Clinical characteristics and treatment outcomes of patients with relapsing polychondritis with airway involvement

Hong, Goohyeon; Kim, Hojoong

doi:10.1007/s10067-013-2279-2

Cited by 31 publications

(27 citation statements)

References 23 publications

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“…Airway involvement while uncommon at presentation, occurs in approximately 50% of patients with RP 9. Clinical manifestations of RP with airway involvement include dyspnoea, cough, wheeze, stridor, hoarseness and aphonia 10.…”

Section: Discussionmentioning

confidence: 99%

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Critical airway involvement in relapsing polychondritis

Gorard

Kadri

2014

BMJ Case Reports

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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.

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Section: Discussionmentioning

confidence: 99%

“…In RP (unlike asthma), inhaled corticosteroids and bronchodilators are ineffective, spirometry demonstrates upper airway obstruction, CT imaging can reveal thickening and stenosis of the airways, and bronchoscopy can show inflammation, narrowing or collapse of the airways 9 12 18 19…”

Section: Discussionmentioning

confidence: 99%

Critical airway involvement in relapsing polychondritis

Gorard

Kadri

2014

BMJ Case Reports

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“…Up to 50% of patients with relapsing polychondritis have airway disease, which can occur anytime during its course and is typically diffuse [73][74][75] (Fig. 14).…”

Section: Relapsing Polychondritismentioning

confidence: 99%

“…Early detection and treatment are crucial to delay destruction of cartilage [75]. Continued positive airway pressure, various types of bronchoscopic intervention such as laser therapy, balloon dilatation, and stenting, and tracheostomy in severe stenosis of the upper trachea can improve the symptoms related to airway involvement [73][74][75].…”

Section: Relapsing Polychondritismentioning

confidence: 99%

Imaging of Large Airways Disorders

Heidinger

Occhipinti

Eisenberg

et al. 2015

American Journal of Roentgenology

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“…RP is an immune-mediated and multisystem disease that causes progressive inflammatory destruction of the cartilage 1. About one-third of the RP cases have airway involvement, including constriction and malacia in the trachea and relatively large bronchi, which is usually associated with a worse prognosis 2.…”

Section: Descriptionmentioning

confidence: 99%

Mediastinal emphysema: a rare respiratory complication of relapsing polychondritis

Wang

Jiao

2013

BMJ Case Reports

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DESCRIPTIONA 50-year-old woman presented to our department with a 6-year history of recurrent dyspnoea and cough. Six year ago she started to have external ear and nasal pain, episcleritis and dyspnoea. She was diagnosed with relapsing polychondritis (RP). Her disease was under control after treatment with a high dose of oral prednisone (1 mg/kg/day) and intravenous cyclophosphamide. The patient experienced a flare of respiratory symptoms 3 months before admission and her prednisone dose was increased to 60 mg/kg/day. A CT scan of the chest showed only airway stenosis of trachea and bilateral main bronchi, but with mostly clear lung fields ( figure 1A). However 3 weeks before admission the patient presented with severe dyspnoea, cough and stridor again, the dose of the prednisone was then tapered to 50 mg/kg/day. The patient reported no trauma or other medical history. On admission, physical examination revealed sibilant wheezing sound on the entire lung field. Laboratory findings demonstrated increased serum levels of white cell count and erythrocyte sedimentation rate. CT of the chest showed pneumomediastinum, bilateral pneumohypoderma and slight left-sided pneumothorax (figure 1B). The patient was treated with a high dose of intravenous methylprednisolone therapy and cyclophosphamide. Her symptoms relieved quickly and a repeated CT scan preformed after 1 week showed significant improvement of the pneumomediastinum and pneumohypoderma ( figure 1C).RP is an immune-mediated and multisystem disease that causes progressive inflammatory destruction of the cartilage.1 About one-third of the RP cases have airway involvement, including constriction and malacia in the trachea and relatively large bronchi, which is usually associated with a worse prognosis.2 However, pneumomediastinum has been rarely reported in RP except in patients who underwent intervention for airway problems or had underlying pulmonary fibrosis or bullae.3 If the symptoms of pneumomediastinum develop in a patient with RP as in our case, the diagnoses of the relapse of RP or pulmonary infections would be more easily suspected. It was supposed that the rupture of the damaged tracheal cartilage might contribute to the air leak in our case. Learning points▸ Dyspnoea, cough and stridor are the most common respiratory symptoms of relapsing polychondritis, which is usually associated with constrictions of the trachea or main bronchi. ▸ Pneumomediastinum is an uncommon pulmonary presentation of relapsing polychondritis and it should be considered when patients with RP are presented with dyspnoea.Contributors YJ and SW collected the clinical information, treated the patient and wrote the manuscript. Funding This study was funded by Beijing Nova Program (2008B49).Competing interests None. Patient consent Obtained.Provenance and peer review Not commissioned; externally peer reviewed. REFERENCES

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Clinical characteristics and treatment outcomes of patients with relapsing polychondritis with airway involvement

Cited by 31 publications

References 23 publications

Critical airway involvement in relapsing polychondritis

Critical airway involvement in relapsing polychondritis

Imaging of Large Airways Disorders

Mediastinal emphysema: a rare respiratory complication of relapsing polychondritis

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