Journal of Otology & RhinologyISSN: 2324-8785

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Case Report, J Otol Rhinol S Vol: 0 Issue: 1

A Case of Relapsing Polychondritis diagnosed by Biopsy of Thyroid Cartilage

Daisuke Sasaki, Junkichi Yokoyama*, Shinichi Ooba, Mitsuhisa Fujimaki, Masataka Kozima and Katsuhisa Ikeda
Department of Otorhinolaryngology, Head and Neck Surgery, Juntendo University Faculty of Medicine, Tokyo, Japan
Corresponding author : Junkichi Yokoyama, MD, PhD
Department of Otolaryngology-Head and Neck Surgery, Juntendo University Faculty of Medicine, Hongo 3-1-3, Bunkyo-ku, Tokyo 113-8431, Japan
Tel: 81-3-3813-3111; Fax: 81-3-5840-7103
E-mail: [email protected]
Received: November 18, 2014 Accepted: March 11, 2014 Published: March 17, 2015
Citation: Sasaki D, Yokoyama J, Ooba S, Fujimaki M, Kozima M, et al. (2015) A case of Relapsing Polychondritis diagnosed by Biopsy of Thyroid Cartilage. J Otol Rhinol S1:1. doi:10.4172/2324-8785.S1-017

Abstract

Relapsing polychondritis is a rare autoimmune disease characterized by recurrent inflammation and destruction of cartilage throughout the body. Prior to a definitive diagnosis, our patient suffered from a sore throat, hoarseness and persistent cough over a period of 1 month. Bone scintigraphy showed high integration of costochondral regions and thyroid cartilage. CT (computed tomography) demonstrated a hypodense area surrounded by enhancement around the thyroid cartilage. Due to respiratory dysfunction associated with general anesthesia, a diagnostic biopsy was delayed for 2 weeks. Therefore, the patient was referred to Otolaryngology Head and Neck Surgery and underwent biopsy of the thyroid cartilage under local anesthesia. The thyroid cartilage was inflamed and surrounded with aseptic abscesses. The patient was diagnosed as having relapsing polychondritis. The patient was administered a course of oral steroids. Most of the general symptoms including high fever and dysphasia disappeared. The early diagnosis based on the thyroid cartilage biopsy under local anesthesia enabled prevention of significant airway obstruction that would otherwise have been caused by thyroid cartilage inflammation. This is the first reported case of relapsing polychondritis diagnosed by biopsy of the thyroid cartilage.

Keywords: Biopsy; Early diagnosis; Local anesthesia; Relapsing polychondritis; Thyroid cartilage

Keywords

Biopsy; Early diagnosis; Local anesthesia; Relapsing polychondritis; Thyroid cartilage

Introduction

Relapsing polychondritis is a rare autoimmune disease characterized by recurrent inflammation and eventual destruction of cartilage throughout body. Frequently affected areas include various sites of cartilage such as the external ears, nose, larynx, upper air way, tracheobronchial tree, eyes, and large joints [1]. The mortality rate for relapsing polychondritis is reported to be approximately 20-30% [2]. The cause of death is related to respiratory failure in more than half of all reported cases [1].
Generally, relapsing polychondritis is diagnosed through the identification of several symptoms and biopsy of the cartilage [1,3]. The area typically utilized for biopsy is the external ear. However, in our patient we could not identify chondritis in the external ears. The risk of respiratory dysfunction precluded biopsy under general anesthesia. Consequently, the patient underwent biopsy of the thyroid cartilage while under local anesthesia. This is the first reported case of relapsing polychondritis diagnosed by biopsy of the thyroid cartilage.

Case Report

A 63-year-old man with hypertension, hyperlipidemia, diabetes and a previous history of myocardial infarction was referred to our hospital. The patient indicated that he had been suffering from a persistent sore throat, hoarseness and coughs over a period of 1 month. Prior to being referred to our hospital the patient had been treated with antibiotics. However, even high doses of antibiotics could not decrease the inflammation or cure the symptoms. Moreover, as time passed the disease progressed and the patient described experiencing frontal chest pain. It was at this point that the patient was referred to our department for consultation.
The results of a blood culture were negative and no autoimmune antibodies were detected. Laryngoscopy showed normal function and movement of the vocal fold and airways. Echocardiography indicated normal heart function. In addition, upper gastrointestinal endoscopy and lower gastrointestinal endoscopy indicated normal function.
Chest CT with contrast medium demonstrated a hypo-dense area with ring enhancements present in costochondral parts (Figure 1A) and a thickening of the tracheal wall (Figure 1B). Neck CT with contrast medium demonstrated a hypo-dense area surrounded by enhancement around thyroid cartilage (Figure 2). Bone scintigraphy showed high integration of costochondral parts, thyroid cartilage and nasal cartilage (Figure 3).
Figure 1: (A) Chest CT demonstrated a hypo-dense area surrounded by ring enhancement around the costochondralregion (Arrow); (B) Chest CT with contrast medium showed a thickening of the tracheal wall (Arrow).
Figure 2: Neck CT with contrast medium showed a hypo-dense area surrounded by enhancement around the thyroid cartilage (Arrow).
Figure 3: Bone scintigraphy showed high integration of costochondral parts, thyroid cartilage and nasal cartilage..
On the basis of these radiologic findings, a diagnosis of relapsing polychondritis was suggested and the patient underwent biopsy of the thyroid cartilage. By external incision of the thyroid cartilage, drainage of surrounding abscesses was performed (Figure 4A). Although swelling that surrounded the thyroid cartilage was observed, the outer perichondrium was intact. The space between the inner and outer perichondria contained pus and pieces of necrotic cartilage. The pus was drained and the necrotic tissue was removed.
Figure 4: (A) Operative findings: External incision of the thyroid cartilage and drainage of the surrounding abscesses (Arrow); (B) Pathological findings (Hematoxilin-Eosin satin): Acute and chronic inflammatory cells infiltrating the thyroid cartilage and chondrocytes resulted in drop-out and necrosis; (C) Pathological findings (Elastica van Gieson stain): There was no pathological findings indicating any vasculitis as, observed in Wegener’s glanulomatosis.
A drainage tube was left in the thyroid cartilage. The culture of pus indicated that it was aseptic. The biopsy revealed acute and chronic inflammation of the perichondrium and cartilage with patchy necrosis and destruction of the cartilage as well as mild chronic inflammation. These findings were consistent with the histologic changes of relapsing polychondritis (Figure 4B and C). In addition, autoimmune antibodies of collagen type II were detected by a blood examination.
As a result, the patient was diagnosed as having relapsing polychondritis. The patient was administered a course of oral steroids. On the fourth day of administration, the patient indicated that he was no longer experiencing a sore throat, hoarseness, cough or frontal chest pain. The neck wound from the incision had also improved.
The patient’s progress has been followed for approximately 1 year with no evidence of recurrence.

Discussion

Although the cause of relapsing polychondritis remains unclear, an immune-mediated mechanism has been postulated. Among the supporting evidence is the finding that antibodies associated with specific collagen types are present in some patients with relapsing polychondritis [4]. In particular, antibodies associated with type II collagen appear to be associated with relapsing polychondritis [4]. Relapsing polychondritis may develop at any age, but most cases are diagnosed in the fourth to fifth decades of life, predominantly among whites, with an equal sex distribution [1].
The most frequent clinical features of relapsing polychondritis are recurrent pain and swelling of the external ear and nose, uveitis, and arthropathy of the peripheral joints. The most common feature in relapsing polychondritis is auricular chondritis, which affects 80%-90% of such patients [1]. However, in our case the patient did not have auricular chondritis. Involvement of the ear results in pinna deformity, and involvement of the nose results in a classic “saddle” deformity of the nasal bridge.
The respiratory tract is involved in approximately one half of such patients [5]. Clinical symptoms include dyspnea, cough, wheezing, stridor, and dysphonia. Approximately 14% of patients have respiratory symptoms at the initial presentation [1]. However, this subset of patients appears to have a more fulminant course of disease that is less responsive to steroid treatment [6]. In general, respiratory involvement portends a worse prognosis because of deaths related to respiratory failure in more than one half of reported cases [1]. Fortunately, in our case the patient didn’t have subglottic stenosis or tracheobronchial stenosis and there was no deformation of his thyroid cartilage or cricoid cartilage. The patient didn’t require any airway intervention.
A primary complaint of relapsing polychondritis is general symptoms. A high index of suspicion is necessary because accurate diagnosis can be easily missed or delayed. Early airway intervention should be considered when the cricoid cartilage is involved or in cases with significant airway obstruction. Early treatment is required to control symptoms and to prevent complications related to treatments. The mainstay of treatment for relapsing polychondritis is systemic corticosteroid therapy. Prednisone (20-60 mg/d) is administered in the acute phase and is tapered to 5-25 mg/d for maintenance. Therefore, it is very important to determine a precise diagnosis before the administration of corticosteroid.
However, in our patient, we could not identify chondritis of the external ears. The risk of respiratory dysfunction prevented biopsy under general anesthesia. Accordingly, the biopsy for the definitive diagnosis was postponed. Consequently, the patient underwent biopsy of the thyroid cartilage while under local anesthesia. The patient was soon diagnosed as having relapsing polychondritis. The patient was administered a course of oral steroids. As soon as the steroids were administered, the patient ceased to experience sore throat, hoarseness, cough and frontal chest pain. The patient’s status has been followed for approximately 1 year with no evidence of recurrence or complication. It is important to make an early diagnosis by biopsy in order to commence early treatment. This is the first report of relapsing polychondritis diagnosed by biopsy of the thyroid cartilage.

Conclusion

Early diagnosis of polychondritis by biopsy from the thyroid cartilage while under local anesthesia is important in order to prevent significant airway obstruction caused by airway cartilage inflammation.

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