Clinical Characteristics of Relapsing Polychondritis: A Report of 8 Cases in Japan.
Kurume Med J
; 65(2): 47-53, 2019 May 16.
Article
en En
| MEDLINE
| ID: mdl-30853688
ABSTRACT
OBJECTIVES:
Relapsing polychondritis (RP) is a very rare autoimmune disorder characterized by recurrent episodes of inflammation and destruction of cartilaginous tissues. We examined the clinical characteristics, management, and outcomes of Japanese RP patients.METHODS:
We identified 8 RP cases in our department between 2003 and 2017. Detailed clinical features, testing, treatment, and outcomes were recorded.RESULTS:
The mean time from symptom onset to diagnosis was 9 months. Four cases presented with auricular chondritis and laryngotracheal involvement and 3 cases presented with a saddle nose deformity. Anti-type II collagen antibody was positive in 5 of 6 cases. Of 3 cases with associated diseases (rheumatoid arthritis, ulcerative colitis, and Sjögren's syndrome), 2 died of respiratory failure.CONCLUSIONS:
When RP is diagnosed, early computed tomography or pulmonary function testing is essential to enable early treatment. Undiagnosed airway involvement can cause tracheobronchial wall fibrosis, leading to fixed stenosis.Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Policondritis Recurrente
Tipo de estudio:
Prognostic_studies
Límite:
Adult
/
Aged
/
Female
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Humans
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Male
/
Middle aged
País como asunto:
Asia
Idioma:
En
Año:
2019
Tipo del documento:
Article