A new role for magnetic resonance imaging in the diagnosis of laryngeal relapsing polychondritis

Abstract

Background Relapsing polychondritis involving the upper airway is a rare cause of airflow obstruction and hoarseness. The diagnosis of relapsing polychondritis depends on clinical signs, characteristic findings on cartilage biopsy, and response to treatment. Delays in diagnosis and treatment can increase the morbidity and mortality of the disease. Methods We present a case report of primary laryngeal relapsing polychondritis. Results Serologic testing, direct laryngoscopy, and endoscopic biopsy could not establish the diagnosis of relapsing polychondritis. Magnetic resonance imaging (MRI) examination demonstrated findings consistent with the histopathological diagnosis obtained on open biopsy. Follow‐up MRI after treatment showed resolution of the initial findings. Conclusion This case demonstrates the usefulness of MRI in the diagnosis and management of relapsing polychondritis involving the upper airway. © 1999 John Wiley & Sons, Inc. Head Neck 21: 484–489, 1999.

Obstructive sleep apnoea/hypopnoea as the initial presentation of relapsing polychondritis

“…Distal airways are rarely involved and may also be associated with impaired mucociliary clearance. Spirometry, flow‐volume loops and measures of airway resistance together are more sensitive than bronchoscopy and radiographic imaging in detecting the presence and nature of the airway obstruction and determining its severity (13,15). Obstruction is variable in the early stages of the disease, and the ratio of maximal expiratory to inspiratory flow at 50% of VC is either reduced (intrathoracic obstruction) or elevated (in extrathoracic obstruction) (13,14).…”

“…At this stage, our patient appears to have early involvement of the upper airway alone manifesting as extrathoracic obstruction on his flow‐volume loop. It is not uncommon to have normal endoscopic/bronchoscopic appearances with marked functional impairment (15–17). Dynamic abnormalities of the upper airway in patients in the awake state may be asymptomatic but have been shown to manifest as sleep apnoea at night (17).…”

Relapsing Polychondritis with Tracheobronchial Involvement: A Detailed Description of Two Pediatric Cases and Review of the Literature

“…Magnetic resonance imaging (MRI) with contrast enhancement may be useful in evaluating the airways of patients with RP. Even in the presence of subclinical disease, it distinguishes fibrosis from inflammation (unlike a CT scan) and inflammation from edema 45 . PET-CT may help to detect chondritis indirectly, as in our cases 46 47 .…”

Case Report: A Swollen and Red Ear

“…Diagnosis is based on clinical criteria, but the demonstration of necrotizing chondritis, perivascular inflammatory infiltrate, and fibrosis at biopsy can be necessary in selected cases 3 . Magnetic resonance imaging will probably decrease the need for histological confirmation 4 . Chondritis can result in deformities such as saddle nose or cauliflower ear; 3 hearing impairment and vestibular symptoms have also been reported.…”