A case of life-threatening refractory polychondritis successfully treated with combined intensive immunosuppressive therapy with methotrexate.
Relapsing polychondritis (RP) is a rare disease of unknown etiology causing systematic inflammation and resulting in destruction of cartilaginous tissues. We describe here an 18-year-old Japanese woman who developed severe airway stenosis as the initial symptom with auricular, nasal, and ocular inflammation. The effect of high dose oral steroid, methylprednisolone pulse therapy, and cyclophosphamide was temporary and her conditioning was worsening. Finally we added methotrexate to the immunosuppressive treatment and achieved reduction of disease activity. This case illustrates the potentially fatal sudden onset of airway inflammation that can occur with this disorder, and the effectiveness of methotrexate.
Yamaoka
Kunihiro
K
First Department of Internal Medicine, University of Occupational and Environmental Health, Japan, 1-1 Iseigaoka, Yahata-nishi-ku, Kitakyushu 807-8555, Japan. yamaoka@med.uoeh-u.ac.jp
Saito
Kazuyoshi
K
Hanami
Kentaro
K
Nakayamada
Shingo
S
Nawata
Masao
M
Iwata
Shigeru
S
Azuma
Taeko
T
Tanaka
Yoshiya
Y
D000293:Adolescent; D018501:Antirheumatic Agents / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007249:Inflammation / Q000209:etiology; D008727:Methotrexate / Q000627:therapeutic use*; D008775:Methylprednisolone / Q000627:therapeutic use; D011081:Polychondritis, Relapsing / Q000000981:diagnostic imaging / Q000188:drug therapy*; D014057:Tomography, X-Ray Computed
D018501:Antirheumatic Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone; D008727:Methotrexate