1,788 results match your criteria Polychondritis

Meningoencephalitis in relapsing polychondritis: A case report.

Medicine (Baltimore) 2021 Jun;100(24):e26315

Department of Neurology, Fukushima Medical University School of Medicine, Fukushima.

Rationale: Aseptic meningoencephalitis is a rare central nervous system complication of relapsing polychondritis (RP).

Patient: We report a 61-year-old Japanese male patient with spiking fever and impaired consciousness. Neurological examination revealed meningealirritation, and cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis with elevated protein (199 mg/dL) and interleukin-6 (3810 pg/mL). Read More

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Subglottic Mass as Isolated Manifestation of Relapsing Polychondritis at Presentation.

Ear Nose Throat J 2021 Jun 3:1455613211016718. Epub 2021 Jun 3.

Unit of Otorhinolaryngology, University Luigi Vanvitelli, Napoli, Italy.

Relapsing polychondritis (RP) is a rare systemic disease that is characterized by recurrent episodic inflammation of the cartilaginous structures of the body, resulting in their progressive destruction and subsequent replacement with fibrotic scar. We present a case of RP that initially manifested with subglottic involvement and we propose an innovative strategy for the treatment for laryngeal RP in phase of active inflammation. A multidisciplinary approach (rheumatologist, otolaryngologist, immunologist, internist, cardiologist, etc) and adequate follow-up are essential. Read More

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Nodular Pachymeningitis Associated With Relapsing Polychondritis and Crohn Disease Responsive to Adalimumab and Prednisone.

Neurol Neuroimmunol Neuroinflamm 2021 Jul 2;8(5). Epub 2021 Jun 2.

From the Division of Neuroimmunology and Neuroinfectious Diseases (S.K.H., N.V.), Department of Neurology, Massachusetts General Hospital, Boston; Division of Neuroradiology (M.D.M), Department of Radiology, Massachusetts General Hospital, Boston; and Division of Rheumatology, Allergy and Immunology (E.M.M), Department of Medicine, Massachusetts General Hospital, Boston.

Objectives: To review the previous literature on the associations of pachymeningitis with Crohn disease (CD) and relapsing polychondritis (RP) and to describe a new case occurring in association with both in addition to highlighting its positive response to steroid and adalimumab treatment.

Methods: We review the patient's clinical presentation, diagnostic workup (serum and CSF testing), and MRI findings in detail and chronicle the response of the pachymeningitis to intensive immunotherapy. We contrast this case against previous reports of pachymeningitis occurring in association with RP and inflammatory bowel disease that were found on PubMed. Read More

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Somatic Mutation in and ANCA-associated Vasculitis.

J Rheumatol 2021 Jun 1. Epub 2021 Jun 1.

Vasculitis Clinic, Canadian Network for Research on Vasculitides, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, University of Montreal; Division of Immunology and Allergy, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, University of Montreal, Montreal, Quebec, Canada. The authors declare no conflicts of interest. Address correspondence to Dr. J.P. Makhzoum, Vasculitis Clinic, Division of Internal Medicine, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, University of Montreal, 5400 Gouin O Blvd., Montreal, QC H4J 1C5, Canada. Email:

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was recently discovered in 25 men with late-onset severe and refractory inflammatory syndromes and associated hematologic abnormalities. Various diseases have been described, such as relapsing polychondritis, myelodysplastic syndrome, polyarteritis nodosa, and giant cell arteritis. Read More

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Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: a Chinese retrospective cohort study.

Orphanet J Rare Dis 2021 May 17;16(1):225. Epub 2021 May 17.

Rheumatology Department, The First Affiliated Hospital of Zhengzhou University, E1 Jianshe Road, Zhengzhou, 450052, Henan, China.

Background: Relapsing polychondritis (RPC) is a rare autoimmune disease and its early diagnosis remains challenging. Defining the clinical patterns and disease course may help early recognition of RPC.

Results: Sixty-six males and 60 females were included in this study. Read More

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Pediatric-onset refractory relapsing polychondritis with respiratory tract involvement.

Pediatr Neonatol 2021 Apr 27. Epub 2021 Apr 27.

Department of Pediatrics, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan; Department of Medical Research, National Taiwan University Hospital, Taiwan; Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan. Electronic address:

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Efficacy of Tocilizumab for Refractory Relapsing Polychondritis with Tracheal Stenosis and Respiratory Distress.

Hsien-Tzung Liao

Rheumatology (Oxford) 2021 May 14. Epub 2021 May 14.

Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Taipei Veterans General Hospital, Taipei, Taiwan.

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The VEXAS Syndrome: Uncontrolled Inflammation and Macrocytic Anaemia in a 77-Year-Old Male Patient.

Eur J Case Rep Intern Med 2021 13;8(4):002484. Epub 2021 Apr 13.

Rheumatology, Hirslanden Klinik St. Anna, Lucerne, Switzerland.

The VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described X-linked autoinflammatory condition caused by a somatic mutation of the UBA1 gene and characterized by an evolving phenotype. This includes inflammatory processes such as recurrent fever, Sweet's syndrome of the skin, pulmonary fibrosis, relapsing polychondritis and venous thromboembolism. An important feature, present in almost all cases, is the development of a macrocytic anaemia with vacuolization of myeloid and erythroid precursors. Read More

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Pathogenic UBA1 variants define a subset of relapsing polychondritis.

Jessica McHugh

Nat Rev Rheumatol 2021 Jun;17(6):312

Nature Reviews Rheumatology, .

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Differential diagnosis of relapsing polychondritis from asthma by 18-fluoro-2-deoxyglucose positron emission tomography and computed tomography.

Clin Case Rep 2021 Apr 12;9(4):2475-2476. Epub 2021 Feb 12.

Department of Respiratory Medicine Tokyo Medical University Ibaraki Medical Center Inashiki Japan.

About a half of all patients with relapsing polychondritis show airway involvement, which is a major cause of morbidity and mortality from this disease. FDG-PET/CT is useful in the differential diagnosis of relapsing polychondritis from asthma. Read More

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Application of extracorporeal membrane oxygenation to treatment of serious tracheal stenosis caused by relapsing polychondritis: a case report and literature review.

J Int Med Res 2021 Apr;49(4):3000605211009489

Department of Critical Care Medicine, the First Affiliated Hospital, 12377Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.

Relapsing polychondritis (RP) is a multisystemic rheumatic disease characterized by widespread and potentially destructive inflammatory lesions of the cartilage. The rarity of this disease and the lack of pathological diagnostic laboratory tests can occasionally lead to delayed diagnosis. We herein describe a 51-year-old woman with RP. Read More

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Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis.

Ann Otol Rhinol Laryngol 2021 Apr 3:34894211005979. Epub 2021 Apr 3.

Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University, Baltimore, MD, USA.

Objectives: To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation.

Methods: Case report and literature review.

Results: A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Read More

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Pathogenic variants associated with VEXAS syndrome in Japanese patients with relapsing polychondritis.

Ann Rheum Dis 2021 Mar 31. Epub 2021 Mar 31.

Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.

Objectives: To determine clinical and genetic features of individuals with relapsing polychondritis (RP) likely caused by pathogenic somatic variants in ubiquitin-like modifier activating enzyme 1 ().

Methods: Fourteen patients with RP who met the Damiani and Levine criteria were recruited (12 men, 2 women; median onset age (IQR) 72.1 years (67. Read More

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Effects of the COVID-19 Pandemic on Patients Living With Vasculitis.

ACR Open Rheumatol 2021 Jan 8;3(1):17-24. Epub 2020 Dec 8.

University of Pennsylvania, Philadelphia.

Objective: This study aimed to analyze the concerns and health-related behaviors in patients with vasculitis during the early phase of the coronavirus disease 2019 (COVID-19) pandemic in North America.

Methods: Patients with vasculitis in North America were invited to complete an online survey through the Vasculitis Patient-Powered Research Network in collaboration with the Vasculitis Foundation and the Relapsing Polychondritis Foundation. Questions focused on concerns and behaviors related to doctors' visits, tests, medication, and telehealth use. Read More

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January 2021

Somatic Mutations in UBA1 Define a Distinct Subset of Relapsing Polychondritis Patients with VEXAS Syndrome.

Arthritis Rheumatol 2021 Mar 28. Epub 2021 Mar 28.

Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, MD, USA.

Objective: Somatic mutations in ubiquitin activating enzyme 1 (UBA1) cause a newly defined syndrome known as VEXAS. More than fifty percent of patients currently identified with VEXAS meet diagnostic criteria for relapsing polychondritis (RP). Clinical features that characterize VEXAS within a cohort of RP have not been defined. Read More

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[Autoimmune Encephalitis/Meningitis].

Aki Sato

No Shinkei Geka 2021 Mar;49(2):401-406

Department of Neurology, Niigata City General Hospital.

Less than half of the cases of autoimmune encephalitis have brain MRI abnormalities; however, some patterns of MRI findings help diagnosis. Usually, DWI and FLAIR images reveal hyperintensity lesions in the cortical or subcortical regions or the cerebellum and/or the brainstem. Hyperintensity lesions in the limbic cortex on DWI suggest NMDAR encephalitis. Read More

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Comparison of relapsing polychondritis patients with and without central nervous system involvement: A retrospective study of 181 patients.

Int J Immunopathol Pharmacol 2021 Jan-Dec;35:20587384211000547

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Graduate School of Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.

The relapsing polychondritis (RP) patients with central nervous system (CNS) involvement were rare. We aimed to determine the clinical characteristics of RP patients with CNS involvement. The clinical data of 181 RP patients, hospitalized at Peking Union Medical College Hospital between December 2005 and February 2019, were collected. Read More

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[Autoimmunity and autoinflammation in pathogenesis of immunoinflammatory diseases].

Ter Arkh 2020 Dec 15;92(12):120-125. Epub 2020 Dec 15.

Yaroslavl State Medical University.

Rheumatic diseases relate to the group of the immunoinflammatory diseases (IID), in pathogenesis of which have a value both autoimmune and autoinflammatory processes.

Aim: To present the heterogeneous pathogenesis of inflammation in IID.

Materials And Methods: It is inspected 260 patients (pts) with IID: 242 pts with systemic autoimmune diseases (SAD): 65 systemic lupus erythematosis, 50 systemic sclerosis, 127 systemic vasculitides (SV) and 18 patients with autoinflammatory diseases (AID): 8 Behcets disease, 2 periodic disease, 5 familial cold fever, 2 idiopathic lobular panniculitis and 1 relapsing polychondritis. Read More

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December 2020

Relapsing polychondritis: an inflammatory disorder not to be missed.

Clin Exp Optom 2021 Mar 2:1-2. Epub 2021 Mar 2.

Optometry Department, McAllen Veterans Affairs Community Based Outpatient Clinic, McAllen, TX, USA.

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Natural closing of a tracheal tear caused by intubation in a patient with relapsing polychondritis.

Respirol Case Rep 2021 Apr 22;9(4):e00721. Epub 2021 Feb 22.

Division of Respiratory Diseases, Department of Internal Medicine St. Marianna University School of Medicine Kawasaki Japan.

Relapsing polychondritis (RP) often develops into severe tracheobronchial stenosis with malacia. Although tracheal tears rarely occur by intubation, treatment decisions for tears can be difficult due to airway inflammation in RP patients. In this case, due to advanced age and immunosuppressive treatment, we decided against invasive surgery. Read More

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Relapsing polychondritis associated with heart block.

Eur Rev Med Pharmacol Sci 2021 Feb;25(4):2050-2055

Institute for Health Sciences from Federal University of Bahia, Salvador, Bahia, Brazil.

Objective: The present article aims at describing a rare case of an RP patient who evolved with heart block and was successfully treated with corticoid pulse therapy, without the need for pacemaker insertion.

Patients And Methods: A systematic research on relapsing polychondritis (RP) and heart block (HB) published in PubMed/MEDLINE, Web of Sciences, LILACS, and Scielo from 1966 to August 2020 was performed.

Results: It was found 10 studies on RP associated with HB, and we added a case. Read More

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February 2021

Respiratory subtype of relapsing polychondritis frequently presents as difficult asthma: a descriptive study of respiratory involvement in relapsing polychondritis with 13 patients from a single UK centre.

ERJ Open Res 2021 Jan 15;7(1). Epub 2021 Feb 15.

Louise Coote Lupus Unit, Guy's Hospital, London, UK.

Introduction: Relapsing polychondritis is a rare multisystem vasculitis characterised by recurrent cartilage inflammation. Respiratory involvement, of which tracheobronchomalacia (TBM) is the commonest form, is difficult to treat and is linked to increased mortality. We describe 13 patients with respiratory involvement. Read More

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January 2021

Hospital admissions in inflammatory rheumatic diseases during the peak of COVID-19 pandemic: incidence and role of disease-modifying agents.

Ther Adv Musculoskelet Dis 2021 4;13:1759720X20962692. Epub 2021 Feb 4.

Rheumatology Department and IDISSC, Hospital Clínico San Carlos, Madrid, Spain.

Aims: In this pandemic, it is essential for rheumatologists and patients to know the relationship between COVID-19 and inflammatory rheumatic diseases (IRDs). We wanted to assess the role of targeted synthetic or biologic disease-modifying antirheumatic drugs (ts/bDMARDs) and other variables in the development of moderate-severe COVID-19 disease in IRD.

Methods: An observational longitudinal study was conducted during the epidemic peak in Madrid (1 March to 15 April 2020). Read More

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February 2021

Physician Global Assessment as a Disease Activity Measure for Relapsing Polychondritis.

Arthritis Care Res (Hoboken) 2021 Feb 5. Epub 2021 Feb 5.

Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD, USA.

Objective: Relapsing polychondritis (RP) is a systemic inflammatory disorder of cartilage that lacks validated disease activity measures. Physician global assessment (PhGA), a measure of disease activity commonly used in rheumatologic diseases, has not been tested in a cohort of patients with RP.

Methods: Adult patients in an observational cohort of RP underwent standardized, comprehensive evaluation at approximately 6-month intervals. Read More

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February 2021

Relapsing polychondritis associated with pauci-immune crescentic glomerulonephritis.

Lancet 2020 10;396(10259):e63

Division of Nephrology, Department of Medicine, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Bangkok, Thailand.

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October 2020

Relapsing polychondritis: an unusual cause of sore throat, painful ear swelling and bilateral costal margin pain.

BMJ Case Rep 2021 Feb 4;14(2). Epub 2021 Feb 4.

Rheumatology, Sun Yat-sen University First Affiliated Hospital, Guangzhou, Guangdong, China.

A 54-year-old Chinese woman presented with a 3-month history of sore throat and dry cough, which was treated as chronic pharyngitis with minimal improvement. One month ago, she presented with painful right ear swelling without signs or symptoms of otitis media or otitis externa. She was treated with antibiotics and antiviral drugs without any improvement. Read More

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February 2021