Fatal relapsing tracheobronchial polychondritis diagnosed at autopsy

Abstract

SUMMARYA 31-year-old woman was referred to chest clinic with an 18-month history of cough, breathlessness and weight loss. She had previously been treated unsuccessfully with bronchodilators. In clinic she was found to have vocal hoarseness and harsh, monophonic inspiratory breath sounds. A bronchoscopy was arranged, but the patient was found dead before it could be performed. A postmortem examination with histology found evidence of active and chronic inflammation as well as destruction of cartilage, in keeping with a diagnosis of relapsing tracheobronchial polychonditis. BACKGROUND

Patient Perception of Disease‐Related Symptoms and Complications in Relapsing Polychondritis

“…RP is defined by inflammation of cartilaginous structures and the disease course and clinical manifestations are highly variable. End organ complications due to inflammation of cartilage and proteoglycan rich structures are diverse and include deformity of the ears(2), nose(3) and chest(4), subglottic stenosis (5), obstructive lung disease, tracheomalacia(6), bronchomalacia(7), recurrent pneumonia(8), mitral and aortic regurgitation(9, 10), large and medium vessel aneurysms(11), blindness(12), deafness(13), renal insufficiency(14), chronic pain, and sudden death(15–17). The protean aspects of RP pose diagnostic challenges.…”

Relapsing polychondritis: an unusual cause of sore throat, painful ear swelling and bilateral costal margin pain

“…RP is defined as an immune-mediated inflammation of cartilaginous tissues that can affect the ear, sclera, nose or various joints of the body 11 12. Airway involvement, including symptoms such as dyspnoea, hoarseness or stridor, may also occur in up to 50% of cases 13.…”