Search results for "Relapsing polychondriti"
showing 3 items of 3 documents
The Relapsing Polychondritis Disease Activity Index: Development of a disease activity score for relapsing polychondritis.
2012
Abstract Objective The rarity of relapsing polychondritis (RP) has hindered the development of standardized tools for clinical assessment. Here, we describe the development of a preliminary score for disease assessing activity in RP, the Relapsing Polychondritis Disease Activity Index (RPDAI). Methods Twenty-seven RP experts participated in an international collaboration. Selection and definition of items for disease activity were established by consensus during a 4-round internet-based Delphi survey. Twenty-six experts assessed the Physician's Global Assessment (PGA) of disease activity on 43 test cases on a 0–100 scale, yielding a total of 1118 PGA ratings. The weight of each item was est…
Laryngeal involvement in relapsing polychondritis: Case report and review of literature
2018
Relapsing polychondritis is a rare multisystem autoimmune disease of unknown origin characterised by recurrent episodes of inflammation and progressive destruction of the cartilaginous structures and connective tissue of the whole body. The diagnosis of relapsing polychondritis is difficult. We present a review of the literature and describe a case of 49-year old woman. Her symptoms began in June 2004 with sore throat, dysphonia, pain in the thoracic wall and some joints, a slightly raised temperature and cough. The objective picture was immediately apparent after carrying out a high definition neck-thorax computed tomography and a laryngoscopy with fiber optics, which showed considerable l…
Efficacy of Azacitidine (AZA) in Autoimmune and Inflammatory Disorders (AID) Associated with Myelodysplastic Syndromes (MDS) and Chronic Myelomonocyt…
2015
Abstract Background: AID are seen in 10-30% of MDS and CMML. After initial response to steroids, AID are often poorly controlled and steroid-sparing drugs are difficult to use due to the underlying MDS/CMML. Some case reports suggest a beneficial role of AZA treatment in AID associated to MDS/CMML. Methods: We retrospectively analyzed 22 MDS/CMML patients (pts) with AID who received AZA in French centers between January 2007 and May 2014. Results: Median age of the 22 pts was 70y (range 41-84), including 6F/16M. Diagnosis of MDS/CMML preceded AID (n=8) by a median of 17 months (mo), was concomitant with (n=7) or followed AID (n=7) by a median of 20 mo. 14 pts had lower risk IPSS and 8 highe…