Jérémie Dion1, Nathalie Costedoat-Chalumeau2, Damien Sène3, Judith Cohen-Bittan4, Gaëlle Leroux5, Charlotte Dion6, Camille Francès7, Jean-Charles Piette5. 1. Université René Descartes Paris V, AP-HP, and Centre de référence maladies auto-immunes et systémiques rares, Hôpital Cochin, AP-HP, Paris, France. 2. Nathalie Costedoat-Chalumeau, MD, PhD: Université René Descartes Paris V, AP-HP, Centre de référence maladies auto-immunes et systémiques rares, Hôpital Cochin, AP-HP, and INSERM U1153, Center for Epidemiology and Statistics Sorbonne Paris Cité, Paris, France. 3. Université Paris-Diderot Paris VII, AP-HP, and Hôpital Lariboisière, Paris, France. 4. Université Pierre et Marie Curie Paris VI, AP-HP, and Hôpital Pitié Salpêtrière, Paris, France. 5. Université Pierre et Marie Curie Paris VI, AP-HP, and Centre de référence maladies auto-immunes et systémiques rares, Hôpital Pitié Salpêtrière, Paris, France. 6. INRA-INSERM, UR 1303, Ivry, France. 7. Université Pierre et Marie Curie Paris VI, AP-HP, and Hôpital Tenon, Paris, France.
Abstract
OBJECTIVE: Relapsing polychondritis (RP) is a rare condition characterized by recurrent inflammation of cartilaginous tissue and systemic manifestations. Data on this disease remain scarce. This study was undertaken to describe patient characteristics and disease evolution, identify prognostic factors, and define different clinical phenotypes of RP. METHODS: We performed a retrospective study of 142 patients with RP who were seen between 2000 and 2012 in a single center. RESULTS: Of the 142 patients, 86 (61%) were women. The mean ± SD age at first symptoms was 43.5 ± 15 years. Patients had the following chondritis types: auricular (89%; n = 127), nasal (63%; n = 89), laryngeal (43%; n = 61), tracheobronchial (22%; n = 32), and/orcostochondritis (40%; n = 57). The main other manifestations were articular (69%; n = 98), ophthalmologic (56%; n = 80), audiovestibular (34%; n = 48), cardiac (27%; n = 38), and cutaneous (28%; n = 40). At a mean ± SD followup of 13 ± 9 years, the 5- and 10-year survival rates were 95 ± 2% and 91 ± 3%, respectively. Factors associated with death on multivariable analysis were male sex (P = 0.01), cardiac abnormalities (P = 0.03), and concomitant myelodysplastic syndrome (MDS) (P = 0.004) or another hematologic malignancy (P = 0.01). Cluster analysis revealed that separating patients into 3 groups was clinically relevant, thereby separating patients with associated MDS, those with tracheobronchial involvement, and those without the 2 features in terms of clinical characteristics, therapeutic management, and prognosis. CONCLUSION: This large series of patients with definite RP revealed an improvement in survival as compared with previous studies. Factors associated with death were male sex, cardiac involvement, and concomitant hematologic malignancy. We identified 3 distinct phenotypes.
OBJECTIVE: Relapsing polychondritis (RP) is a rare condition characterized by recurrent inflammation of cartilaginous tissue and systemic manifestations. Data on this disease remain scarce. This study was undertaken to describe patient characteristics and disease evolution, identify prognostic factors, and define different clinical phenotypes of RP. METHODS: We performed a retrospective study of 142 patients with RP who were seen between 2000 and 2012 in a single center. RESULTS: Of the 142 patients, 86 (61%) were women. The mean ± SD age at first symptoms was 43.5 ± 15 years. Patients had the following chondritis types: auricular (89%; n = 127), nasal (63%; n = 89), laryngeal (43%; n = 61), tracheobronchial (22%; n = 32), and/orcostochondritis (40%; n = 57). The main other manifestations were articular (69%; n = 98), ophthalmologic (56%; n = 80), audiovestibular (34%; n = 48), cardiac (27%; n = 38), and cutaneous (28%; n = 40). At a mean ± SD followup of 13 ± 9 years, the 5- and 10-year survival rates were 95 ± 2% and 91 ± 3%, respectively. Factors associated with death on multivariable analysis were male sex (P = 0.01), cardiac abnormalities (P = 0.03), and concomitant myelodysplastic syndrome (MDS) (P = 0.004) or another hematologic malignancy (P = 0.01). Cluster analysis revealed that separating patients into 3 groups was clinically relevant, thereby separating patients with associated MDS, those with tracheobronchial involvement, and those without the 2 features in terms of clinical characteristics, therapeutic management, and prognosis. CONCLUSION: This large series of patients with definite RP revealed an improvement in survival as compared with previous studies. Factors associated with death were male sex, cardiac involvement, and concomitant hematologic malignancy. We identified 3 distinct phenotypes.
Authors: Thomas Rose; Udo Schneider; Martina Bertolo; Jens Klotsche; Vincent Casteleyn; Robert Biesen; Gerd R Burmester; Falk Hiepe Journal: Rheumatol Int Date: 2018-08-06 Impact factor: 2.631
Authors: Marcela A Ferrada; Peter C Grayson; Shubhasree Banerjee; Keith A Sikora; Robert A Colbert; Ninet Sinaii; James D Katz Journal: Arthritis Care Res (Hoboken) Date: 2018-07-04 Impact factor: 4.794
Authors: David B Beck; Marcela A Ferrada; Keith A Sikora; Amanda K Ombrello; Jason C Collins; Wuhong Pei; Nicholas Balanda; Daron L Ross; Daniela Ospina Cardona; Zhijie Wu; Bhavisha Patel; Kalpana Manthiram; Emma M Groarke; Fernanda Gutierrez-Rodrigues; Patrycja Hoffmann; Sofia Rosenzweig; Shuichiro Nakabo; Laura W Dillon; Christopher S Hourigan; Wanxia L Tsai; Sarthak Gupta; Carmelo Carmona-Rivera; Anthony J Asmar; Lisha Xu; Hirotsugu Oda; Wendy Goodspeed; Karyl S Barron; Michele Nehrebecky; Anne Jones; Ryan S Laird; Natalie Deuitch; Dorota Rowczenio; Emily Rominger; Kristina V Wells; Chyi-Chia R Lee; Weixin Wang; Megan Trick; James Mullikin; Gustaf Wigerblad; Stephen Brooks; Stefania Dell'Orso; Zuoming Deng; Jae J Chae; Alina Dulau-Florea; May C V Malicdan; Danica Novacic; Robert A Colbert; Mariana J Kaplan; Massimo Gadina; Sinisa Savic; Helen J Lachmann; Mones Abu-Asab; Benjamin D Solomon; Kyle Retterer; William A Gahl; Shawn M Burgess; Ivona Aksentijevich; Neal S Young; Katherine R Calvo; Achim Werner; Daniel L Kastner; Peter C Grayson Journal: N Engl J Med Date: 2020-10-27 Impact factor: 91.245