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...
ConclusionAutoimmune diseases were present in 26% of patients. One-third of patients with anterior necrotizing scleritis or posterior scleritis experienced decreased vision, mostly due to secondary glaucoma and optic neuritis. Therefore, controlling intraocular pressure by methods such as administration of steroid-sparing immunosuppressive medication and appropriate treatment for posterior scleritis are essential for scleritis treatment.
International Journal of Dermatology, EarlyView.
Clinical and Experimental Dermatology, EarlyView.
Journal Name: Drug Metabolism and Personalized Therapy Issue: Ahead of print
New England Journal of Medicine,Volume 378, Issue 18, Page 1715-1715, May 2018.
PMID: 29760256 [PubMed - in process]
Journal Name: Drug Metabolism and Personalized Therapy Volume: 33 Issue: 2 Pages: 105-108
Relapsing polychondritis (RP) is an uncommon immune-related disease with unknown causes. It is characterized by inflammation of cartilaginous or non-cartilaginous structures, such as the ears, nose, respirator...
Annals of the American Thoracic Society,Volume 15, Issue 3, Page 290-292, March 2018.