Arsène Mekinian1, Eric Grignano1, Thorsten Braun2, Olivier Decaux3, Eric Liozon4, Nathalie Costedoat-Chalumeau5, Jean-Emmanuel Kahn6, Mohammed Hamidou7, Sophie Park8, Xavier Puéchal5, Eric Toussirot9, Géraldine Falgarone10, David Launay11, Nathalie Morel5, Sébastien Trouiller12, Alexis Mathian13, Bruno Gombert14, Yoland Schoindre6, Bertrand Lioger15, Benoit De Wazieres16, Zahir Amoura13, Anne-Laure Buchdaul17, Sophie Georgin-Lavialle18, Jérémie Dion5, Serge Madaule19, Loïc Raffray20, Pascal Cathebras21, Jean Charles Piette5, Christian Rose22, Jean Marc Ziza23, Olivier Lortholary24, Francois Montestruc25, Mohammed Omouri26, Guillaume Denis27, Julien Rossignol28, Stanislas Nimubona29, Lionel Adès30, Claude Gardin2, Pierre Fenaux30, Olivier Fain31. 1. AP-HP, Hôpital Saint Antoine, service de medicine interne and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Paris. 2. Service d'hématologie clinique, Université Paris 13, AP-HP, Avicenne, Bobigny. 3. Service de médecine interne, Université Rennes 1, Hôpital Universitaire de Rennes, Rennes. 4. Service de médecine interne, Université Limoges, Hôpital Limoges, Limoges. 5. Université Paris Descartes, AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne, Paris. 6. Service de médecine interne, Hôpital Foch, Université Versailles Saint Quentin en Yvelines, Suresnes. 7. Service de médecine interne, Hôpital Nantes, Université Nantes, Nantes. 8. Service d'hématologie clinique, Hôpital La Tronche, Grenoble. 9. Centre Investigation Clinique Biothérapie CIC-1431 & Service de rhumatologie, CHU Besançon, Besançon. 10. USPC Université Paris 13, INSERM, APHP; UMR 1125, service de rhumatologie; Hôpital Avicenne, 125 route de Stalingrad, 93009 Bobigny. 11. Service de médecine interne, CHU Lille, Université Lille II, Lille. 12. Service de médecine interne, Hôpital Henri Mondor, Aurillac. 13. Service de médecine interne, Hôpital Pitié Salpetrière, Université Paris 6, AP-HP, Paris. 14. Service de médecine interne et rhumatologie, Groupe hospitalier La Rochelle Ré Aunis, La Rochelle. 15. Service de médecine interne, Hôpital de Tours, Université de Tours, Tours. 16. Service de médecine interne et gériatrique, CHU Nîmes, Nîmes. 17. Service de médecine interne, CH Douai, Douai. 18. Service de médecine interne, Hôpital HEGP, Université Paris 5, AP-HP, Paris. 19. Service de médecine interne, CHG Albi, Albi. 20. Service de médecine interne, CHU de Bordeaux, Bordeaux. 21. Service de médecine interne, Hôpital Nord CHU Saint Etienne, Saint Etienne. 22. Service d'hématologie clinique, Hôpital Saint-Vincent de Paul, UC Lille, Université Nord de France, Lille. 23. Service de rhumatologie, CH Croix Saint Simon. 24. Service de maladies infectieuses, Hôpital Necker, Université Paris 5, AP-HP, Paris. 25. Biostatistics, eXYSTAT SAS, Levallois-Perret. 26. Service de rhumatologie, CH Romilly/Seine. 27. Service de médecine interne, Hôpital de Rochefort, Rochefort. 28. Service d'hématologie, Hôpital Necker, Université Paris 5, APHP, Paris. 29. Service d'hématologie clinique, Université Rennes 1, Hôpital Universitaire de Rennes, Rennes and. 30. Service d'hématologie clinique, Hôpital Saint-Louis, AP-HP, Université Paris 7, Paris, France. 31. AP-HP, Hôpital Saint Antoine, service de medicine interne and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Paris, olivier.fain@sat.aphp.fr.
Abstract
OBJECTIVE: We describe myelodysplastic syndrome (MDS)-associated systemic inflammatory and autoimmune diseases (SIADs), their treatments and outcomes and the impact of SIADs on overall survival in a French multicentre retrospective study. METHODS: In this study, 123 patients with MDS and SIADs were analysed. RESULTS: Mean age was 70 years (s.d. 13) and the male:female ratio was 2. The SIADs were systemic vasculitis in 39 (32%) cases, CTD in 31 (25%) cases, inflammatory arthritis in 28 (23%) cases, a neutrophilic disorder in 12 (10%) cases and unclassified in 13 cases (11%). The SIADs fulfilled the usual classification criteria in 75 (66%) cases, while complete criteria were not reached in 21 (19%) cases. A significant association was shown between chronic myelomonocytic leukaemia (CMML) and systemic vasculitis (P = 0.0024). One hundred and eighteen (96%) SIAD patients were treated (91% with steroids), with an 83% response to first-line treatment, including 80% for steroids alone. A second-line treatment for SIADs was required for steroid dependence or relapse in 48% of cases. The effect of MDS treatment on SIADs could be assessed in 11 patients treated with azacytidine and SIAD response was achieved in 9/11 (80%) and 6/11 (55%) patients at 3 and 6 months, respectively. Compared with 665 MDS/CMML patients without SIADs, MDS/CMML patients with SIADs were younger (P < 0.01), male (P = 0.03), less often had refractory anaemia with ring sideroblasts (P < 0.01), more often had a poor karyotype (16% vs 11%, P = 0.04) and less frequently belonged to low and intermediate-1 International Prognostic Scoring System categories, but no survival difference was seen between patients with MDS-associated SIADs and without SIADs (P = 0.5). CONCLUSION: The spectrum of SIADs associated to MDS is heterogeneous, steroid sensitive, but often steroid dependent.
OBJECTIVE: We describe myelodysplastic syndrome (MDS)-associated systemic inflammatory and autoimmune diseases (SIADs), their treatments and outcomes and the impact of SIADs on overall survival in a French multicentre retrospective study. METHODS: In this study, 123 patients with MDS and SIADs were analysed. RESULTS: Mean age was 70 years (s.d. 13) and the male:female ratio was 2. The SIADs were systemic vasculitis in 39 (32%) cases, CTD in 31 (25%) cases, inflammatory arthritis in 28 (23%) cases, a neutrophilic disorder in 12 (10%) cases and unclassified in 13 cases (11%). The SIADs fulfilled the usual classification criteria in 75 (66%) cases, while complete criteria were not reached in 21 (19%) cases. A significant association was shown between chronic myelomonocytic leukaemia (CMML) and systemic vasculitis (P = 0.0024). One hundred and eighteen (96%) SIAD patients were treated (91% with steroids), with an 83% response to first-line treatment, including 80% for steroids alone. A second-line treatment for SIADs was required for steroid dependence or relapse in 48% of cases. The effect of MDS treatment on SIADs could be assessed in 11 patients treated with azacytidine and SIAD response was achieved in 9/11 (80%) and 6/11 (55%) patients at 3 and 6 months, respectively. Compared with 665 MDS/CMMLpatients without SIADs, MDS/CMMLpatients with SIADs were younger (P < 0.01), male (P = 0.03), less often had refractory anaemia with ring sideroblasts (P < 0.01), more often had a poor karyotype (16% vs 11%, P = 0.04) and less frequently belonged to low and intermediate-1 International Prognostic Scoring System categories, but no survival difference was seen between patients with MDS-associated SIADs and without SIADs (P = 0.5). CONCLUSION: The spectrum of SIADs associated to MDS is heterogeneous, steroid sensitive, but often steroid dependent.
Authors: Keith A Sikora; Kristina V Wells; Ertugrul Cagri Bolek; Adrianna I Jones; Peter C Grayson Journal: Rheumatology (Oxford) Date: 2022-08-03 Impact factor: 7.046
Authors: Andreas Glenthøj; Andreas Due Ørskov; Jakob Werner Hansen; Sine Reker Hadrup; Casey O'Connell; Kirsten Grønbæk Journal: Int J Mol Sci Date: 2016-06-15 Impact factor: 5.923
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