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[18F]fluorodeoxyglucose
positron emission tomography imaging in a case of relapsing
polychondritis.J Comput Assist Tomogr.2007 May-Jun;31(3):381-3.
Relapsing
polychondritis is a rare multisystemic disease that is characterized
by recurrent inflammation of the cartilaginous structures of the
external ear, nose, joint, larynx, and tracheobronchial tree. Airway
involvement is present in up to 50% of patients with the disease and
is a major cause of morbidity and mortality. We describe a patient
with relapsing polychondritis presenting with tracheal and bronchial
abnormalities that were identified by an increased uptake on
[18F]fluorodeoxyglucose positron emission tomography.
Relapsing
polychondritis.Clin Dermatol. 2006 Nov-Dec;24(6):482-5.
Relapsing
polychondritis is a rare disease most commonly presenting as
inflammation of the cartilage of the ears and nose. Auricular
chondritis, with red ears resembling infectious cellulitis, is the
most common initial finding. Antibodies to type II collagen in
cartilage are found, and the earlobes are classically spared.
Chronic disease may result in a flabby, droopy ear, cauliflower ear,
or saddle nose deformity. Acute involvement of the tracheal
cartilage may cause collapse of the airway with obstruction and
pulmonary infections. Arthritis may be oligoarticular or
polyarticular, most often involving the costochondral junctions.
Other manifestations include audiovestibular damage; heart valve
disease; and neurologic, ocular, and renal disease. Corticosteroids
remain the major treatment. Other therapies include nonsteroidal
anti-inflammatory drugs, dapsone, colchicine, azathioprine,
methotrexate, cyclophosphamide, hydroxychloroquine, cyclosporine,
and infliximab.
Otologic
manifestations of relapsing polychondritis. Review of literature and
report of nine cases. Auris Nasus Larynx.2006 Jun;33(2):135-41. Epub
2006 Jan 20.
OBJECTIVE:
Relapsing polychondritis (RP) is an episodic disease most likely of
autoimmune etiology, characterized by recurrent inflammation of
cartilaginous structures. METHODS: Retrospective case study at two
tertiary referral centers with presentation of nine patients with
otologic involvement of RP, review of the spectrum of otologic
disorders seen, and treatment. RESULTS: The clinical course of
otologic manifestations of RP was highly variable and ranged from
mild to moderate. In 6/9 patients there was an association with
other autoimmune disorders. In addition to recurrent auricular
chondritis, which was present in 8/9 patients, our patients had
otitis externa, chronic myringitis, Eustachian tube dysfunction,
conductive hearing loss, sensorineural hearing loss, and tinnitus.
All patients had their diagnosis of RP made on the basis of their
otologic involvement and the response to systemic corticosteroids.
CONCLUSION: The diagnosis of RP is primarily clinical, but
laboratory studies and biopsy may contribute as well. Once the
diagnosis is suspected, the otolaryngologist should consider
consultation with a rheumatologist to assist in the management of
additional systemic manifestations.
Relapsing
polychondritis -- a case report and review of the literature.Laryngorhinootologie.
2005 May;84(5):352-6.
PATIENT: A
case of a 54-year-old woman with a three month history of recurrent
bilateral chondritis of the auricles, cochlear and vestibular inner
ear damage and conjunctivitis is described. The diagnosis was based
only on clinical criteria (auricular chondritis, conjunctivitis,
inner ear damage). Antinuclear antibodies, ANCA and HLA-DR 4 antigen
were negative. The only laboratory abnormality was an elevated
erythrocyte sedimentation rate. The patient has been treated
successfully with Methotrexate 20 mg 1 x /week and Prednisone 15
mg/die for 4 month now. DISCUSSION: The relapsing polychondritis
(RP) is a rare, multisystemic and potentially fatal disease. The
pathogenesis and optimal therapeutic approach is poorly understood.
The disease is characterized by episodic inflammation of cartilage
such as auricular, nasal and laryngotracheal. Many other
proteoglycan-rich structures like inner ear, eye, kidney and blood
vessels, may be involved as well. RP has an equal sex prevalence.
The majority of cases appear between 40 and 60 years. Auricular
inflammation is the most common feature. Effectiveness of
non-steroidal anti-inflammatory drugs, dapsone, immunosuppressive
drugs and prednisone has been described. The overall survival rates
were 74 % at 5 years and 55 % at 10 in one 1986 series. CONCLUSION:
The most common clinical presentation of RP regularly involves ENT-structures.
Therefore ENT-specialists should be familiar with this disease. A
close interdisciplinary cooperation is essential for therapy and
follow-up, because pulmonary and cardiac involvement are limiting
prognostic factors.
Relapsing
polychondritis as a rare different diagnosis of erysipelas. J Dtsch
Dermatol Ges. 2004 Apr;2(4):286-9.
A 76-year
old patient with painful erythematous swelling of the right ear was
initially treated with antibiotics under the suspected diagnosis of
erysipelas. Her failure to respond and a history of previous
laryngeal and nasal swelling suggested the possibility of relapsing
polychondritis. High dose prednisolone therapy produced marked
improvement. Relapsing polychondritis should be considered as a
rare, but important differential diagnostic consideration for
erysipelas.
Relapsing
polychondritis--an Oriental case series. Singapore Med J. 2003
Apr;44(4):197-200.
INTRODUCTION:
Relapsing polychondritis (RPC) has been described mainly in
Caucasian populations. Reports from other ethnic groups are few.
OBJECTIVES: To describe the clinical characteristics, management and
outcome of RPC patients seen in an Oriental population in Singapore.
METHODS: The case records of RPC patients treated in our department
from 1989 to 2001 were reviewed. Only 12 fulfilled the
McAdam-Michet-Damiani-Levine diagnostic criteria and these were
studied. RESULTS: The female-to-male ratio in our series was 3:1.
There were 10 ethnic Chinese and two Malay patients. The age of
onset of symptoms ranges from three to 65 years, with a mean of 34
years. A diagnosis was made from two weeks to three years after
onset, with a median of 4.5 months. There were 10 patients with
pinna, nine articular, eight ocular, six laryngotracheal, five inner
ear, four nasal and one cardiac involvement. Five presented with
fever. None of them had cutaneous, renal or central nervous system
involvement. Ten had raised ESR at presentation. One patient
developed discoid lupus erythematosus two years later. All 12
patients received prednisolone with eight of them requiring
additional immunosuppressants. Two patients had resistant disease
failing to respond adequately to various immunosuppressants together
with prednisolone. There was no mortality amongst the nine patients
who had remained on follow-up at the time of this report. Five of
the six patients with laryngotracheal involvement had tracheostomy
and one of them had airway stenting as well. CONCLUSION: Our series
suggests that although the clinical manifestations of RPC are
similar in the Oriental and the Caucasian populations, Oriental
patients may have less cutaneous, renal or nervous system
involvement and more serious airway complications.
Relapsing
polychondritis: a clinical review.Semin Arthritis Rheum.2002
Jun;31(6):384-95.
OBJECTIVE:
This study comprehensively reviews the literature related to
relapsing polychondritis (RP). METHODS: A detailed search via
MEDLINE (PubMed) was performed using relapsing polychondritis as the
key term. Relevant articles were analyzed with a focus on history,
epidemiology, etiology, pathogenesis, clinical manifestations,
diagnosis, treatment, and prognosis of RP. RESULTS: RP is a rare
episodic and progressive inflammatory disease of presumed autoimmune
etiology first described in 1923. RP affects cartilage in multiple
organs, such as the ear, nose, larynx, trachea, bronchi, and joints.
In addition, it can affect proteoglycan-rich tissues, such as the
eyes, aorta, heart, and skin. The diagnosis of RP is based on the
presence of clinical criteria. A standardized therapeutic protocol
for RP has not been established. Nonsteroidal anti-inflammatory
drugs, dapsone and/or colchicine, may control disease activity in
some patients. In other patients, immunosuppressive drugs and
prednisone have been effective. RP is a potentially lethal disease;
pulmonary infection, systemic vasculitis, airway collapse, and renal
failure are the most common causes of death. Earlier studies
indicate survival rates between 70% at 4 years and 55% at 10 years.
In a recent study, a survival rate of 94% at 8 years may be due to
improved medical and surgical management. CONCLUSIONS: RP is a rare,
multisystemic, and potentially fatal disease. The pathogenesis and
optimal therapeutic approach to patients with RP is poorly
understood.
Relapsing
polychondritis revealed by ENT symptoms: clinical characteristics in
three patients.
Ann Otolaryngol Chir Cervicofac. 2002 Sep;119(4):202-8.
Relapsing
polychondritis (RP) is a recurrent, chronic and rare disease of
unknown etiology, considered as a systemic vasculitis. RP is
characterized by inflammation of cartilaginous structures of the
ears, nose, respiratory tract and joints. RP is likely initiated by
ENT symptoms. Etiology is unknown but the association with HLA-DR4
and the occurrence of antibodies to type-II collagen suggest that an
immunologic mechanism is involved in its pathogenesis. Diagnosis is
difficult requiring identification of elastic cartilaginous
injuries. Delay before diagnosis is usually important after the
first attack. Neither serum investigation nor histological
confirmation are necessary to establish the RP diagnosis, and ENT
symptoms are generally sufficient to achieve the diagnosis.
Prognosis is linked to laryngeal, tracheal and cardiovascular
involvements. An association with myelodysplasia is acknowledged.
Based on these three cases and data in the literature, we review
classical diagnostic criteria (McAdam), prognosis and therapeutic
outcome.
A case of
multisymptomatic relapsing polychondritis in a 22-year-old woman.
Acta Otorhinolaryngol Belg. 2001;55(3):227-33.
A case of
multisymptomatic relapsing polychondritis in a 22-year-old woman. We
report a case of a 22-year-old woman with relapsing polychondritis
(RP)--a rare and little known systemic autoimmune disease
characterised by episodic inflammation of cartilaginous structures
(ear, nose, bronchi, trachea, larynx, ribs, cardiovascular system).
This patient presents with a seven-year history, initiated by the
saddle nose. The patient developed a multitude of symptoms:
auricular chondritis, ocular symptoms, recurrent arthritis,
respiratory complications (laryngotracheomalacia, bilateral vocal
cord palsy), sensorineural hearing loss and enchondroma of the
humeral bone. The examination of an auricle biopsy by an
immunofluorescent method and a positive serum reaction from the
patient to normal cartilage supported the immunological nature of
relapsing polychondritis. Treatment consisted of orally administered
prednisone and diaminodiphenylsulfone (Dapsone).
Head
and neck manifestations of relapsing polychondritis: review of 29
cases.
Otolaryngology.1978 May-Jun;86(3 Pt 1):ORL473-8.
This review
of 29 patients with relapsing polychondritis seen at the Mayo Clinic
between 1962 and 1976 emphasizes the head and neck manifestations of
the disease and the role of the otolaryngologist in its diagnosis
and treatment. The major clinical features included inflammation of
the pinna, eye involvement, nasal cartilage involvement,
laryngotracheal involvement, arthropathy, hearing loss, costal
chondritis, and fever. The erythrocyte sedimentation rate was often
elevated, and mild anemia was not uncommon.
Relapsing
polychondritis: an ultrastructural study.Arthritis Rheum.1977
Jan-Feb;20(1):91-9.
Ear
cartilage from a typical case of relapsing polychondritis was
examined with the electron microscope. A large number of dense
granules and vesicles, which were compatible with matrix vesicles or
lysosomes, surrounded the affected chondrocytes. In less severely
damaged chondrocytes, these granules and vesicles appeared to be
formed by pinching off of the cytoplasmic processes or by budding
from the processes. Calcification of the granules was minimal. In
severely damaged chondrocytes, an admixture of these granules and
cytoplasmic organelles occurred. It is speculated that many of these
dense granules are lysosomal in nature and that they may produce
inflammation and reduce the proteoglycan content of cartilage.
Relapsing
polychondritis: prospective study of 23 patients and a review of the
literature. Medicine (Baltimore).1976 May;55(3):193-215.
Relapsing
polychondritis (RP) is not a totally rare rheumatic disease. We have
seen 23 patients from 1960-1975, and there are now a total of 159
reported cases, which form the basis of this study. RP occurs
equally in both sexes, and has a maximum frequency in the fourth
decade. 2) Empirically defined diagnostic criteria are proposed, to
include the most common clinical features: a) Bilateral auricular
chondritis b) Nonerosive sero-negative inflammatory polyarthritis c)
nasal chondritis d) Ocular inflammation e) Respiratory tract
chondritis f) Audiovestibular damage The diagnosis is based primarly
upon the unique clinical features, and is quite certain if three or
more criteria are present together with histologic confirmation. 3)
Fifty percent of patients present with either auricular chondritis
or the arthropathy of RP; but with prolonged follow-up, a majority
of patients develop four or more of the above mentioned criteria. 4)
Approximately 30 percent of patients have a preceding or coexistent
rheumatic or autoimmune disease, which can lead to initial
diagnostic confusion. 5) Laboratory and radiographic investigations
help mainly to rule out other diagnostic possibilities, with no
characteristic abnormalities being present in a majority of
patients. 6) On follow-up, three-fourths of our patients required
chronic corticosteroid therapy with an average dose of 25 mg per day
of prednisone. Corticosteroids decrease the frequency, duration, and
severity of flares, but do not stop disease progression in severe
cases. 7) The mortality rate has been 30 percent in our series and
22 percent in the other 136 reported cases. Of the 29 cases where
the cause of death was known, 17 were from respiratory tract
involvement and 9 from cardiac valvular or vasculitic involvement,
emphasizing the need to search for critical involvement of either of
these organ systems in each patient. 8) Detailed reports of selected
cases are presented to illustrate the clinical diagnosis and
differential diagnosis, and to demonstrate the need for careful
prolonged follow-up. 9) Although the etiology remains unknown, there
is a frequent association with, and clinical similarity to, other
rheumatic diseases. 10) Careful clinicopathological study of our 23
patients leads us to postulate an underying systemic vascultis as an
important pathologic mechanism in RP.
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