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Guillaume Moulis, Grégory Pugnet, Nathalie Costedoat-Chalumeau, Alexis Mathian, Gaëlle Leroux, Jonathan Boutémy, Olivier Espitia, Laurence Bouillet, Sabine Berthier, Jean-Baptiste Gaultier, Pierre-Yves Jeandel, Amadou Konaté, Arsène Mékinan, Elisabeth Solau-Gervais, Benjamin Terrier, Daniel Wendling, Fanny Andry, Camille Garnier, Pascal Cathébras, Laurent Arnaud, Aurore Palmaro, Patrice Cacoub, Zahir Amoura, Jean-Charles Piette, Philippe Arlet, Maryse Lapeyre-Mestre, Laurent Sailler
OBJECTIVES: To assess the efficacy and the safety of biologics in a cohort of patients with relapsing polychondritis (RP). METHODS: We conducted a French multicentre retrospective cohort study including patients treated with biologics for RP. Efficacy outcomes were clinical response (partial or complete) and complete response during the first 6 months of exposure, plus daily corticosteroid dose at 6 months. Other outcomes were adverse drug reactions (ADRs), persistence of biologics and factors associated with a response...
March 13, 2018: Annals of the Rheumatic Diseases
Yuan He, Huifeng Liu, Zhi Ji, Binliang Zhu
Purpose: To report a case of surgical interventions for a patient with relapsing polychondritis who presented with late ocular complications. Observations: A 44-year-old male was diagnosed to have relapsing polychondritis on the basis of recurrent acute auricular chondritis, deformity of the ear, saddle nose deformity and painful nasal chondritis, acute ocular inflammation with conjunctivitis, episcleritis and keratouveitis, laryngotracheal chondritis, erythema nodosum in the skin, a history of polyarthritis, and abnormal blood examination findings...
April 2017: American Journal of Ophthalmology Case Reports
Yi-Yi Xuan, Tian-Fang Li, Lei Zhang, Sheng-Yun Liu
RATIOINALE: Relapsing polychondritis (RP) is a rare and heterogeneous disease complex of unknown origin which basically affects cartilaginous structures, 40% of which accompanied by rheumatic, hematologic, and endocrine disease. Among them, vasculitis is the most common accompanying type and usually presented with positive antineutrophilic cytoplasmic antibody (ANCA). The presence of ANCA could be primary or drug-induced like propylthiouracil (PTU). Central involvement of RP is very rare, and there is almost no report of cerebral vasculopathy manifested as moyamoya...
December 2017: Medicine (Baltimore)
A-G Lelong, S Mallet, C Le Treut, J-C Dubus, A Carsin, E Bosdure
The frequency of body piercing has increased in France over the past few years, particularly among teenagers. Piercing can be performed at different sites on the body, especially in the cartilage of the ears. We relate two cases of Pseudomonas aeruginosa chondritis. A 10-year retrospective study at the Marseille University Hospital found no additional pediatric cases. These infectious complications can sometimes be necrotizing. It is therefore important to inform the medical staff on the hygiene measures that need to be respected and the patients on the treatment to follow after the piercing as well as the signs to watch for, possibly indicating chondritis...
December 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
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
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)
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)
Jérémie Dion, Nathalie Costedoat-Chalumeau, Jean-Charles Piette
No abstract text is available yet for this article.
January 2018: Arthritis & Rheumatology
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
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
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
Nathalie Droin, Nolwenn Lucas, Vincent Parinet, Dorothée Selimoglu-Buet, Marc Humbert, Véronique Saada, Olivier Lambotte, Eric Solary, Nicolas Noël
Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic disorder that associates dysplastic and proliferative features. Tissue inflammatory disorders occur in a fraction of CMML patients during the course of their disease. Here, we describe the occurrence of eosinophil-rich tissue inflammation, including eosinophilic pneumonia, chondritis, and cystitis, in CMML patients. Whole exome sequencing of leukemic cells did not identify a recurrent genetic abnormality among these three patients who were clinically improved by local or oral corticosteroids...
December 2017: Leukemia & Lymphoma
Kazuo Kubota, Hiroyuki Yamashita, Akio Mimori
FDG is a tracer for visualizing glucose metabolism. PET/CT using FDG is widely used for the diagnosis of cancer, because glycolysis is elevated in cancer cells. Similarly, active inflammatory tissue also exhibits elevated glucose metabolism because of glycolysis in activated macrophages and proliferating fibroblasts. Elevated FDG uptake by active inflammatory tissues, such as those affected by arthritis, vasculitis, lymphadenitis, and chondritis, has enabled the diagnosis of inflammatory diseases using FDG-PET/CT...
July 2017: Seminars in Nuclear Medicine
Franca Kraenzlin, Oren Mushin, Shahin Ayazi, John Loree, Derek E Bell
Auricular burns represent a unique type of injury. The acute management and clinical course of these injuries can be different from other facial burns. There is a paucity of literature pertaining to the epidemiology and acute management of auricular burns. Most studies focus on deformity reconstruction. The aim of this study was to characterize the epidemiology, treatment, and outcomes of auricular burns. Data from all patients presenting to a regional burn center in a 4-year period were reviewed and those with auricular burns were identified...
May 23, 2017: Journal of Burn Care & Research: Official Publication of the American Burn Association
Shintaro Hayashi, Norihiko Akao, Koichi Okamoto
A 77-year-old man showed an asymptomatic meningeal lesion beneath the dura matter in the left fronto-parietal region on MRI during an examination for recurrent hoarsness. The lesion showed no gadolinium enhancement, and extended to neither the sulci nor skull. Neurological examinations revealed hoarseness, cochlear and vestibular dysfunction of the right ear, and mildly decreased Achilles tendon reflexes bilaterally. Laboratory findings showed marked inflammatory responses, but no abnormalities for LDH, IgG4, angiotensin-converting enzyme, or soluble IL-2 receptor...
June 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
Do-Youn Kim, Sung Hoon Nam, Sami E Alharethy, Yong Ju Jang
Improtance: Correction of caudal septal deviation with a batten graft has been popularized recently. However, few reports have documented the surgical outcomes of this technique, especially the use of bony batten grafts in septoplasty. Objective: To evaluate the surgical outcomes of bony batten grafting for the management of caudal septal deviation in endonasal septoplasty. Design, Setting, and Participants: This retrospective cohort study evaluates the medical records of 141 patients with caudal septal deviation who underwent septoplasty using bony batten grafts from September 1, 2011, through February 29, 2016, at a tertiary referral hospital...
December 1, 2017: JAMA Facial Plastic Surgery
Yasuhiro Suyama, Shin-Ichi Ishimoto, Kiyofumi Hagiwara
No abstract text is available yet for this article.
June 2017: Arthritis & Rheumatology
Benjamin Terrier, Agnès Dechartres, Christophe Deligny, Pascal Godmer, Pierre Charles, Gilles Hayem, Bertrand Dunogué, Michel de Bandt, Pascal Cohen, Xavier Puéchal, Claire Le Jeunne, Serge Arfi, Luc Mouthon, Loïc Guillevin
Objectives.: Granulomatosis with polyangiitis (GPA) mainly affects white Europeans, but rarely GPA may also affect non-Europeans. This study aimed to describe GPA clinical-biological presentation and outcome in black sub-Saharan Africans and Afro-Caribbeans and in North Africans. Methods.: Among 914 GPA patients included in the French Vasculitis Study Group database, geographic origin and ethnicity were known for 760. Clinical-biological presentations and outcomes of white Europeans vs black sub-Saharans and Afro-Caribbeans and vs North Africans were analysed...
March 1, 2017: Rheumatology
Satoru Ushiyama, Tomomi Kinoshita, Yasuhiro Shimojima, Nobuhiko Ohashi, Dai Kishida, Daigo Miyazaki, Katsuya Nakamura, Yoshiki Sekijima, Shu-Ichi Ikeda
Neurological involvement in relapsing polychondritis (RP) is relatively rare. We describe the case of an 80-year-old man who presented with hypertrophic pachymeningitis (HP) together with arthritis as the first manifestation of RP. Auricular chondritis, which subsequently determined the diagnosis of RP, occurred a few weeks after the detection of HP. The neurological symptoms, as well as arthritis, were promptly improved by treatment with corticosteroids. It is generally difficult to diagnose RP in the absence of typical cartilaginous involvement; however, the present case suggests that HP may occur as an early clinical manifestation of RP...
September 2016: Case Reports in Neurology
Hakan Emmungil, Sibel Zehra Aydın
Relapsing polychondritis (RPC) is a unique and rarely observed autoimmune condition regarded as recurrent extensive chondritis of the auricular, nasal, and tracheal cartilages. Moreover, heart, main arteries, skin, and eyes may be involved. Several forms of clinical manifestations may be seen, and the pathogenesis still remains anonymous. A concomitant disease, particularly myelodysplasia or other systemic autoimmune disease can be detected in one-third of the patients with RPC. The treatment of RPC should be considered on personal basis and classified according to disease activity and severity...
December 2015: European Journal of Rheumatology
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