AB1096 CLINICAL SPECTRUM AND TREATMENT OF THE RELAPSING POLYCHONDRITIS: A SERIES OF 12 CASES
Authors: Pablo Moreno, Dolores Martinez-Quintanilla Jimenez, Noelia Garca Castaeda, Jose-Maria Alvaro-Gracia, Isidoro Gonzlez-lvaro, Alicia Humbra, Irene Llorente, Ana Ortiz, Esther Patio, Eva Tomero Muriel, Esther Vicente, Rosario Garcia de Vicuna, Santos Castaeda
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Relapsing polychondritis (RP) is a systemic autoimmune disease characterized by inflammation of the cartilaginous tissues associated with the appearance of symptoms caused by the involvement of other non-cartilaginous tissues and the presence of systemic symptoms. <h3>Objectives</h3> To describe the clinical characteristics and treatment of patients diagnosed with RP in a third level university hospital. <h3>Methods</h3> Data from RP patients were obtained from the computerized medical history of our center, including demographics, disease phenotype, treatment, laboratory and imaging studies. <h3>Results</h3> A total of 12 patients were included; 66.7% were women, age at diagnosis was 36 [34-52.5] years (median [p25-p75]). The main manifestation observed was auricular involvement (75%) followed by nasal (66.7%), joint (66.7%), and laryngo-tracheo-bronchial disease (50%). All patients were on DMARDs treatment and two thirds of the patients were receiving biological therapy at the time of analysis. Infliximab was the first biological line in all the cases (100%). Other biological therapies used, in order of frequency, were: adalimumab (25%), rituximab (25%) and tocilizumab (12.5%). 50% of the patients associated another autoimmune disease diagnosed before or during RP follow-up: Crohn9s disease, epidermolysis bullosa, primary biliary cholangitis, autoimmune hypothyroidism, systemic lupus erythematosus, granulomatosis with polyangiitis and ulcerative colitis. Only one patient (8.33%) associated hematologic disease (myelodysplastic syndrome) and another one died during follow-up. <h3>Conclusion</h3> RP is a rare disease with a broad spectrum of clinical expression, which often requires intensive management to control its manifestations, potentially serious when there is involvement of the tracheobronchial tree or is associated with other autoimmune diseases. A large part of our patients require biological therapy at some point in their evolution. <h3>References</h3> <h3>Disclosure of Interests</h3> Pablo Moreno: None declared, DOLORES MARTINEZ-QUINTANILLA JIMENEZ: None declared, Noelia Garca Castaeda: None declared, Jose-Maria Alvaro-Gracia Consultant for: AbbVie, Bristol-Myers Squibb, Eli Lilly, MSD, Novartis, Pfizer Inc, Roche, Sanofi, and UCB, Speakers bureau: AbbVie, Bristol-Myers Squibb, Eli Lilly, MSD, Novartis, Pfizer Inc, Roche, Sanofi, and UCB, Isidoro Gonzlez-lvaro: None declared, Alicia Humbra: None declared, Irene Llorente : None declared, Ana Ortiz: None declared, Esther Patio: None declared, Eva Tomero Muriel: None declared, Esther Vicente: None declared, Rosario Garcia de Vicuna Grant/research support from: Abbvie, BMS, Lilly, MSD, Novartis, Roche, Consultant for: Biogen, BMS, Mylan, Pfizer, Sanofi and Sandoz, Speakers bureau: BMS, Pfizer, Lilly, Sandoz, Santos Castaeda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB