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Relapsing Polychondritis

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https://www.readbyqxmd.com/read/29283700/relapsing-polychondritis-is-a-lung-disease-my-story
#1
Marcela A Ferrada
Relapsing polychondritis is a rare autoimmune disease that can be fatal. This systemic condition with a predilection for cartilage can inflame the trachea, the distal airways, ear and nose, blood vessels, eyes, kidneys, and brain. If relapsing polychondritis is not diagnosed early and treated effectively, there can be irreversible damage to the cartilage that can lead to tracheal or bronchial collapse, blindness, and deafness. I am a physician and a clinical investigator. I have severe relapsing polychondritis with prominent involvement of my airways...
December 28, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29280824/a-case-of-relapsing-polychondritis-mimicking-hemophagocytic-lymphohistiocytosis-after-propionibacterium-acnes-infection
#2
William E Monaco, Carey J Field, Thomas H Taylor
We describe a 69-year-old man who presented with a right sternocleidomastoid intramuscular abscess that grew Propionibacterium acnes. Despite initial improvement with antibiotics, he subsequently developed signs and symptoms fulfilling diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH)-fevers, cytopenias, hyperferritinemia, splenomegaly, and low natural killer cell activity. The patient improved spontaneously but at outpatient follow-up complained of erythema over the bridge of his nose as well as bilateral ear redness sparing the earlobes at which point relapsing polychondritis (RP) was diagnosed...
December 27, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29245187/news-on-relapsing-polychondritis-the-patients-experience
#3
EDITORIAL
Jean-Charles Piette, Jérémie Dion, Nathalie Costedoat-Chalumeau
In this issue of Arthritis Care & Research, the authors of Patient-Perception of Disease-Related Symptoms and Complications in Relapsing Polychondritis report for the first time the patient's view over the disease (1). Thanks go to them for this important step forward. The method used however, carries some limitations, as indicated very honestly by the authors. This article is protected by copyright. All rights reserved.
December 15, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/29245173/patient-perception-of-disease-related-symptoms-and-complications-in-relapsing-polychondritis
#4
Marcela A Ferrada, Peter C Grayson, Shubhasree Banerjee, Keith Sikora, Robert Colbert, Ninet Sinaii, James D Katz
OBJECTIVE: To assess patient-reported symptoms and burden of disease in relapsing polychondritis (RP). METHODS: Patients with RP completed a disease-specific online survey to identify symptoms attributed to illness. Patients were divided into subgroups based upon presence or absence of ear/nose, airway, or joint involvement. Pathway to diagnosis, treatment, and disease-related complications were assessed within each subgroup. RESULTS: Data from 304 respondents were included in this analysis...
December 15, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/29234846/fdg-pet-ct-as-a-powerful-tool-for-diagnosing-and-monitoring-treatment-outcomes-of-relapsing-polychondritis
#5
Pauline Baudart, Achille Aouba, Marie Beaufrère, Nicolas Aide
No abstract text is available yet for this article.
December 12, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29218258/breaking-the-magic-mouth-and-genital-ulcers-with-inflamed-cartilage-syndrome
#6
REVIEW
Stella Pak, Shaina Logemann, Christine Dee, Adam Fershko
Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome refers to a condition in which features of Behcet's disease (BD) and relapsing polychondritis (RP) occur in the same individual. The existence of MAGIC syndrome suggests a potential common etiology for BD and RP. However, connecting these two diseases and referring to this condition as MAGIC syndrome might have been premature, as there is currently insufficient knowledge on BD and RP. In this critical review, we argue that these two clinical entities could possibly be unique disease processes rather than two ends of the same disease spectrum...
October 4, 2017: Curēus
https://www.readbyqxmd.com/read/29200026/severe-aortic-valve-regurgitation-in-relapsing-polychondritis
#7
Francesca Wanda Rossi, Valentina Mercurio, Felice Rivellese, Antonio Lobasso, Letizia Barbieri, Carlo Gabriele Tocchetti, Amato de Paulis, Gianni Marone
No abstract text is available yet for this article.
December 1, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29189373/incidental-case-of-relapsing-polychondritis-detected-by-18f-fdg-pet-ct
#8
Hayato Kaida, Kazunari Ishii, Soichiro Hanada, Yuji Tohda, Takamichi Murakami
A 78-year-old man had fever, persistent wheezing, and serum C-reactive protein elevation. Malignant lymphoma was suspected because of mediastinal lymph nodes swelling on CT and soluble interleukin 2 receptor elevation. Symmetric F-FDG uptake in the tracheobronchial tree and bilateral auricles was observed on PET/CT. He was finally diagnosed as having relapsing polychondritis by auricular cartilage biopsy. F-FDG PET/CT may have crucial role in evaluating the extent of inflammation and deciding the biopsy site of relapsing polychondritis...
November 17, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29187087/heart-transplantation-as-last-resort-treatment-for-relapsing-polychondritis-with-severe-cardiac-involvement
#9
Laurent Dormoy, Annette Belin, Fabien Labombarda
Relapsing polychondritis is a rare multisystem autoimmune disease characterized by recurrent inflammation and destruction of different cartilaginous and proteoglycan-rich structures like cardiovascular system. We reported a case of heart transplantation in a 20-year-old man having a relapsing polychondritis with severe multiple cardiovascular involvement. The immunosuppressive treatment used to prevent graft rejection allowed to turn out the inflammatory disease. Cardiac transplantation may be considered as last resort treatment option in relapsing polychondritis with intractable extensive heart lesions...
September 2017: Progress in Transplantation
https://www.readbyqxmd.com/read/29173700/relapsing-polychondritis-and-lymphocytic-meningitis-with-varied-neurological-symptoms
#10
Renata Dal-Prá Ducci, Francisco Manoel Branco Germiniani, Letícia Elizabeth Augustin Czecko, Eduardo S Paiva, Hélio Afonso Ghizoni Teive
No abstract text is available yet for this article.
November 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/29145318/an-unusual-cause-of-fever-of-unknown-origin-with-enlarged-lymph-nodes-relapsing-polychondritis-a-case-report
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
Hiroshi Oiwa, Kentaro Egusa
No abstract text is available yet for this article.
October 1, 2017: Rheumatology
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