Relapsing polychondritis

Rapini, Ronald P.; Warner, Noranna B.

doi:10.1016/j.clindermatol.2006.07.018

Cited by 98 publications

(89 citation statements)

References 11 publications

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“…The literature has references on the use of pulse therapy with methylprednisolone and/or cyclophosphamide, as well as the use of immunosuppressants, such as methotrexate, azathioprine, and mycophenolate mofetil, with different results, in cases refractory to oral corticosteroids. 11 Besides, the successful treatment of refractory RP and cases with involvement of the respiratory tract with Tumor Necrosis Factor α -TNF-α -antagonists have been reported.…”

Section: Discussionmentioning

confidence: 99%

Uso de infliximabe na policondrite recidivante

Barros¹,

Nakamura²,

Santana³

et al. 2010

Rev. Bras. Reumatol.

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Relapsing Polychondritis (RP) is a rare systemic disease of unknown etiology, characterized by recurrent inflammation of cartilaginous structures and other connective tissues, including the ears, nose, joints, respiratory tract, and others. Due to the presence of typical signs and symptoms, biopsy is seldom necessary. Treatment includes corticosteroids, occasionally associated with immunosuppressive agents, but refractory cases are described. Recent reports suggest that anti-TNF agents, such as infliximab, may be of value in patients who do not respond to conventional therapy, but experience with this treatment is scarce. In this paper, the authors report the case of a patient with RP refractory to combined treatment with corticosteroids and immunosuppressive agents, who showed a good response to infliximab.

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Section: Discussionmentioning

confidence: 99%

Uso de infliximabe na policondrite recidivante

Barros¹,

Nakamura²,

Santana³

et al. 2010

Rev. Bras. Reumatol.

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“…Relapsing polychondritis (RP) is a rare connective tissue disease characterized by inflammation and destruction of cartilaginous tissue 1 . Although the exact underlying pathologic mechanism is unknown, it is widely accepted that there is a role of an autoimmune response in most cases 2 .…”

Section: To the Editormentioning

confidence: 99%

Combination of Hypertrophic Pachymeningitis, PR3-ANCA-positive Vasculitis, and Relapsing Polychondritis

Cañas¹,

Díaz-Martínez²,

Tobón³

2011

J Rheumatol

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“…Auricular chondritis is the most common initial presentation [2], and the prevalence of airway involvement is about…”

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confidence: 99%

“…Auricular chondritis is the most common initial presentation [2], and the prevalence of airway involvement is about [3]. Tracheal collapse is reported to be from 14 to 42% [4].…”

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confidence: 99%

A saddle nose with acute respiratory failure

Lin

Liang

2011

Intern Emerg Med

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A 17-year-old youth came to the Emergency Department (ED) with stridor in March, 2009. Commencing 6 months prior, he had developed bilateral progressive hearing loss along with frequent vertigo. Both auricles appeared erythematous and swollen over the prior 2 months. Intermittent dyspnoea with episodic stridor and easy choking had developed. The neck soft tissue X-ray study showed a narrowing of the airway (Fig. 1). The nasal fiberscopic examination revealed bilateral vocal paralysis with oedematous swelling of the arytenoids and false vocal cords. Left external ear destruction (Fig. 2a), right ear induration ( Fig. 2b) and a saddle nose deformity ( Fig. 2c) were noted. Because of the acute respiratory stress and stridor, the patient was immediately treated with endotracheal intubation in the ED. Due to soft tissue swelling, the intubation could only be performed with 6.5 French endotracheal tube. A computed tomography (CT scan) of the neck showed circumferential thickening of the tracheal wall along with tracheal lumen narrowing of the subglottic trachea (Fig. 3). The patient then underwent tracheostomy, and was successfully weaned from mechanical ventilation. The biopsy of the trachea showed acute and chronic inflammatory infiltrates surrounding the elastic cartilage with foci of perichondrial erosion and chondrocyte destruction. Biopsy of the ear revealed acutely inflamed granulation tissue with patchy organising microhemorrhage.A diagnosis of relapsing polychondritis was established by the combination of clinical typical findings (McAdam's criteria) [1], imaging studies, and biopsies of the trachea and ear.Relapsing polychondritis is a rare multisystem inflammatory process that may potentially compromise the structural and function of all of the cartilage in the body, especially the ears, nose, eyes, joints, and respiratory tract. Auricular chondritis is the most common initial presentation [2], and the prevalence of airway involvement is about

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Relapsing polychondritis

Cited by 98 publications

References 11 publications

Uso de infliximabe na policondrite recidivante

Uso de infliximabe na policondrite recidivante

Combination of Hypertrophic Pachymeningitis, PR3-ANCA-positive Vasculitis, and Relapsing Polychondritis

A saddle nose with acute respiratory failure

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