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Conditions of the External Ear
Published in R James A England, Eamon Shamil, Rajeev Mathew, Manohar Bance, Pavol Surda, Jemy Jose, Omar Hilmi, Adam J Donne, Scott-Brown's Essential Otorhinolaryngology, 2022
Ayeshah Abdul-Hamid, Samuel MacKeith
The presentation is usually with dull pain increasing in severity, and there is inflammation of the cartilaginous pinna. Relapsing polychondritis is an autoimmune condition, and it may present similarly but is differentiated on the basis of the systemic nature of the condition, which may also affect joints, the eye, and cartilage of the nose and airway. Perichondritis is treated with prompt broad-spectrum antibiotics with anti-pseudomonal cover, ideally intravenously. If there is a subperichondrial abscess it should be drained. If perichondritis is untreated, it could result in avascular necrosis of the cartilage and deformity of the pinna.
Autologous Stem Cell Transplantation in Relapsing Polychondritis
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Falk Hiepe, Andreas Thiel, Oliver Rosen, Gero Massenkeil, Gerd-Rüdiger Burmester, Andreas Radbruch, Renate Arnold
Relapsing polychondritis is a rare multisystem autoimmune disorder of unknown etiology that was first described by Jaksch-Wartenhorst in 1923.1 It is an episodic and progressive inflammatory disease of the cartilaginous structures, including the elastic cartilage of the ear and nose, the hyaline cartilage of peripheral joints, the fibrocartilage at axial sites, and cartilaginous structures of the tracheobronchial tree. Inflammation of other proteoglycan-rich structures such as the eyes, heart, blood vessels, inner ear, and kidneys may also occur. Since relapsing polychondritis was first described, more than 300 cases of the disease have been reported. The diagnosis is based on the clinical findings and may be confirmed histologically by biopsy of the affected ear. The disease is treated by corticosteroids and immunsuppressive agents. Here, we report on a woman suffering from refractory relapsing polychondritis whom we successfully treated by autologous stem cell transplantation (ASCT), resulting in a long-term remission which has lasted for 55 months now. We, therefore, conclude that ASCT may be of therapeutic benefit to patients with relapsing polychondritis who failed to respond to conventional therapy. Eligibility criteria for ASCT in relapsing polychondritis are discussed.
Cardiology
Published in Fazal-I-Akbar Danish, Essential Lists of Differential Diagnoses for MRCP with diagnostic hints, 2017
Aortic dissection – predisposing conditions:1 Essential HTN (in patients of Afro-Caribbean origin); secondary HTN (d/t coarctation of aorta).2 Marfan’s syndrome.3 Ehlers–Danlos’ syndrome.4 Pregnancy.5 Relapsing polychondritis.
Reconstructive rhinoplasty using cadaver cartilage in relapsing polychondritis
Published in Baylor University Medical Center Proceedings, 2023
Rishabh Shah, Eugene L. Alford
Relapsing polychondritis is a rare autoimmune disorder that primarily affects cartilaginous structures of the upper airways. It is characterized by recurrent episodes of inflammation leading to progressive anatomical deformation with cosmetic and functional impairment of the auricular cartilage, nasal cartilage, and respiratory tract.1 Patients may experience nasal stuffiness, rhinorrhea, and epistaxis. The bridge of the nose and surrounding tissue become erythematous, edematous, and tender and may collapse, producing a saddle nose deformity. The pathologic basis of the saddle nose is represented by a substantial loss of the dorsal height along with middle vault depression, internal nasal valve insufficiency, columellar retraction, loss of tip support, shortened vertical length, and overrotated tip.2 Nasal reconstruction can be challenging due to impaired wound healing and impaired graft integration that is caused both by the disease and by prolonged immunosuppressant treatment.3 Several types of reconstruction techniques have been used in the correction of saddle nose deformity, including autologous rib cartilage, fascia lata, and calvarial bone grafts. Costal and calvarial bone were the most used graft materials in an analysis done by Ezzat et al in 2017.4 In this case report, we present a patient with a history of relapsing polychondritis in whom cadaver cartilage was used for reconstruction.
Sustained Remission with Tocilizumab in Refractory Relapsing Polychondritis with Ocular Involvement: A Case Series
Published in Ocular Immunology and Inflammation, 2021
Rebecca Farhat, Gaël Clavel, Delphine Villeneuve, Youssef Abdelmassih, Marwan Sahyoun, Eric Gabison, Thomas Sené, Isabelle Cochereau, Cherif Titah
Relapsing polychondritis is a rare multisystemic disease and to date, no guidelines for the management have been validated. Therapy remains largely empirical and based on case reports.26–9 It is selected based on the clinical presentation and consists of non-steroidal anti-inflammatory drugs for mild manifestations, colchicine or dapsone, whereas more severe cases are treated with systemic corticosteroids. In life-threatening disease or when corticosteroid sparing is not possible, immunosuppressant agents such as cyclophosphamide, methotrexate, and azathioprine are indicated.5 In recent years, the arrival of new biologic agents widened the treatment options and changed the management of patients with RP resistant to classical immunosuppressive treatment.10
Tocilizumab in Recalcitrant Bilateral Scleritis in a Case of Relapsing Polychondritis: A 17-year Follow Up
Published in Ocular Immunology and Inflammation, 2023
Mariya Doctor, Somasheila I. Murthy, Liza Rajasekhar
Hence, it is also important to keep in mind that about 18%11 patients with relapsing polychondritis can have ocular manifestations as the presenting feature, with scleritis being the most common. The diagnosis of relapsing polychondritis becomes very difficult due to the rarity of the disease, overlapping clinical features with other autoimmune conditions, and absence of any specific investigation. Scleritis in these patients is usually resistant to first-line therapy of systemic steroids and immunomodulators, and hence early initiation of treatment with newer biologics such as Tocilizumab (IL-6 R blockers) would help reduce the disease severity and achieve complete remission.