Relapsing Polychondritis Complicated by Myelodysplastic Syndrome Is Resistant to Immunosuppression: Comment on the Article by Dion et al.
Relapsing Polychondritis Complicated by Myelodysplastic Syndrome Is Resistant to Immunosuppression: Comment on the Article by Dion et al.
复制标题
骨髓增生异常综合征并发的复发性多软骨炎对免疫抑制有抵抗力：对 Dion 等人的文章的评论。
DOI：
10.1002/art.39969
复制
发表时间：
2017
影响因子：
13.3
通讯作者：
Harigae H.
中科院分区：
文献类型：
--
作者：
Shirai T;Fujii H;Saito R;Nasu K;Kamogawa Y;Fukuhara N;Fujita Y;Shirota Y;Ishii T;Harigae H.
我们饶有兴趣地阅读了Dion及其同事最近发表的一篇文章，他们在文章中报道，复发性多软骨炎（RP）患者可分为3种临床表型：血液病、呼吸性和轻度(1)。血液学表型与死亡有关，呼吸系统表型与感染和入住重症监护病房有关，轻度表型与没有严重并发症有关。这篇报道引起了我们的极大兴趣，因为我们也遇到了一个致命的RP合并骨髓增生异常综合征（MDS）和Sweet综合征的病例。该患者的临床过程非常不寻常，提示RP合并这些疾病是一种独特的综合征，需要制定特定的治疗策略。我们在我院治疗了14例RP患者（男性57.1%，平均SD年龄53.366 ~ 16.51岁，平均SD随访时间5.6764岁）。2年)。其中耳部（85.7%）、鼻腔（14.3%）、气管（7.1%）软骨受影响。57.1%的患者出现软骨外症状，包括脑炎和脑膜炎、巩膜炎、葡萄膜炎、甲状腺功能减退、类风湿关节炎、behet综合征、IgA肾病、MDS和Sweet综合征。患者接受皮质类固醇治疗（平均剂量42.36 20.9 mg/天），但大多数单独接受类固醇治疗的患者复发（复发率71.4%），需要额外的免疫抑制剂（甲氨蝶呤、环孢素或环磷酰胺）。然而，除了一例合并MDS和Sweet综合征外，他们的生存率都很好。患者为56岁男性，右耳廓软骨出现关节炎、皮疹和肿胀。根据症状和组织学结果（图1A），诊断为RP。1个月后，患者面部出现红斑和丘疹，诊断为Sweet综合征（图1B）。次年，他出现葡萄膜炎和MDS（难治性贫血）（图1C）。他有时会出现疾病发作，即使加入其他免疫抑制药物（甲氨蝶呤、环孢素、咪唑利啶或硫唑嘌呤），也很难将强的松龙的剂量减少到15mg以下。
We read with interest the recent article by Dion and colleagues, in which they reported that patients with relapsing polychondritis (RP) could be classified into 3 clinical phenotypes: hematologic, respiratory, and mild (1). The hematologic phenotype was associated with death, the respiratory phenotype was associated with infections and admission to the intensive care unit, and the mild phenotype was associated with the absence of severe complications. This report was of great interest to us, because we also encountered a lethal case of RP complicated by myelodysplastic syndrome (MDS) and Sweet syndrome. The clinical course of this patient was quite unusual, suggesting that RP complicated by these diseases is a distinct syndrome, and development of specific therapeutic strategies is needed. We had been treating 14 RP patients at our institution (57.1% male, mean6 SD age 53.366 16.51 years, mean6 SD follow-up time 5.6764. 2 years). The affected cartilages were auricular (85.7%), nasal (14.3%), and tracheal (7.1%). In 57.1% of the patients, extracartilage symptoms developed, including encephalitis and meningitis, scleritis, uveitis, hypothyroidism, rheumatoid arthritis, Behçet’s syndrome, IgA nephropathy, MDS, and Sweet syndrome. Patients were treated with corticosteroids (mean6 SD prednisolone dosage 42.36 20.9 mg/day), but most of the patients who were treated solely with steroids experienced a relapse (relapse rate 71.4%), and additional immunosuppressants (methotrexate, cyclosporine, or cyclophosphamide) were needed. However, their survival was excellent except for a case that was complicated by MDS and Sweet syndrome. The patient, a 56-year-old man, developed arthritis, eruptions, and swelling of the right auricular cartilage. Based on the symptoms together with the histologic findings (Figure 1A), he was diagnosed as having RP. One month later, erythema and papules appeared on his face, and he was diagnosed as having Sweet syndrome (Figure 1B). The next year, he developed uveitis and MDS (refractory anemia)(Figure 1C). He sometimes experienced disease flare, and it was difficult to reduce the dose of prednisolone below 15 mg even though other immunosuppressive drugs (methotrexate, cyclosporine, mizorivine, or azathioprine) were added.