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Relapsing Polychondritis

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https://www.readbyqxmd.com/read/29145318/an-unusual-cause-of-fever-of-unknown-origin-with-enlarged-lymph-nodes-relapsing-polychondritis-a-case-report
#1
Wei Liu, Hongli Jiang, Han Jing, Bing Mao
INTRODUCTION: Fever of unknown origin (FUO) is a common initial presentation leading to a diagnostic challenge. PATIENT CONCERNS: A 3-month history of moderate-to-high fever was reported in an otherwise healthy 54-year-old man. Enhanced computed tomography (CT) scans of his chest showed a remarkable progressive enlargement of bilateral cervical, supraclavicular, hilar, and mediastinal lymph nodes within 2 weeks. Bronchofibroscopy manifested obvious luminal stenosis with swelling, thick pale mucosa, and disappearing of structures of trachea cricoid cartilage, followed by a 18F-fluorodeoxyglucose positron-emission tomography-computed tomography (18F-FDG PET/CT) with intense symmetric FDG uptake in larynx, tracheobronchial tree, and hilar, mediastinal, and axillary lymph nodes being demonstrated...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29135403/a-case-of-relapsing-polychondritis-mimicking-lung-cancer
#2
Fatma Canbay, Aydın Yılmaz, Ayperi Öztürk, Zafer Aktaş, Ebru Tatcı
Relapsing polychondritis (RP) is a rare autoimmune disease, characterized by episodic inflammatory attacks on cartilaginous tissue. Elastic cartilage of the ears and nose, hyaline cartilage of the peripheral joints, vertebral fibrocartilage, tracheobronchial cartilage, and tissues rich in proteoglycan such as those in the eyes, heart, blood vessels, and inner ear are more likely to be affected. A 35-year-old male presented with complaints of hoarseness, tinnitus and dyspnea for 19 years, with a history of several diagnostic and therapeutic interventions for laryngeal and respiratory tract...
September 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/29126262/relapsing-polychondritis-a-clinical-review-for-rheumatologists
#3
Jack Kingdon, Joseph Roscamp, Shirish Sangle, David D'Cruz
Relapsing polychondritis (RPC) is a rare autoimmune rheumatic disorder that is traditionally classified as a systemic vasculitis. It is characterized by inflammation of cartilage, and typical presenting features include chondritis of the nasal bridge, auricular chondritis, ocular inflammation and involvement of the bronchial tree. Its rarity often leads to considerable delay in establishing a diagnosis and poses a significant management challenge to clinicians, as no conventional guidelines exist. This review summarizes the clinical features of RPC and provides guidance for rheumatologists on making the diagnosis and assessing organ involvement...
November 6, 2017: Rheumatology
https://www.readbyqxmd.com/read/29069021/relapsing-polychondritis-in-a-liver-transplant-recipient-a-case-report
#4
Anna Mydlak, Dariusz Sołdacki, Bartosz Foroncewicz, Zygmunt Stopa, Agnieszka Powała, Tadeusz Budlewski, Leszek Pączek, Krzysztof Mucha
RATIONALE: Relapsing polychondritis (RP) is a multisystemic, progressive disease of unknown etiology characterized by recurrent inflammation and progressive cartilage destruction. It can involve all types of cartilage including ears and nose, tracheobronchial tree, joints, and any other tissue rich in proteoglycans such as heart, eyes, and blood vessels. Recurrent chondritis can be life-threatening if the respiratory tract, heart valves, or blood vessels are affected. To date there is no data in the literature on the post solid organ transplantation RP...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29048114/relapsing-polychondritis-as-a-presenting-feature-of-acute-myeloid-leukaemia
#5
Ya-Ting Hsu, Tsai-Yun Chen
No abstract text is available yet for this article.
October 19, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29021454/a-case-of-sudden-respiratory-failure-due-to-tracheobronchomalacia-by-relapsing-polychondritis-successfully-rescued-by-multiple-metallic-stenting-and-tracheostomy
#6
Daisuke Oryoji, Nobuyuki Ono, Daisuke Himeji, Kyoko Yoshihiro, Yasufumi Kai, Motohiro Matsuda, Hiroshi Tsukamoto, Akira Ueda
Relapsing polychondritis (RP) is a rare systemic autoimmune disease that affects cartilaginous structures. RP causes tracheobronchomalacia (TBM) by affecting the bronchial cartilage. TBM is a fatal condition characterized by excessive weakening of the walls of the trachea and bronchi. We herein report a case of a 73-year-old man who experienced sudden respiratory failure due to TBM caused by RP. Immunosuppressive treatment did not improve his respiratory failure. Multiple metallic stentings dramatically improved his severe airway symptoms...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29019876/unique-circumferential-peripheral-keratitis-in-relapsing-polychondritis-a-case-report
#7
Naohiro Motozawa, Takahiro Nakamura, Seiji Takagi, Masashi Fujihara, Yasuhiko Hirami, Kazuhiro Ishida, Chie Sotozono, Yasuo Kurimoto
RATIONALE: Relapsing polychondritis (RP) is a rare collagen disease characterized by inflammation and destruction of cartilage throughout the body. The paper details the clinical course of a case of RP with unique circumferential peripheral keratitis. PATIENT CONCERNS: A 54-year-old Japanese woman was referred to the hospital presenting with auricular and ocular pain. DIAGNOSES: Based on the auricle biopsy results and the three presenting symptoms (bilateral auricular chondritis, inflammatory arthritis and ocular inflammation), her condition was diagnosed as RP...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28994110/relapsing-polychondritis-an-underestimated-dermatological-urgency-case-report-and-literature-review
#8
Daniel Cuestas, Elkin Peñaranda, Sergio Mora, Carolina Cortes, Ingrid Galvis, Mónica Patiño, Oscar Velasquez
BACKGROUND: Relapsing polychondritis is an autoimmune multisystemic disease with primary chondral involvement. Its high mortality and morbidity make it a real clinical challenge. CASE DESCRIPTION: A 32-year-old woman with a history of relapsing polychondritis, refractory to multiple treatments, with multisystem compromise, imminent risk of death due to severe tracheobronchial damage and difficult ventilatory support, and successful treatment with infliximab. DISCUSSION AND EVALUATION: Several treatments have been described in the literature, such as nonsteroidal anti-inflammatory drugs, corticosteroids, dapsone, azathioprine, cyclosporine, cyclophosphamide, and methotrexate...
October 9, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28965131/efficacy-of-tocilizumab-highlighted-by-fdg-pet-ct-in-a-patient-with-relapsing-polychondritis-associated-aortitis
#9
REVIEW
Ghassan Elourimi, Michael Soussan, Ursula Warzocha, Hélène Bugaud, Robin Dhôte, Sébastien Abad
Relapsing polychondritis (RP) is a rare systemic inflammatory disease primarily affecting the ears, nose and tracheobronchial tree cartilage, but also the cardiovascular system. Cardiovascular complications are the second cause of mortality in RP. We report the case of a woman with a corticosteroid-resistant RP-associated aortitis, who was successfully treated with tocilizumab (TCZ). The FDG-PET/CT was a useful tool for diagnosing aortitis and assessing the effect of biotherapy. We conducted a systematic literature review confirming this is the first case of rapid and sustained remission in a patient with corticosteroid-resistant RP-associated aortitis after TCZ treatment administered as a first-line immunotherapy...
November 2017: Rheumatology International
https://www.readbyqxmd.com/read/28957548/cartilage-improvement-in-a-case-of-relapsing-polychondritis
#10
Hiroshi Oiwa, Kentaro Egusa
No abstract text is available yet for this article.
October 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28953580/relapsing-polychondritis-with-increased-bone-marrow-signal-on-magnetic-resonance-imaging-in-a-13-year-old-girl
#11
Kanako Nakayama, Masafumi Yamada, Yusuke Tozawa, Yuji Nakamaru, Masahiro Ueki, Shunichiro Takezaki, Hiroki Nishimura, Tomoko Mitsuhashi, Noriko Oyama-Manabe, Keita Sakamoto, Ryuta Arai, Sayaka Yamamoto, Kimiaki Uetake, Tadashi Ariga
Relapsing polychondritis (RPC) is an inflammatory disorder predominantly affecting cartilaginous structures including ears, nose, joints, and respiratory tracts. Although RPC is rare in children, previous reports indicated childhood RPC is atypical or severe. A 13-year-old girl presented with redness and swelling of the bilateral auricles and subsequently developed arthralgia of multiple joints. Histopathologic findings of the auricular cartilages were compatible with RPC. Magnetic resonance imaging (MRI) for the evaluation of arthralgia unexpectedly demonstrated increased bone marrow signal of metacarpals and the distal epiphysis of radii and ulnae...
September 26, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28940817/answer-to-shimizu-et%C3%A2-al-organ-involvement-pattern-suggests-subgroups-within-relapsing-polychondritis
#12
Jérémie Dion, Nathalie Costedoat-Chalumeau, Jean-Charles Piette
We read with interest the letter from Shimizu et al. regarding their large survey of 121 Japanese physicians following a total of 239 patients with relapsing polychondritis (RP). Using correlation matrix, they found that airway involvement (laryngo and tracheobronchial (TB)) was strongly associated with nasal chondritis whereas the relationship was inverse with external ear chondritis suggesting that the nasal and the auricular chondritis define two subgroups of RP patients. This article is protected by copyright...
September 21, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28890262/-relapsing-polychondritis-what-s-new-in-2017
#13
J Dion, G Leroux, L Mouthon, J-C Piette, N Costedoat-Chalumeau
Relapsing polychondritis (RP) is a rare condition characterized by recurrent inflammation of cartilaginous tissue and systemic manifestations. Data on pathophysiology are scarce and suggest an autoimmune mechanism. Recently, the possibility of dividing patients with RP into three distinct clinical phenotypes has been suggested: the hematological form representing less than 10% of patients, essentially older men with associated myelodysplasia and poor prognosis, the respiratory form representing about 25% of patients with predominant tracheobronchial involvement, and the mild and most frequent form, representing 65% of patients, with a good prognosis...
September 7, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28834348/clinical-images-relapsing-polychondritis-diagnosed-by-fusion-images-of-gallium-67-uptake-on-computed-tomography-and-single-photon-emission-computed-tomography
#14
Kazuhisa Nakano, Takafumi Aritomi, Naoaki Ohkubo, Yoshiya Tanaka
No abstract text is available yet for this article.
August 22, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28746194/18f-fdg-pet-ct-is-an-ideal-imaging-modality-for-the-early-diagnosis-of-relapsing-polychondritis-a-case-report
#15
JianJie Wang, XiaoFei Liu, Chaoyu Pu, Yan Chen
BACKGROUND: Relapsing polychondritis (RP) is a rare autoimmune disease of unknown etiology that may affect multiple cartilage throughout the body. CASE REPORT: We report on a middle-aged man presented with cough, chest tightness, and fever of unknown origin, 18F-fluorodeoxyglucose positron emission tomography/computed tomography (F-FDG PET/CT) was performed. And the imaging shows multiple increased FDG accumulation in tracheobronchial tree and all intercostal cartilages, as well as in nasal, right auricule, laryngeal cartilage...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28743361/relapsing-polychondritis-prevalence-of-cardiovascular-diseases-and-its-risk-factors-and-general-disease-features-according-to-gender
#16
Pablo Arturo Olivo Pallo, Maurício Levy-Neto, Rosa Maria Rodrigues Pereira, Samuel Katsuyuki Shinjo
The comorbidities in relapsing polychondritis have been scarcely described in the literature. Moreover, apart from a few relapsing polychondritis epidemiological studies, no studies specifically addressing relapsing polychondritis distribution according to gender are available. Therefore, the objectives of the present study were: (a) to analyze the prevalence of cardiovascular diseases and its risk factors in a series of patients with relapsing polychondritis; (b) to determine the influence of gender on relapsing polychondritis...
July 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28739564/silent-tracheobronchial-chondritis-in-a-patient-with-a-delayed-diagnosis-of-relapsing-polychondritis
#17
Faraz Afridi, Seema Frosh
Relapsing polychondritis is a very rare autoimmune disease characterised by a relapsing inflammation of hyaline, elastic and fibrous cartilaginous tissues. The incidence is estimated to be between 3.5 and 4.5 per million people per year. Clinical signs and symptoms can be very subtle, and if left undiagnosed for a prolonged period, airway involvement can cause fibrosis of the tracheobronchial wall, leading to a fixed tracheobronchial stenosis. Eventually, this can progress to life-threatening tracheobronchomalacia due to irreversible damage and loss of tissue integrity...
July 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28720307/diagnosis-of-relapsing-polychondritis-in-a-patient-with-chronic-cough-and-without-nasal-or-auricular-chondritis
#18
Luis Gorospe Sarasúa, Deisy Barrios-Barreto, Ismael Said-Criado, Carlos de la Puente-Bujidos
No abstract text is available yet for this article.
July 15, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28710538/-manifestations-of-autoimmune-disorders-in-otorhinolaryngology-classical-symptoms-and-diagnostic-approach
#19
B Hofauer, A Chaker, K Thürmel, A Knopf
Manifestations of autoimmune disorders represent a diagnostic challenge. Many autoimmune disorders show typical manifestations in the ear, nose and throat and should be taken into account in the differential diagnosis. Sjogren's syndrome, various vasculitides and sarcoidosis represent the most common autoimmune disorders in otorhinolaryngology, but IgG4-related diseases, relapsing polychondritis and autoimmune disorders of the mucosa are also observed. Autoimmune diseases can lead to sensorineural deficits...
August 2017: HNO
https://www.readbyqxmd.com/read/28705782/biologics-in-myelodysplastic-syndrome-related-systemic-inflammatory-and-autoimmune-diseases-french-multicenter-retrospective-study-of-29-patients
#20
REVIEW
Arsene Mekinian, Guillaume Dervin, Nathanael Lapidus, Jean-Emmanuel Kahn, Louis Terriou, Eric Liozon, Eric Grignano, Jean-Charles Piette, Odile Beyne Rauzy, Vincent Grobost, Pascal Godmer, Jerome Gillard, Julien Rossignol, David Launay, Achille Aouba, Thierry Cardon, Laurence Bouillet, Jonathan Broner, Julien Vinit, Lionel Ades, Fabrice Carrat, Clementine Salvado, Eric Toussirot, Mathilde Versini, Nathalie Costedoat-Chalumeau, Jean Baptiste Fraison, Philippe Guilpain, Pierre Fenaux, Olivier Fain
BACKGROUND: Systemic inflammatory and autoimmune diseases (SIADs) associated with myelodysplastic syndromes are often difficult to treat. Corticosteroids are efficient but only usually at high doses. The use of biologics needs to be specified. METHODS: In a French multicenter retrospective study, we analyzed the efficacy and safety of biologics (tumor necrosis factor-α [TNF-α] antagonists, tocilizumab, rituximab and anakinra) for SIADs associated with myelodysplastic syndromes (MDSs)...
September 2017: Autoimmunity Reviews
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