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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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Authors: Héron E, Bourcier T Abstract Episcleritis is generally a benign disease with a self-limited course, while scleritis is a severe ocular condition due to a risk of impaired vision in one-fifth of patients and its association with systemic diseases in one third of them. Infectious scleritis, representing 8 % of the etiologies, is mainly of herpetic origin (varicella zoster and herpes simplex viruses). A systemic autoimmune disease is observed in roughly 30 % of scleritis patients: inflammatory rheumatisms (15 %), firstly rheumatoid polyarthritis, systemic vasculitides (8 %), mainly ...
Source: Journal Francais d Ophtalmologie - Category: Opthalmology Tags: J Fr Ophtalmol Source Type: research
ABSTRACT 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. A cross-sectional tertiary single center study evaluating 30 relapsing polychondritis ...
Source: Revista Brasileira de Reumatologia - Category: Rheumatology Source Type: research
Authors: Cao Y, Zhang W, Wu J, Zhang H, Zhou H Abstract Peripheral ulcerative keratitis (PUK) is type of crescent-shaped inflammatory damage that occurs in the limbal region of the cornea. PUK is always combined with an epithelial defect and the destruction of the peripheral corneal stroma. PUK may have a connection to systemic conditions, such as long-standing rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Wegener granulomatosis (WG), relapsing polychondritis, classic polyarteritis nodosa and its variants, microscopic polyangiitis, and Churg-Strauss syndrome. However, the most common connection is ...
Source: Journal of Ophthalmology - Category: Opthalmology Tags: J Ophthalmol Source Type: research
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Source: Arthritis and Rheumatism - Category: Rheumatology Authors: Tags: Clinical Image Source Type: research
Authors: Hofauer B, Chaker A, Thürmel K, Knopf A Abstract 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. Both hypo- and hyperthyroidism can be cau...
Source: HNO - Category: ENT & OMF Tags: HNO Source Type: research
Conclusion This nationwide study demonstrates the efficacy of steroids for SIAD-associated MDSs but a high frequency of steroid dependence. The response to biologics seems low, but rituximab and azacytidine seem promising.
Source: Autoimmunity Reviews - Category: Allergy & Immunology Source Type: research
CONCLUSIONS: VPS along with corticosteroids and immunosuppressants represents an effective treatment approach for patients who suffer from hydrocephalus associated with autoimmune diseases. PMID: 28686532 [PubMed - as supplied by publisher]
Source: Autoimmunity - Category: Allergy & Immunology Tags: Autoimmunity Source Type: research
Authors: Liu HL, Yang CD PMID: 28668816 [PubMed - in process]
Source: Journal of Rheumatology - Category: Rheumatology Tags: J Rheumatol Source Type: research
Conclusion: Our case demonstrates that FDG PET/CT is an useful diagnostic tool to accurately determine the extent of inflammation throughout the body and to guiding the selection of a biopsy site.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
PMID: 28558922 [PubMed - as supplied by publisher]
Source: Archivos de Bronconeumologia - Category: Respiratory Medicine Authors: Tags: Arch Bronconeumol Source Type: research
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