Critical airway involvement in relapsing polychondritis

Abstract

Relapsing polychondritis (RP) is a rare multisystem disease characterised by progressive inflammation and destruction of cartilaginous structures. Airway involvement in RP occurs in approximately 50% of cases. We present a 70-year-old woman with a diagnosis of early adult onset asthma. She required multiple hospital admissions for recurrent episodes of acute severe dyspnoea for 1 year. These were treated as asthma. Months later she developed saddle nose deformity and hoarseness of voice. CT revealed tracheal/bronchial wall thickening and luminal narrowing. Based on these findings, RP was diagnosed. Insertion of a tracheobronchial stent was necessary due to severe tracheobronchomalacia. This intervention improved the patient's dyspnoea immediately. This case is reported to raise awareness of airway involvement in RP and discuss its current management. Early diagnosis of RP is essential to allow prompt treatment and to decrease the risk of life-threatening airway collapse.

Nivolumab‐related tracheobronchial chondritis: Extremely rare manifestation of an immune‐related adverse effect

“…Multivariate analysis of 142 cases of RP with tracheal chondritis found that although tracheal chondritis was not a major cause of death, infectious diseases were common and were responsible for admission to intensive care 13 . Some previous reports of cases that initially occurred in the respiratory tract found that 5 of 11 patients required tracheostomy and three required stent placement, and four of these died of respiratory failure or pneumonia 14‐22 . This suggests that airway chondritis requiring local treatment, such as tracheostomy or stenting, has a poor prognosis.…”

Relapsing Polychondritis with Seronegative Spondyloarthritis: A Rare Case Report

“…Respiratory disturbance results from airway collapse secondary to destruction of tracheal rings or narrowingof the tracheal lumen caused by fibrous or inflammatory edema. Airway manifestations are the commonest cause of morbidity and mortality in the disease 6 . The management of respiratory tract involvement in relapsing polychondritis is important because sudden respiratory arrest mayoccur 5 , as manifested by an acute episode of choking in our patient.…”

“…Failure of early diagnosis of airway involvement in relapsing polychondritis can lead to irreversible cartilage damage. Steroids or other immunosuppressants are ineffective in these advanced cases, and would require airway interventions such as tracheostomy or tracheobronchial stents 6 .…”

“…Corticosteroids are considered to be the first line treatment. 6 Steroid sparing agents, such as cyclophosphamide, azathioprine, methotrexate and cyclosporine are also found to be helpful. Our patient achieved remission following cyclophosphamide pulse therapy.…”

A case of relapsing polychondritis developing tracheomalacia

“…Relapsing polychondritis (RP) is a rare, autoimmune, multisystem disease characterized by antibodies to type II collagen in its pathogenesis, which is accompanied by episodic inflammatory attacks in articular and non-articular cartilage tissue [1,2]. Airway manifestations are the most comment cause of morbidity and mortality in the disease [3].…”