A Case of Relapsing Polychondritis With Severe Obstructive Ventilatory Defect Caused by a Single Flare‐Up

ABSTRACT

Relapsing polychondritis (RP) is a rare progressive autoimmune disorder affecting cartilaginous and/or proteoglycan‐rich structures. Tracheobronchial involvement is observed in almost half of RP cases and significantly impacts the prognosis. We present a case of RP with a rapidly progressive obstructive ventilatory defect caused by a single flare‐up, despite significant improvements in the thickening of the airway walls after starting steroid administration. Although the airway walls appeared normal on computed tomography after starting treatment, tracheobronchial cartilage tissues were considered to have been extensively destroyed by chondritis, leading to tracheobronchomalacia and resulting in dynamic respiratory collapse. Preventing fibrosis of the airway walls caused by recurrent flare‐ups will be extremely important to avoid fixed airway stenosis.

We present a case of relapsing polychondritis with a rapidly progressive obstructive ventilatory defect caused by a single flare‐up, despite significant improvements in the thickening of the airway walls after starting steroid administration. Although the airway walls appeared normal on computed tomography after starting treatment, tracheobronchial cartilage tissues were considered to have been extensively destroyed by chondritis, leading to tracheobronchomalacia.

1. Introduction

Relapsing polychondritis (RP) is a rare autoimmune disorder characterised by recurrent episodes of inflammation of cartilaginous and/or proteoglycan‐rich structures. Although the 10‐year survival rate is estimated to be 91% [1], tracheobronchial involvement remains the main cause of severe symptoms affecting prognosis. We present herein a case of RP with a rapidly developing obstructive ventilatory defect, which was caused by a single flare‐up of airway inflammation.

2. Case Report

A 66‐year‐old never‐smoker woman was admitted to our hospital with hoarseness and rapidly worsening dyspnea. For 1 month prior to admission, she had been suffering from a dry cough. Three days prior to admission, she attended our outpatient clinic with a sore throat and was diagnosed with acute pharyngitis. Pulmonary function testing (PFT) at that time showed an expiratory flow‐volume almost within the normal range, a forced vital capacity (FVC) of 2.00 L (88.1% of predicted), a forced expiratory volume in 1 s (FEV₁) of 1.54 L (91.6% of predicted) and a FEV₁/FVC of 77.0% (Figure 1A). However, the testing also revealed flattening of the inspiratory flow‐volume curve, suggesting extra‐thoracic upper airway obstruction. Three months earlier, the patient had experienced bilateral auricular pain that had resolved spontaneously. She reported no joint pain and had no history of asthma. She had been diagnosed with uveitis at 64 years old and had been treated with steroid eye drops ever since.

FIGURE 1.

(A) A flow‐volume curve at the initial visit shows an almost‐normal expiratory flow‐volume, but reveals flattening of the inspiratory flow‐volume curve, suggesting extra‐thoracic upper airway obstruction. (B) Four months after initiating steroid therapy, the flow‐volume curve reveals a severe obstructive ventilatory defect.

On physical examination, body temperature was 36.5°C. Oxygen saturation was 94% in room air. The nose and auricles showed no redness, swelling or deformity. Auscultation revealed inspiratory stridor in the neck and mild wheezing in both lung fields. Laboratory data showed a total leukocyte count of 11,530/μL, a serum C‐reactive protein level of 22.07 mg/dL, and an erythrocyte sedimentation rate of 84 mm/h. Serum autoantibodies for collagen vascular diseases, including antineutrophil cytoplasmic antibodies, were negative. Serum immunoglobulin G4 level was normal. Tests for anti‐type II collagen antibodies and measurements of serum matrix metalloproteinase‐3 levels were not conducted. Chest radiography showed no abnormalities in the lung fields. However, contrast‐enhanced computed tomography (CT) revealed swelling of the cartilaginous parts of the trachea and main bronchi, as well as thickening of the vascular walls of the left common carotid and subclavian arteries (Figure 2A). On ¹⁸F‐fluorodeoxyglucose positron emission tomography/CT, significant uptake of ¹⁸F was evident in the cartilages of the larynx, trachea and main bronchi, as well as in the costal cartilages and the vascular walls of the left common carotid and subclavian arteries (Figure 2B). Echocardiography revealed no abnormalities, including valvular disease. Bronchoscopy revealed subepithelial edema and vascular proliferation, and subepithelial protruding lesions at the carina and the bifurcation between the right middle and lower lobe bronchi (Figure 3A). Haematoxylin and eosin staining of biopsy specimens from the protruding lesions demonstrated the presence of eosinophilic microfragments of anucleated cartilage tissues within the degenerated stroma (Figure 3B). Cartilage tissue with a worm‐eaten appearance was apparent adjacent to soft tissue with dense infiltration of inflammatory cells.

FIGURE 2.

(A) Computed tomography (CT) on admission reveals swelling of the cartilaginous parts of the trachea and main bronchi (yellow arrows), as well as thickening of the vascular walls of the left common carotid and subclavian arteries (blue arrows). (B) On ¹⁸F‐fluorodeoxyglucose positron emission tomography/CT, significant ¹⁸F uptake is seen for cartilages of the larynx, trachea and main bronchi, as well as in the costal cartilages and vascular walls of the left common carotid and subclavian arteries (yellow arrows). (C) CT taken 4 months after starting steroid treatment shows significant improvement in swelling of the respiratory tract and vascular walls.

FIGURE 3.

(A) Bronchoscopy on admission reveals subepithelial edema and vascular proliferation, and subepithelial protruding lesions at the carina and the bifurcation between right middle and lower lobe bronchi (yellow arrows). (B) Haematoxylin and eosin staining of the biopsy specimens demonstrates eosinophilic microfragments of anucleated cartilage tissues within the degenerated stroma (yellow arrows). Cartilage tissue showing a worm‐eaten appearance is adjacent to soft tissue with dense infiltration of inflammatory cells.

According to the diagnostic criteria described by Damiani and Levine [2], the patient was diagnosed with RP based on clinical findings including respiratory tract chondritis and ocular inflammation (uveitis), and the histopathological findings of chondritis. Thickening of the vascular walls (probably caused by vasculitis) and bilateral auricular pain (probably caused by chondritis) were also thought to be due to RP. The patient was started on prednisolone at 30 mg/day, which rapidly resolved her respiratory symptoms. The prednisolone dose was tapered gradually and tacrolimus was added as an immunosuppressant. Approximately 4 months later, when the patient was taking prednisolone at 12.5 mg/day, CT showed significant improvements in swelling of the cartilaginous parts of the trachea and bronchi, as well as in the thickening of the vascular walls of the left common carotid and subclavian arteries (Figure 2C). However, PFT at that time demonstrated a severe obstructive pattern: FVC, 2.17 L (97.7% of predicted); FEV₁, 0.81 L (50.6% of predicted); and FEV₁/FVC, 37.3% (Figure 1B). This obstructive ventilatory defect was attributed to tracheobronchomalacia caused by RP. The dose of prednisolone is being carefully reduced at the outpatient clinic. And administration of biologics (infliximab) has been initiated.

3. Discussion

Respiratory impairment is observed in 30%–60% of RP cases and can significantly impact prognosis [3]. Dion et al. identified the respiratory subtype of RP, which was associated with infections and admission to the intensive care unit [1]. Episodic inflammation of the tracheobronchial cartilage can lead to progressive morphological deformation and functional impairment, resulting in tracheobronchomalacia and ultimately respiratory failure. Early diagnosis and treatment of RP are thus critical, particularly when the airways are affected.

The patient in the present case was correctly diagnosed with RP during the initial episode of airway inflammation. Administration of prednisolone immediately resolved the airway symptoms and, 4 months later, restored the thickness of the cartilaginous parts of the airway to normal levels. However, the patient developed a severe obstructive ventilatory defect. Although the airway wall appeared normal on CT at that time, the supporting cartilage tissue was thought to have been extensively destroyed, leading to tracheobronchomalacia. RP patients with airway involvement have been reported to be at increased risk of relapse [4]. Recurrent flare‐ups in the respiratory tract can lead to fibrous development and, ultimately, fixed airway stenosis. Since it has been demonstrated that early administration of biologics has been effective in preventing severe airway involvement [5], administration of infliximab has been initiated for the present case. Although PFT showed a severe obstructive ventilatory defect in this case, non‐invasive positive pressure ventilation has not been used because the patient is living a normal daily life without respiratory support. The present case will need to be monitored extremely carefully.

Author Contributions

Ryusei Nakagawa and Tohru Sakamoto contributed to the conception and drafting of this manuscript. Ayaka Kishimoto, Tomohiro Namiki, Hiroya Sunabe, Toshihide Inui, Hiroaki Ishikawa, Hiroko Watanabe and Yuichi Dai critically reviewed and edited the manuscript. All authors read and approved the final version of the manuscript.

Ethics Statement

The authors declare that written informed consent was obtained for the publication of this manuscript and accompanying images using the form provided by the Journal.

Conflicts of Interest

The authors declare no conflicts of interest.

Nakagawa R., Kishimoto A., Namiki T., et al., “A Case of Relapsing Polychondritis With Severe Obstructive Ventilatory Defect Caused by a Single Flare‐Up,” Respirology Case Reports 13, no. 11 (2025): e70415, 10.1002/rcr2.70415.

Data Availability Statement

Data sharing is not applicable to this article, as no datasets were generated or analysed during the current study.