208例SLE临床和免疫学特征分析

为探讨皮肤科门诊定期就诊的SLE患者临床特征，自身抗体分布及长期皮质类固醇治疗引起的副作用，统计分析了208例SLE的首发症状及病程中临床表现和自身抗体分布情况，并分年龄、性别组进行分析，比较。结果：所有患者一般特征，临床表现和免疫学特征和国内外报道基本一致，首发症状为蝶形红斑者占60.58%，少年组及年轻男性组内脏受累重，皮类固醇副作用以浅表真菌感染最常见。     

112例皮肌炎临床分析

目的探讨皮肌炎患者的临床特点、血清学改变、实验室检查及合并恶性肿瘤的发生率。方法总结112例皮肌炎患者发病年龄、首发症状、皮疹表现、受累肌群、血清肌酶特征、伴发恶性肿瘤情况,并进行分析比较。结果①患者62.5%以皮炎为首发症状,主要以眶周融合紫红色斑为主要表现。②患者以四肢肌群受累为主。③恶性肿瘤发生率占17.86%,④乳酸脱氢酶(LDH)和磷酸肌酸激酶(CK)阳性率分别为72.32%,46.43%,与国内报道基本一致。结论特殊的皮疹表现及血清肌酶增高对皮肌炎有诊断意义,在诊疗皮肌炎患者中,应注意有无合并肿瘤,对判断预后有重要意义。     

伴恶性肿瘤皮肌炎的研究进展

皮肌炎／多发性肌炎的恶性肿瘤的伴发率约为2．5％～29．0％，中老年皮肌炎患者更易伴发恶性肿瘤，多见于40～69岁，多数皮肌炎发病后2年内发生肿瘤，可伴各种肿瘤，以鼻咽癌最常见，皮肤血管炎表现者可能与肿瘤有关，其发生机制涉及免疫学、分子生物学改变及其他等，皮肌炎症状、体征与肿瘤消长相平行，伴恶性肿瘤者疗效和预后均差。     

儿童皮肌炎27例临床特点分析

目的探讨儿童皮肌炎的临床特点。方法对27例儿童皮肌炎的临床表现及实验室检查结果进行回顾性分析。结果本组JDM中,男女比例为1:1.7,首发症状以皮疹为主,儿童患者的面部水肿性紫红斑阳性率显著高于成人患者,发热、关节痛、心脏受累、吞咽困难的阳性率显著低于成人患者,未见间质性肺炎及恶性肿瘤。结论儿童皮肌炎以女性多见,皮肤损害为首发症状,系统损害轻,且不伴发恶性肿瘤,预后较好。     

成人Still病30例分析

总结了30例成人Still病（Adult-OnsetStill‘Disease简称AOSD）的临床和实验室资料，分析其发病诱因，首发症状、临床表现、实现室检查以及治疗, 结果显示：除有一定的内科症状及体征之外，93．33％的患者有皮疹，占第二位，且有10％的患者以皮疹为首发症状，皮疹具有一过性、多形性之特点，以躯干多见，具有诊断意义，但容易误诊或漏诊，治疗以皮质类固醇激素和非甾体抗炎药为首选。     

51例患者外阴外用皮质类固醇激素的副作用及治疗情况分析

目的:探讨外阴外用皮质类固醇激素引起的副作用及治疗情况.方法:对51例外阴外用皮质类固醇激素引起副作用患者的临床症状进行分析.结果:51例患者表现多种多样,主要为局部毛细血管扩张、潮红、粟丘疹、丘脓疱疹、皮肤萎缩、色素沉着等临床表现.在停用皮质类固醇激素后又出现局部皮肤发红、水肿、灼热和瘙痒,复用皮质类固醇激素后又可以控制症状.结论:长期外阴外用皮质激素副作用明显,采用中西医结合疗法和激素渐退疗法,疗效满意.     

以皮肌炎为首发症状的鼻咽癌一例

患者,男,19岁,3个月前以皮肌炎为首发症状就诊我院,查肌酶、皮肤活检、肌肉活检等明确诊断为皮肌炎。病程中缺乏鼻咽癌的常见临床症状,查血清EB病毒抗体及头颈部影像学结果异常,鼻咽部穿刺组织病理检查：低分化鳞状细胞癌（淋巴上皮癌）。诊断：皮肌炎;鼻咽癌。给予甲泼尼龙及放射治疗,目前仍在随访中。     

皮肌炎患者皮质类固醇治疗前后sIL—2R,IL—6水平的研究

目的 探讨可溶性白介素２受体（ｓＩＬ－２Ｒ）及白介素６（ｓＩＬ－６）与皮肌炎发病机理的关系,分析ｓＩＬ－２Ｒ与血清肌酶的关系。方法 用双抗体夹心ＥＬＩＳＡ法及＾３Ｈ－胸腺嘧核苷掺入法检测了３０例皮肌炎患博者用皮质类固醇治疗前后及２８例正常人ｓＩＬ－２Ｒ,ＩＬ－６水平。结果 皮肌炎患者治疗前ｓＩＬ－２Ｒ,ＩＬ－６显著升高。     

合并肿瘤的皮肌炎10例临床分析

目的：探讨皮肌炎合并恶性肿瘤的临床特点。方法：回顾性分析10例皮肌炎合并恶性肿瘤的临床特征。结果：患者年龄58～72岁，男：女=1．5：1，5例有典型皮损，3例出现异色病样改变，4例有明显的呛咳及吞咽困难，4例有肺间质的改变，皮肌炎多出现在肿瘤发生之前，手术治疗后皮肌炎症状明显好转甚至完全消失。结论：皮肌炎合并肿瘤与患者年龄，典型皮损，吞咽困难有一定相关性，与血清肌酶升高无明显关系，应积极查找恶性肿瘤。     

98例皮肌炎患者临床分析

<正>皮肌炎(DM)是一种自身免疫性结缔组织病,该病典型症状是肌肉炎症和皮肤损害,可累及多系统病变,如肺部、关节及心脏等,部分患者还可伴发恶性肿瘤。现将我科近年收治的98例皮肌炎患者的临床资料进行回顾性分析,旨在探讨皮肌炎的首发症状、临床表现、实验室检查等特点,分析易误诊的原因和合     

A comparative study of clinical characteristics,work‐up,treatment, and association to malignancy in dermatomyositis between two tertiary skin centers in the USA and Singapore

Background To date, no study has compared the clinical characteristics, malignancy associations, and treatment of dermatomyositis in predominantly Caucasian vs. Asian populations. Materials and methods This prospective study was conducted to compare clinical characteristics of dermatomyositis, its relationship to malignancy, and treatment between two tertiary medical centers in the USA and Singapore. A total of 19 newly‐diagnosed patients in the USA and 15 patients in Singapore were enrolled. Dermatomyositis or amyopathic dermatomyositis were diagnosed based on clinical assessment, skin and muscle biopsies, and muscle testing. Results Ninety‐five percent of patients in the USA group were of Caucasian descent, while 93% of patients in the Singapore group were of Chinese descent. Both groups were predominantly female. Pruritus was the most common initial symptom reported in both groups, while periungual erythema and Gottron’s papules were the most common skin presentations. Heliotrope eruption was more common in the Singapore group, occurring in 80% of patients vs. 32% of patients in the USA group (P = 0.007). Three patients in the Singapore group developed a malignancy, with two of these patients having nasopharyngeal carcinoma. None of the USA patients developed malignancies in a follow‐ up period of 2–5 years. Immunosuppressive steroid sparing therapy with hydroxychloroquine was more frequently used in Singapore, while topical tacrolimus was more frequently used in the USA. Conclusion The clinical presentations of dermatomyositis vary among different ethnic populations. Chinese patients with dermatomyositis have a significant risk for nasopharyngeal carcinoma.     

系统性红斑狼疮伴肌炎的临床研究

目的探讨系统性红斑狼疮(SLE)伴肌炎患者的临床特点及其意义。方法采用对照研究方法 ,检测了64例SLE伴肌炎患者和43例皮肌炎(DM)患者的血清肌酶、肌电图、肌肉活检等 ,并做统计学分析。结果SLE肌炎的发生率为35 5 % ,症状轻 ,有轻度肌萎缩 ;与DM比较肌无力不明显(P<0 01) ;未见吞咽困难 ;SLE伴肌炎患者病情活动发生率[75 %(48/64)]高于非肌炎组[43 9 %(29/66)] ,有显著性差异(P<0 01) ;肌酶有所升高 ,但升高幅度均不超过正常值的50 % ,与DM患者比较有显著性差异(P<0 01) ;肌电图肌炎检出率为74 4 %(32/43) ,与DM组81 4 %(35/43)比较无显著性差异(P>0 05) ;肌活检显示 :肌间质血管炎 ,淋巴细胞和组织细胞浸润明显 ;肌纤维灶性肿胀 ,匀质变性 ,横纹模糊 ,未见坏死断裂 ,病变轻微。结论SLE肌炎较为常见 ,与SLE活动相关 ,症状轻 ,肌酶升高幅度小 ;肌电图与DM无异 ;肌活检间质病变明显 ,肌纤维病变轻微 ,有明显的临床和病理特征而有别于DM。     

Update: Dermatomyositis

Dermatomyositis is an idiopathic inflammatory myopathy and an “orphan disease” (incidence 1:100,000). It comprises a heterogenous clinical spectrum with periorbital heliotrope erythema, acral Gottron papules, and proximal muscle weakness. Muscle-specific antibody profiles correlate with clinical variants. Overlap with other collagen vascular disorders occurs and about one-third of patients have an underlying malignancy (paraneoplastic dermatomyositis). High-dose oral corticosteroids are the mainstay of treatment, given until improvement of muscle symptoms and/or normalization of muscle enzymes Additional options include steroid-sparing immunosuppressants, or high dose intravenous immunoglobulins. Prognosis has improved considerably since use of high-dose corticosteroids, with about 90% of patients responding. Follow-up and search for a possible malignancy should be performed yearly.     

Malignancy in adult dermatomyositis

Background Dermatomyositis has been reported to be associated with malignancies in 15%–34% of patients in Western countries, but in as many as two-thirds of patients in Singapore. The aim of this study was to determine whether a diagnostic measure could be helpful in the diagnosis of a malignancy in patients with dermatomyositis. Methods This is a retrospective study on 38 adult patients with dermatomyositis that were seen over a 6-year period from 1989 to 1994. Results All the patients presented with cutaneous features that suggest the clinical diagnosis of dermatomyositis; however, not all cases show all the key features of the disease. Of the studied patients, 86.8% were noted to have photosensitivity as a key cutaneous presentation. Thirty (78.9%) of our patients were above the age of 40 years, and 12 (31.6%) of these were found to have an associated malignancy. Nasopharyngeal carcinoma was the most commonly associated malignancy (38.4%) in our study population. Conclusions In our study population, otorhinolaryngologic screening is an essential investigation for the evaluation of dermatomyositis in association with malignancy.     

Dermatomyositis and malignancy in Tunisia: a multicenter national retrospective study of 20 cases

OBJECTIVE AND METHODS: The aim of our study was to report the epidemiologic, clinical, and biologic profiles of dermatomyositis (DM) associated with malignancy in patients from Tunisia. From January 1982 to January 2000, we collected retrospectively 20 case reports of DM associated with cancer from the different university hospital centers of Tunisia. Initial workup included anamnesis, clinical examination, cancer staging and classification, serum muscle enzymes (creatine phosphokinase, lactate dehydrogenase, aldolase, and transaminases), electromyography, and muscular biopsy. We calculated the median survival and mean value of all the variables. Comparisons of statistical tests were done with the Kruskal-Wallis test. RESULTS: Among the 130 DM cases of our study, 20 were associated with cancer (15.38%). The mean age of our patients was 49.6 years and the sex ratio (female/male) was equal to 3. Cancers were mainly those of the breast (35%) and nasopharynx (25%). DM followed a paraneoplastic course in 90% of the cases. The profile of seric muscular enzymes showed a significant statistical difference (P =.05) between a group of patients with severe muscular weakness and a group with moderate muscle weakness only for creatine kinase. The median survival was 36.5 months after diagnosis of DM and 48.6 months after that of cancer. The 5-year actuarial survival was 38% as related to cancer and 16% as related to DM. Mortality was 45%, in 90% as a result of cancer. CONCLUSIONS: In our study, nasopharyngeal carcinoma represents the second cancer associated with DM, after breast neoplasm, demonstrating the frequency of these 2 cancers in our country. Despite our reduced number of study samples, our study also suggests a relationship between severe muscle weakness and high seric muscle enzymes.     

A systematic review of juvenile-onset clinically amyopathic dermatomyositis

BACKGROUND: Dermatomyositis (DM) presenting during childhood or adolescence classically encompasses hallmark cutaneous changes, proximal muscle weakness, and laboratory evidence of myositis. When cutaneous manifestations of DM are present without muscle weakness for > 6 months, the term 'clinically amyopathic DM' applies (syn. DM sine myositis). OBJECTIVES: To review the clinical and epidemiological features of published cases of juvenile-onset clinically amyopathic DM, with comparison with adult-onset clinically amyopathic DM and juvenile-onset classical DM. METHODS: Systematic review of the published literature. RESULTS: We identified 68 cases of juvenile-onset clinically amyopathic DM published during 1963-2006. The disease in 18 of 68 (26%) patients subsequently evolved to classical DM. Overall, the mean age at diagnosis was 10.8 years (range 2-17) with nearly equal male/female ratio and mean follow-up of 3.9 years. Among cases with diagnostic testing, 10 of 19 had a positive antinuclear antibody titre, two of nine had elevated erythrocyte sedimentation rate and two of 51 had elevated creatine kinase (CK). Of patients with normal CK, three of 22 had abnormal electromyography, one of 19 had abnormal muscle biopsy, and one of nine had abnormal magnetic resonance imaging. Calcinosis was reported in three of 68. No cases of severe vasculopathy (resulting in ulceration), interstitial lung disease or internal malignancy were reported. CONCLUSIONS: This review suggests a good prognosis for children with clinically amyopathic DM. A minority of patients with negative muscle enzymes had positive ancillary testing for myositis, and these patients rarely developed muscle weakness. Predictive factors for progression to classical DM were not identified. Symptomatic treatment of cutaneous involvement and close clinical monitoring may be an alternative to aggressive immunosuppression.     

成人皮肌炎患者抗核抗体与临床特征及肿瘤风险的关系

目的 探讨成人皮肌炎患者抗核抗体与临床特征及肿瘤风险的关系.方法 回顾性分析2008年4月至2018年4月在苏州大学附属第一医院皮肤科住院的101例皮肌炎患者的临床资料,分为抗核抗体阳性组和阴性组,比较两组之间肌病、肿瘤发生风险以及其他临床特征的差异.92例患者随访2年.采用卡方检验分析比较两组的临床特征,利用多因素回...     

儿童多发性肌炎与皮肌炎17例临床分析

目的探讨儿童皮肌炎/多发性肌炎(JDM/PM)的临床特点、辅助检查与治疗。方法回顾性分析本院1994年1月~2004年12月诊治的17例JDM/JPM。结果主要有肌无力、肌痛或肌压痛、关节疼痛和发热等;存在肺、心脏、胃肠道、肾损害,未见伴恶性肿瘤和钙质沉着者;LDH、HBDH、AST、CPK、α2、γ球蛋白升高,HB降低,ESR增快,RF阳性;EMG示肌源性改变,肌活检异常。结论本病以肌肉症状最多见,肌酶升高显著,以心、肺损害多见,经激素治疗均有所缓解,预后较好。