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is a rare disorder characterized by features of both relapsing polychondritis (RP) and Behçet’s disease (BD), with multi-organ involvement, including inflammation and destruction of cartilaginous tissues. This report describes a patient with MAGIC syndrome who responded to immunosuppressive therapy for long-lasting laryngeal inflammation and provides the first description of a patient with MAGIC syndrome positive for human leukocyte antigen (HLA)-A26. Here we present a 49-year-old male with recurrent oral and genital ulcers, hoarseness, and swallowing difficulties. Laryngoscopy showed bilateral vocal fold immobility, and contrast-enhanced magnetic resonance imaging (MRI) demonstrated inflammation in the cricoid, arytenoid, and auricular cartilages. The patient was diagnosed with MAGIC syndrome based on the presence of both RP and BD criteria. Treatment with high-dose corticosteroids and adalimumab biosimilar resulted in significant improvement in hoarseness and MRI findings of cartilage inflammation. This case highlights the effective use of immunosuppressive therapy for managing long-lasting laryngeal involvement in MAGIC syndrome, potentially avoiding the need for invasive interventions such as tracheostomy.

Keywords Mouth and genital ulcers with inflamed cartilage syndrome, Behçet’s disease, Relapsing polychondritis, Hoarseness, Vocal cords

Article

On-line First

J Rheum Dis

Published online March 10, 2025

Copyright © Korean College of Rheumatology.

Resolution of long-lasting laryngeal manifestations using glucocorticoids and tumor necrosis factor-α inhibitor therapy in a patient with mouth and genital ulcers with inflamed cartilage syndrome

Tomohiro Suzuki, M.D.1 , Toshihiko Komai, M.D., Ph.D.1 , Misaki Koyama, M.D.2 , Kento Koda, M.D.2 , Rumi Ueha, M.D., Ph.D.2,3 , Hirofumi Shoda, M.D., Ph.D.1 , Keishi Fujio, M.D., Ph.D.1

1Department of Allergy and Rheumatology, Graduate School of Medicine, 2Department of Otolaryngology and Head and Neck Surgery, The University of Tokyo, 3Swallowing Center, The University of Tokyo Hospital, Tokyo, Japan

Correspondence to:Toshihiko Komai, https://orcid.org/0000-0001-5890-3606
Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyoku, Tokyo 113-8655, Japan. E-mail: KOMAIT-INT@h.u-tokyo.ac.jp

Received: December 9, 2024; Revised: February 12, 2025; Accepted: March 1, 2025

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is a rare disorder characterized by features of both relapsing polychondritis (RP) and Behçet’s disease (BD), with multi-organ involvement, including inflammation and destruction of cartilaginous tissues. This report describes a patient with MAGIC syndrome who responded to immunosuppressive therapy for long-lasting laryngeal inflammation and provides the first description of a patient with MAGIC syndrome positive for human leukocyte antigen (HLA)-A26. Here we present a 49-year-old male with recurrent oral and genital ulcers, hoarseness, and swallowing difficulties. Laryngoscopy showed bilateral vocal fold immobility, and contrast-enhanced magnetic resonance imaging (MRI) demonstrated inflammation in the cricoid, arytenoid, and auricular cartilages. The patient was diagnosed with MAGIC syndrome based on the presence of both RP and BD criteria. Treatment with high-dose corticosteroids and adalimumab biosimilar resulted in significant improvement in hoarseness and MRI findings of cartilage inflammation. This case highlights the effective use of immunosuppressive therapy for managing long-lasting laryngeal involvement in MAGIC syndrome, potentially avoiding the need for invasive interventions such as tracheostomy.

Keywords: Mouth and genital ulcers with inflamed cartilage syndrome, Behç,et’s disease, Relapsing polychondritis, Hoarseness, Vocal cords

JRD
Jan 01, 2025 Vol.32 No.1, pp. 1~7
COVER PICTURE
Cumulative growth of rheumatology members and specialists (1980~2024). Cumulative distribution of the number of the (A) Korean College of Rheumatology members and (B) rheumatology specialists. (J Rheum Dis 2025;32:63-65)

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