Journal of Otology & Rhinology

Sasaki et al., J Otol Rhinol 2015, S1:1
http://dx.doi.org/10.4172/2324-8785.S1-017
Journal of Otology &
Rhinology
Case Report
A SCITECHNOL JOURNAL
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: jyokoya@juntendo.ac.jp
Rec date: Nov 18, 2014 Acc date: Mar 11, 2014 Pub date: Mar 17, 2015
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.
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).
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.
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].
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).
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
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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:http://dx.doi.org/10.4172/2324-8785.S1-017
Figure 2: Neck CT with contrast medium showed a hypo-dense
area surrounded by enhancement around the thyroid cartilage
(Arrow).
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
Figure 3: Bone scintigraphy showed high integration of
costochondral parts, thyroid cartilage and nasal cartilage.
Volume S1 • Issue 1 • S1-017
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
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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:http://dx.doi.org/10.4172/2324-8785.S1-017
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
Volume S1 • Issue 1 • S1-017
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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