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Relapsing Polycondritis With a Cobble-Sone Appeara ...
Relapsing Polycondritis With a Cobble-Sone Appearance of Tracheal Mucosa
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This case report describes a 77-year-old man with one month of low-grade fever, dry cough, dyspnea, and nasal obstruction, initially without obvious ear or eye findings. He had a history of bronchial asthma and no smoking or alcohol use. On examination, he was tachypneic, unable to speak in full sentences, and had bilateral late-inspiratory crackles.<br /><br />Chest CT showed smooth thickening of the cartilaginous trachea. Bronchoscopy revealed a rare “cobble-stone” appearance of the tracheal mucosa with severe erythema and edema, raising suspicion for relapsing polychondritis (RP). Laboratory studies showed marked inflammation, including elevated WBC, CRP, and ESR, while autoimmune markers such as ANA, anti-CCP, PR3-ANCA, and MPO-ANCA were negative. Nasal cartilage biopsy was nonspecific.<br /><br />RP is a rare autoimmune disease that causes recurrent inflammation of cartilaginous structures, commonly affecting the ears, nose, eyes, and large airways. Airway disease can occur in up to half of patients and may progress to irreversible tracheomalacia if diagnosis is delayed. Because this patient lacked the classic auricular and ocular signs, diagnosis was challenging.<br /><br />He was treated with high-dose glucocorticoids followed by tapering and tocilizumab. His symptoms and bronchoscopic airway inflammation improved markedly by day 46, and pulmonary function also improved. The report emphasizes that bronchoscopy is useful not only for diagnosis but also for monitoring disease progression. Prompt recognition of atypical RP and early rheumatology involvement are important to prevent irreversible airway damage and improve outcomes.
Asset Subtitle
Yoshitaka Tomoda
Meta Tag
Author List
Haruka Tsuchiya, Hiroaki Tanaka, Kenji Motohashi, Ryo Itoh, Yoshitaka Tomoda, Yusuke Yasumoto
Category
Clinical Vignettes
Concept
Relapsing Polychondritis
Concept
Cartilaginous Inflammation
Concept
Airway involvement
Concept
Early Diagnosis
Concept
Airway Damage
Distinguished
Non-Finalist
Presenter Organization
Itabashi Chuo Medical Center
Presenting Author
Yoshitaka Tomoda
Track
Adult
Keywords
relapsing polychondritis
tracheal inflammation
bronchoscopy
cobble-stone appearance
airway disease
glucocorticoid therapy
tocilizumab
tracheomalacia
cartilaginous trachea
autoimmune disease
Relapsing Polychondritis
Cartilaginous Inflammation
Airway involvement
Early Diagnosis
Airway Damage
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