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Relapsing polychondritis: What's new in 2017?

[Relapsing polychondritis: What's new in 2017?] Rev Med Interne. 2017 Sep 07;: Authors: Dion J, Leroux G, Mouthon L, Piette JC, Costedoat-Chalumeau N Abstract 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. Recent data on survival shows an improvement of overall prognosis compared to historical series. Reported poor prognosis factors are male gender, associated haemopathies and cardiac involvement. Few recent series suggest an interest for positron emission tomography for the diagnosis and the follow-up of treatment. Due to the lack of randomized therapeutic trial, treatment remains empirical and is mainly based on oral corticosteroids sometimes associated with immunosuppressive agents. The use of biologic agents has recently been reported in small retrospective series with different outcome. Finally, some selected patients with mild and occasional periphe...
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research

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No abstract available
Source: JCR: Journal of Clinical Rheumatology - Category: Rheumatology Tags: Letters to the Editor Source Type: research
In January, 2018, Academic Press published my bookPrecision Medicine and the Reinvention of Human Disease. This book has an excellent " look inside " at itsGoogle book site, which includes the Table of Contents. In addition, I thought it might be helpful to see the topics listed in the Book's index. Note that page numbers followed by f indicate figures, t indicate tables, and ge indicate glossary terms.AAbandonware, 270, 310geAb initio, 34, 48ge, 108geABL (abelson leukemia) gene, 28, 58ge, 95 –97Absidia corymbifera, 218Acanthameoba, 213Acanthosis nigricans, 144geAchondroplasia, 74, 143ge, 354geAcne, 54ge, 1...
Source: Specified Life - Category: Information Technology Tags: index jules berman jules j berman precision medicine Source Type: blogs
No abstract available
Source: JCR: Journal of Clinical Rheumatology - Category: Rheumatology Tags: Letters to the Editor Source Type: research
(MedPage Today) -- Lack of ear symptoms or joint involvement hampered diagnosis
Source: MedPage Today Primary Care - Category: Primary Care Source Type: news
Abstract ObjectiveTo assess patient‐reported symptoms and burden of disease in relapsing polychondritis (RP). MethodsPatients 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. ResultsData from 304 respondents were included in this analysis. Prior to diagnosis, most patients with RP went to the emergency room (54%), saw>3 physicians (54%), and had symptoms for&...
Source: Arthritis Care and Research - Category: Rheumatology Authors: Tags: Original Article Source Type: research
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Source: Arthritis Care and Research - Category: Rheumatology Authors: Tags: Editorial Source Type: research
Abstract 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 18F-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. 18F-FDG PET/CT may have crucial role in evaluating the extent of inflammation and deciding the biopsy site of relapsing polychondritis.
Source: Clinical Nuclear Medicine - Category: Nuclear Medicine Tags: Interesting Images Source Type: research
AbstractRatioinale: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.Patient concerns:A 26-year-old woman complained abo...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Source: Arthritis and Rheumatism - Category: Rheumatology Authors: Tags: Letters Source Type: research
Source: Arthritis and Rheumatism - Category: Rheumatology Authors: Tags: Clinical Images Source Type: research
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