Relapsing polychondritis – analysis of symptoms and criteria

Maciążek-Chyra, Beata; Szmyrka, Magdalena; Skoczyńska, Marta; Sokolik, Renata; Lasocka, Joanna; Wiland, Piotr

doi:10.5114/reum.2019.83234

Cited by 17 publications

(12 citation statements)

References 28 publications

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“…However, we do agree that this may not always be the case. In the study of Maciazek-Chyra et al [12], one patient out of 10 (20%) showed costal chondritis and Borgia et al [1] also reported that involvement of costal cartilages occurs in 35% of patients, but rare at the moment of diagnosis. This means that though our patient showed no signs of costal chondritis at diagnosis or time of surgery, it may occur within lifetime.…”

Section: Discussionmentioning

confidence: 97%

Reconstructive rhinoplasty with costal cartilage grafting: A case report of relapsing polychondritis

Lee

Choi

2019

Arch Craniofac Surg

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Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology characterized by recurrent episodes of inflammation and the destruction of cartilaginous tissues, primarily involving the ear, nose, and the respiratory tract. Nasal chondritis is present in 24% of patients at the time of diagnosis and develops subsequently in 53% throughout the diseases progress. Progressive destruction of nasal cartilage leads to the characteristic flattening of the nasal bridge, resulting in the saddle nose deformity. In patients with RP, surgical management for saddle nose is carefully decided due to the disease relapsing characteristics. We present a RP patient with a saddle nose deformity who underwent reconstruction rhinoplasty with autologous costal cartilage grafting. At 6-month follow-up, the patient retained good esthetic results and showed neither complication nor relapse of RP.

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Section: Discussionmentioning

confidence: 97%

Reconstructive rhinoplasty with costal cartilage grafting: A case report of relapsing polychondritis

Lee

Choi

2019

Arch Craniofac Surg

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“…3,4 Tracheal stent implantation is an effective surgical treatment for preserving airway patency in patients with tracheal stenosis due to RP. 5,6 Silicone stents are preferred for patients with mild tracheal stenosis. The tracheal stenosis in the present case was severe, and a silicone stent could not be implanted because of the rigid bronchial tube.…”

Section: Discussionmentioning

confidence: 99%

Application of extracorporeal membrane oxygenation to treatment of serious tracheal stenosis caused by relapsing polychondritis: a case report and literature review

Zhang

et al. 2021

J Int Med Res

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Relapsing polychondritis (RP) is a multisystemic rheumatic disease characterized by widespread and potentially destructive inflammatory lesions of the cartilage. The rarity of this disease and the lack of pathological diagnostic laboratory tests can occasionally lead to delayed diagnosis. We herein describe a 51-year-old woman with RP. She was sent to our hospital 4 days after the development of an upper respiratory tract infection with difficulty breathing. Her clinical condition significantly improved after the performance of extracorporeal membrane oxygenation support in an awake state, implantation of a tracheal stent, and administration of steroid therapy. Airway involvement of RP may be life-threatening. In this case, endotracheal intubation would have undoubtedly been very dangerous. Extracorporeal membrane oxygenation can be performed in an awake state to maintain oxygenation and improve the chance of survival.

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“…Several sets of diagnostic criteria, which are mainly based on the presence of symptoms suggesting chondritis or arthritis at multiple sites, have been proposed previously [9–13]. These sets of criteria, however, have limitations in terms of sensitivity and accuracy, as was demonstrated by recent retrospective case analyses [9,10]. Histologic confirmation has been included into the criteria proposed by Demiani and Levine [12], but the histologic findings are not specific, and the site of inflammation is not always safe for biopsy.…”

Section: Discussionmentioning

confidence: 99%

“…RP is difficult to diagnose due to the rarity, the episodic nature, and its diverse clinical presentation. Significant delays in diagnosis have been described [4,7–9]. Currently, these are still no standardized guidelines for the diagnosis of RP.…”

Section: Discussionmentioning

confidence: 99%

“…Currently, these are still no standardized guidelines for the diagnosis of RP. Several sets of diagnostic criteria, which are mainly based on the presence of symptoms suggesting chondritis or arthritis at multiple sites, have been proposed previously [9–13]. These sets of criteria, however, have limitations in terms of sensitivity and accuracy, as was demonstrated by recent retrospective case analyses [9,10].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Relapsing polychondritis with isolated tracheal involvement and airway‐only symptoms

Wang

Weng

Cheng

et al. 2020

Respirology Case Reports

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Relapsing polychondritis (RP) is a rare autoimmune disorder, characterized by the inflammation of cartilaginous structures and proteoglycan‐rich tissues. Due to its rarity and the notoriously variable presentations, the diagnosis of RP could be challenging. We report an unusual case of RP with isolated tracheal involvement and very non‐specific symptoms of exertional dyspnoea and dry cough. The initial chest radiograph showed long‐segment narrowing of the trachea, and the computed tomography of the chest revealed thickened cartilaginous walls of the trachea, while the posterior membranous portion was spared. The tracheal narrowing was readily observed under bronchoscopy. The patient was treated with oral prednisolone. Although the subsequent course was transiently complicated by an episode of severe Pneumocystis jirovecii pneumonia with acute respiratory distress syndrome, the patient overall responded well to systemic corticosteroid therapy. No new symptoms developed during a two‐year follow‐up.

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Relapsing polychondritis – analysis of symptoms and criteria

Cited by 17 publications

References 28 publications

Reconstructive rhinoplasty with costal cartilage grafting: A case report of relapsing polychondritis

Reconstructive rhinoplasty with costal cartilage grafting: A case report of relapsing polychondritis

Application of extracorporeal membrane oxygenation to treatment of serious tracheal stenosis caused by relapsing polychondritis: a case report and literature review

Relapsing polychondritis with isolated tracheal involvement and airway‐only symptoms

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