Mekinian Arsène, Braun Thorsten, Decaux Olivier, Falgarone Géraldine, Toussirot Eric, Raffray Loic, Omouri Mohamed, Gombert Bruno, De Wazieres Benoit, Buchdaul Anne-Laure, Ziza Jean-Marc, Launay David, Denis Guillaume, Madaule Serge, Rose Christian, Grignano Eric, Fenaux Pierre, Fain Olivier
From the Service de médecine interne (AM, EG, OF), Université Paris 13, AP-HP, Hôpital Jean Verdier, Bondy; Service d'hématologie clinique (TB, PF), Université Paris 13, AP-HP, Avicenne, Bobigny; Service de médecine interne (OD), Université Rennes 1, Hôpital Universitaire de Rennes, Rennes; Service de rhumatologie (GF), Université Sorbonne Paris Cité, Université Paris 13, Li2P, EA4222, Hôpital Avicenne, Bobigny; Centre Investigation Clinique Biothérapie CBT-506 & Service de rhumatologie (ET), CHU Besançon, Besançon; Service de médecine interne (LR), CHU de Bordeaux, Bordeaux; Service de rhumatologie (MO), CH Romilly/Seine; Service de médecine interne (BG), Hôpital de La Rochelle, La Rochelle; Service de médecine interne et gériatrique (BDW), CHU Nîmes, Nîmes; Service de médecine interne (A-LB), CH Douai, Douai; Service de rhumatologie (J-MZ), CH Croix Saint Simon, Paris; Service de médecine interne (DL), CHU Lille, Université Lille II, Lille; Service de médecine interne (GD), Hôpital de Rochefort, Rochefort; Service de médecine interne (SM), CHG Albi, Albi; and Service d'hématologie clinique (CR), Hôpital Saint-Vincent de Paul, UC Lille, Univ Nord de France, Lille; France.
Medicine (Baltimore). 2014 Jan;93(1):1-10. doi: 10.1097/MD.0000000000000011.
We describe the characteristics and outcome of inflammatory arthritis in patients with myelodysplastic syndrome (MDS) in a French multicenter retrospective study. Twenty-two patients with MDS (median age, 77.5 yr [interquartile range, 69-81]; 10 women) were included. Inflammatory arthritis presented as polyarthritis in 17 cases (77%) and with symmetric involvement in 15 cases (68%). At diagnosis, the median disease activity score 28 based on C-reactive protein (DAS28-CRP) was 4.5 [2-6.5]. Two patients had anti-citrullinated protein antibodies (ACPAs), and 1 had radiologic erosions. The median time between the diagnoses of arthritis and MDS was 10 months [6-42], with a median articular symptom duration of 3 months [2-8]. The diagnosis of both diseases was concomitant in 6 cases (27%); arthritis preceded MDS in 12 cases (55%), and occurred after MDS in 4 (18%). While the number of swollen and tender joints significantly decreased during follow-up, as did the median DAS28-CRP (from 4.3 [3.8-4.6] at baseline to 2.9 [1.75-3.3]; p < 0.05), CRP remained elevated (CRP >20 mg/L) in 8 patients (42%). Nevertheless, radiographic progression and new ACPA positivity were not observed during a median follow-up of 29 months [9-76]. While most of the patients were treated with steroids (n = 16) for arthritis, additional treatment was administered in only 4 patients (hydroxychloroquine, n = 2; sulfasalazine [Salazopyrin] and etanercept, n = 1, respectively). Eleven patients died during follow-up from acute myeloid leukemia (n = 5); infections (n = 3); or cerebral bleeding, cardiorespiratory failure, or undetermined cause (n = 1, respectively). Inflammatory arthritis associated with MDS can have various presentations and is often seronegative and nonerosive. Steroids alone are the most common treatment in MDS-associated arthritis, but that treatment is insufficient to control arthritis. Steroid-sparing strategies need to be identified.
在一项法国多中心回顾性研究中,我们描述了骨髓增生异常综合征(MDS)患者炎症性关节炎的特征及转归。纳入了22例MDS患者(中位年龄77.5岁[四分位间距69 - 81];10例女性)。炎症性关节炎表现为多关节炎的有17例(77%),呈对称性受累的有15例(68%)。诊断时,基于C反应蛋白的疾病活动度评分28(DAS28 - CRP)中位数为4.5[2 - 6.5]。2例患者有抗瓜氨酸化蛋白抗体(ACPA),1例有放射学侵蚀表现。关节炎与MDS诊断之间的中位时间为10个月[6 - 42],关节症状的中位持续时间为3个月[2 - 8]。两种疾病同时诊断的有6例(27%);关节炎先于MDS诊断的有12例(55%),在MDS诊断后发生的有4例(18%)。随访期间,肿胀和压痛关节数量显著减少,DAS28 - CRP中位数也下降(从基线时的4.3[3.8 - 4.6]降至2.9[1.75 - 3.3];p < 0.05),但仍有8例患者(42%)的C反应蛋白(CRP)持续升高(CRP > 20 mg/L)。然而,在中位29个月[9 - 76]的随访期间未观察到放射学进展及新的ACPA阳性。大多数患者因关节炎接受了类固醇治疗(n = 16),仅4例患者接受了额外治疗(分别为羟氯喹,n = 2;柳氮磺吡啶[Salazopyrin]和依那西普,n = 1)。随访期间11例患者死亡,死因分别为急性髓系白血病(n = 5)、感染(n = 3)、脑出血、心肺功能衰竭或病因不明(各n = 1)。与MDS相关的炎症性关节炎可有多种表现形式,常为血清阴性且无侵蚀性。单独使用类固醇是MDS相关关节炎最常见的治疗方法,但该治疗不足以控制关节炎。需要确定节省类固醇的策略。