A Patient with Relapsing Polychondritis who Had Been Diagnosed as Intractable Bronchial Asthma

Sato, Ryota; Ohshima, Nobuharu; Masuda, Kimihiko; Matsui, Hirotoshi; Higaki, Naoko; Inoue, Eri; Suzuki, Jun; Nagai, Hirokazu; Akagawa, Shinobu; Hebisawa, Akira; Shoji, Shunsuke

doi:10.2169/internalmedicine.51.7621

Cited by 11 publications

(4 citation statements)

References 12 publications

(12 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Reasons for delayed diagnosis in the present patient include: (1) transient improvement with steroid therapy, (2) dyspnoea and cough were the presenting symptoms, (3) absence of auricular chondritis and arthralgia (she however had non-specific costochondritis) and (4) late development of saddle nose deformity. Other case reports deduce similar reasons for airway involvement in RP misdiagnosed as asthma 12 17…”

Section: Discussionmentioning

confidence: 80%

See 1 more Smart Citation

Critical airway involvement in relapsing polychondritis

Gorard

Kadri

2014

BMJ Case Reports

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 80%

“…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

View full text Add to dashboard Cite

show abstract

“…Unfortunately, extrapulmonary presentations are frequently overlooked due to the reduced number of symptoms and the need for skillful clinical recognition, as respiratory symptoms are often misinterpreted and diagnosed as other diseases (11). Careful physical examination, meticulous chest radiograph interpretation, and recognition of unique pulmonary function test results are required to diagnose this challenging disease.…”

Section: Discussionmentioning

confidence: 99%

Tracheobronchial Involvement in Relapsing Polychondritis Diagnosed on Endobronchial Ultrasound

et al. 2013

View full text Add to dashboard Cite

Respiratory tract chondritis is not uncommon in patients with relapsing polychondritis (RP); however, diagnosing this condition remains problematic, especially in patients whose extrapulmonary manifestations do not predominate, as there are broad differential diagnoses of airway obstruction. We herein report the case of a 56-year-old man who presented with cough and dyspnea. Computed tomography of the chest demonstrated diffuse smooth thickening of the visualized tracheobronchial wall with a moderately narrowed lumen. Airway chondritis was diagnosed on endobronchial ultrasound following demonstration of thickening of the submucosal and cartilaginous layers in the anterior and lateral aspects of the bronchial wall, while the posterior region expressed less involvement. In conjunction with nasal and auricular chondritis, which were previously overlooked, RP was finally diagnosed.

show abstract

“…TP'li birçok hastaya başlangıçta yanlışlıkla astım tanısı konur ve geç evrelere kadar TP tanısı konulamaz. Astımın aksine, TP'deki hava yolu inflamasyonu inhale steroidlere ve bronkodilatörlere yanıt vermez 32 . Ölüm nedeni en sık solunum yolu hastalığı, kalp hastalığı ve kansere atfedilir 3 .…”

Section: Bulgularunclassified

Clinical Characteristics of Our Follow-Up Patients with the Rare Disease Relapsing Polychondritis: A Single-Center Study

Yağız

Coşkun

ÇELİK

et al. 2022

Uludağ Üniversitesi Tıp Fakültesi Dergisi

View full text Add to dashboard Cite

Relapsing polychondritis (RP) is an immune-mediated, systemic inflammatory and degenerative disease that affects cartilaginous structures particularly the ears, nose, eyes, joints, and respiratory tract and other tissues in the body. RP targets non-cartilaginous structures such as skin, kidney, heart, and the central nervous system in addition to cartilage-containing structures. Since it is a rare disease, data on its epidemiology are insufficient. It is prevalent between the ages of 40 and 60. Men and women are affected equally. Clinical characteristics vary among patients. The disease's rarity and broad clinical spectrum frequently result in misdiagnosis or delayed diagnosis. To prevent related complications and death, and to improve prognosis, early diagnosis and timely treatment of RP are crucial. Glucocorticoids, dapsone, disease-modifying antirheumatic drugs (DMARDs), and biologics are available as treatment options. The prognosis, such as the clinical presentation, varies depending on the extent of organ damage. 10 patients with RP had a mean onset age of 49.5±4.1. The median time between symptom onset and diagnosis was 3 months (2-60). 80% of the patients were male. The most frequent clinical manifestation is auricular chondritis (100%). During treatment, oral prednisolone was administered to all patients at least once. Intravenous methylprednisolone was administered to two patients. Due to ineffectiveness of DMARDs, one patient was switched to infliximab. A patient died due to pneumosepsis. This article aims to increase clinicians' awareness of this rare disease that can affect multiple systems by providing an overview of its pathogenesis, clinical course, diagnosis, and treatment.

show abstract

A Patient with Relapsing Polychondritis who Had Been Diagnosed as Intractable Bronchial Asthma

Cited by 11 publications

References 12 publications

Critical airway involvement in relapsing polychondritis

Critical airway involvement in relapsing polychondritis

Tracheobronchial Involvement in Relapsing Polychondritis Diagnosed on Endobronchial Ultrasound

Clinical Characteristics of Our Follow-Up Patients with the Rare Disease Relapsing Polychondritis: A Single-Center Study

Contact Info

Product

Resources

About