皮肌炎伴发肺间质病变的相关因素分析

目的了解皮肌炎肺间质病变的临床特点,探讨影响皮肌炎伴发肺间质病变(ILD)的危险因素。方法回顾性研究近3年来收治的83例皮肌炎(DM)患者临床资料,采用Logistic回归分析皮肌炎伴发ILD的影响因素。结果皮肌炎患者ILD的发生率为42.2%。筛选出皮肌炎伴发ILD的主要相关因素有Gottron’s皮疹、恶性肿瘤、雷诺现象、干咳、关节痛和肌力,其中Gottron’s皮疹及恶性肿瘤与ILD呈负相关,雷诺现象、干咳、关节痛和肌力与ILD呈正相关。结论Gottron’s皮疹阳性或伴发恶性肿瘤的DM患者ILD的发生率降低,有雷诺现象、干咳、关节痛或肌力较好的DM患者发生ILD的概率较高。     

皮肌炎患者Gottron皮疹与肺间质病变

目的 探讨皮肌炎（DM）患者中Gottron皮疹与肺间质病变（ILD）的关系。方法 回顾分析83例皮肌炎患者的临床资料,根据有无Gottron皮疹分组,比较两组的临床特点。结果 DM患者Gottron皮疹的总阳性率为59%（49/83例）,ILD的发生率为42.2%（35/83例）,Gottron皮疹阳性组ILD的发生率明显低于Gottron皮疹阴性组,两者差异有统计学意义（24.5%比67.6%,P ＜ 0.01）。35例ILD中Gottron皮疹阴性者的CO弥散率（DLCO）明显低于Gottron皮疹阳性者（60.9 ± 13.5比72.3 ± 12.7,P ＜ 0.05）。Gottron皮疹阳性组与阴性组的肌力（4.0 ± 0.9比3.8 ± 1.2,P ＞ 0.05）、肌肉压痛（63.3%比70.6%,P ＞ 0.05）、肌酶水平（肌酸磷酸激酶1047.9 ± 1402.7比1359.7 ± 1752.4,乳酸脱氢酶397.2 ± 226.5比402.4 ± 197.0,AST 97.6 ± 98.0比120.1 ± 82.3,P均 ＞ 0.05）比较差异无统计学意义。两组的ESR、CRP水平比较差异亦无统计学意义（分别为26.5 ± 16.4比33.3 ± 25.3,11.9 ± 8.3比15.5 ± 9.8,P均 ＞ 0.05）。Gottron皮疹阳性组与阴性组恶性肿瘤的发生率差异无统计学意义（21.4%比6.5%,P ＞ 0.05）。结论 Gottron皮疹阳性的皮肌炎患者伴发肺间质病变的可能性小,且病变程度可能较轻。Gottron皮疹与DM的肌肉病变程度及病情活动无明显关系,Gottron皮疹可能与恶性肿瘤的发生无明显关系。     

皮肌炎合并肺间质病变

为了解皮肌炎的肺间质病变，对36例皮肌炎患者进行了分析。结果：肺间质病变者8例，占22.2%。皮肌炎合并肺间质病变患者发热、咳嗽发生率高，分别为87.5%、62.5%。抗Jo-1、RNP抗体、ANA阳性率分别为57.1%，42.9%和57.1%，均高于不合并肺间质病变者。     

伴反向Gottron丘疹的皮肌炎三例北大核心CSCD

报道3例伴反向Gottron丘疹的皮肌炎。3例中,男2例,女1例,年龄分别为43、41、46岁,病程1、6、7个月。反向Gottron丘疹表现为指间关节、掌指关节掌侧丘疹,2例呈线状排列。1例伴间质性肺炎,3例有肌炎表现,2例抗核抗体阴性。1例反向Gottron丘疹组织病理示灶性基底细胞液化变性,真皮浅层血管周围见淋巴细胞为主的炎症细胞浸润。3例早期系统应用糖皮质激素及免疫抑制剂治疗3个月后,肌无力、肌痛症状明显改善,但反向Gottron丘疹无明显改善,1例治疗1年后掌侧皮疹明显减轻。     

[开放获取] 临床无肌病性皮肌炎诊断标准的演进

【摘要】 目前皮肌炎诊断使用最广泛的标准是Bohan和Peter标准，该标准要求患者除典型皮疹外，还须满足至少2条肌炎表现才能诊断皮肌炎。临床无肌病性皮肌炎（CADM）因无肌炎表现而被排除在外，这使CADM的早期研究十分匮乏，不被临床医生所重视。另外，诊断CADM依赖于对皮疹的认识和皮损组织病理表现，因CADM早期皮疹表现不典型，往往被误诊为玫瑰痤疮、脂溢性皮炎、红斑狼疮等其他疾病，从而影响早期诊断和治疗以及对疾病预后的判断。本文梳理CADM的诊断标准，以利于其早期识别和诊断，也为开展相关临床研究提供依据。     

皮肌炎伴发肿瘤及死亡的临床分析

目的　探讨皮肌炎致死和伴发肿瘤的相关因素。方法　回顾性分析 1990年～ 2 0 0 3年皮肌炎患者的临床资料 ,分组后用 χ2 检验进行统计学分析。结果　年龄、Gottron丘疹、吞咽困难在皮肌炎伴发肿瘤中有显著意义 ;吞咽困难、声音嘶哑、饮水呛咳、肌肉萎缩、肺纤维化、心脏受累在导致皮肌炎 1年内死亡中有显著意义。结论　高龄、Gottron丘疹、吞咽困难预示皮肌炎伴发肿瘤的可能性大 ;吞咽困难、声音嘶哑、饮水呛咳、肌肉萎缩、肺纤维化、心脏受累提示皮肌炎预后不佳     

皮肌炎83例临床分析

回顾性分析我院2003-2007年明确诊断的83例皮肌炎（DM）患者,针对性别、年龄、职业、诱因、病程、临床表现、实验室检查、伴发疾病、死亡原因等参数做分析.比较活动性间质肺病组、非活动组及无肺病组的病死率. 皮肌炎常见于女性（74.7%）,发病年龄集中在40～60岁.大部分患者无明显诱因,部分病例显示感染（8例）、劳累（5例）、染发（5例）及紫外线（1例）是诱因.从发病到就诊平均时间为半年左右.95.1%有颜面红斑,42.7%有Gottron征,41.5%有吞咽困难,39例做了肌肉活检,其中37例活检阳性,阳性率94.9%.82例作了肌电图,72例显示肌源性损害,阳性率为87.8%,肌电图93.2%有三角肌损害.22例有肺间质病变,15例合并肿瘤,其中80.0%伴肿瘤的皮肌炎患者未见肺间质病变.有5例死亡,4例死于呼吸衰竭,1例死于肿瘤.伴活动性肺间质病变组病死率为25.0%,明显高于非活动性肺间质病变组（0）和无肺间质病变组（0,P＆lt;0.05）.皮肌炎临床表现具有多样化和复杂化的特点,肌肉活检、肌电图、肌酶是皮肌炎重要的诊断依据.皮肌炎常伴发肿瘤和间质性肺炎,肿瘤和活动性肺间质病变是相互独立的预后不良因素.     

皮肌炎83例临床分析

回顾性分析我院2003-2007年明确诊断的83例皮肌炎(DM)患者,针对性别、年龄、职业、诱因、病程、临床表现、实验室检查、伴发疾病、死亡原因等参数做分析.比较活动性间质肺病组、非活动组及无肺病组的病死率. 皮肌炎常见于女性(74.7%),发病年龄集中在40～60岁.大部分患者无明显诱因,部分病例显示感染(8例)、劳累(5例)、染发(5例)及紫外线(1例)是诱因.从发病到就诊平均时间为半年左右.95.1%有颜面红斑,42.7%有Gottron征,41.5%有吞咽困难,39例做了肌肉活检,其中37例活检阳性,阳性率94.9%.82例作了肌电图,72例显示肌源性损害,阳性率为87.8%,肌电图93.2%有三角肌损害.22例有肺间质病变,15例合并肿瘤,其中80.0%伴肿瘤的皮肌炎患者未见肺间质病变.有5例死亡,4例死于呼吸衰竭,1例死于肿瘤.伴活动性肺间质病变组病死率为25.0%,明显高于非活动性肺间质病变组(0)和无肺间质病变组(0,P<0.05).皮肌炎临床表现具有多样化和复杂化的特点,肌肉活检、肌电图、肌酶是皮肌炎重要的诊断依据.皮肌炎常伴发肿瘤和间质性肺炎,肿瘤和活动性肺间质病变是相互独立的预后不良因素.     

Wong型无肌病性皮肌炎一例

患者,女,72岁。头面颈部红斑、丘疹伴瘙痒16年,日晒后加重。实验室检查示：抗TIF1-γ抗体阳性。额部皮肤组织病理表现为表皮角化过度,局灶性角化不全和界面皮炎改变。结合患者典型皮肌炎皮肤表现和额部毛发红糠疹样皮疹,诊断为Wong型无肌病性皮肌炎。给予阿维A、海棠合剂、1%吡美莫司乳膏等治疗,病情逐渐好转。     

65例皮肌炎相关肺间质病变临床分析

目的 总结分析65例皮肌炎相关肺间质病变患者的病史资料,以加强对该病的认识.方法 对2006年1月至2020年12月于四川省人民医院就诊的65例皮肌炎相关肺间质病变患者进行回顾性分析,内容包括临床特征、检验检查结果、治疗及预后.结果 患者以中年女性居多,主要表现为肌无力(76.92％)、肌痛(61.54％)、关节痛(6...     

Amyopathic dermatomyositis: a review by the Italian Group of Immunodermatology.

OBJECTIVE: To analyze the average age, sex distribution, duration of follow-up, clinical course, serologic abnormalities, and incidence of possibly associated malignancy in patients with amyopathic dermatomyositis. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Thirteen patients with amyopathic dermatomyositis. RESULTS: The 13 patients represented 8.2% of 157 patients with dermatomyositis seen retrospectively in a 10-year period by the Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology. Gottron papules and sign and periungual telangiectasias were found in approximately 50% of cases (papules in 7 patients, Gottron sign and periungual telangiectasias in 6), while periorbital violaceous erythema was seen in 70% (9 patients). Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. An elevated erythrocyte sedimentation rate was detected in 6 patients, hepatitis B virus antigen in 3, speckled antinuclear antibodies in 7, and anti-Ro and antimitochondrial antibodies in 1 case each. None of our patients had evidence of internal malignancy. Neither cardiopulmonary nor esophageal dysfunction was demonstrated. Electromyography showed a protopathic muscle abnormality in 3 patients. Muscle biopsy disclosed myositis and a neurogenic myopathy in another one. CONCLUSIONS: Amyopathic dermatomyositis is a rare disease. So far, only 2 series of a few cases each have been reported. The "amyopathic" subset of dermatomyositis is peculiar in that its cutaneous lesions are predominant for long periods or even permanently, although they are indistinguishable from those of classic dermatomyositis. The minimal or absent muscle disease and the rarity of serum immunologic findings imply a favorable prognosis in white patients.     

皮肌炎自身抗体检测研究进展

一些自身抗体包括抗黑素瘤分化相关基因5抗体、抗转录中介因子1γ抗体、抗核基质蛋白2抗体、抗甘氨酰-tRNA合成酶抗体、抗核小体重塑和组蛋白脱乙酰酶蛋白复合物抗体、抗组氨酰-tRNA合成酶抗体等与皮肌炎的临床表型和预后密切相关.抗黑素瘤分化相关基因5抗体、抗甘氨酰-tRNA合成酶抗体、抗组氨酰-tRNA合成酶抗体与皮肌炎并发间质性肺病有关,特别是抗黑素瘤分化相关基因5抗体与皮肌炎并发的间质性肺病的发生、病情活动和患者高死亡率显著相关.通常抗黑素瘤分化相关基因5抗体阳性的患者对常规的免疫抑制治疗疗效欠佳.抗转录中介因子1γ抗体、抗核基质蛋白2抗体与恶性肿瘤相关性皮肌炎有关.此外抗转录中介因子1γ抗体和抗核基质蛋白2抗体还与皮肌炎其他一些特征性临床表型相关,如抗转录中介因子1γ抗体阳性的患者发生银屑病样损害、手掌角化过度性丘疹明显增加,而抗核基质蛋白2抗体阳性的患者发生前臂、小腿和颈部无力更为常见.抗核小体重塑和组蛋白脱乙酰酶蛋白复合物抗体阳性患者发生间质性肺病风险和维持过长时间治疗的风险明显降低.因此临床上广泛地开展这些自身抗体的检测有助于指导皮肌炎的临床诊疗.     

无肌病性皮肌炎一例

患者,女,21岁。面部、躯干、四肢皮肤红斑、丘疹伴脱发6年,皮损基本损害为红斑,双眼睑见明显水肿性紫红斑,其他部位皮损为弥漫性对称性暗紫红色斑,双手可见Gottron丘疹。肌力正常。皮损组织病理检查：表皮轻度角化过度,基底膜增厚,真皮浅层水肿,血管周围单一核细胞浸润。诊断：无肌病性皮肌炎。给予泼尼松联合甲氨蝶呤治疗2个月后皮损明显好转。     

皮肌炎的早期诊断和内脏损伤早期识别

皮肌炎是一种临床常见的自身免疫性疾病, 常有肌肉和肺的累及, 也常合并恶性肿瘤, 肺累及常是致命性的。因此, 早期诊断及治疗, 对减少肌肉和肺的损伤、识别和诊治合并的恶性肿瘤、提高患者预后和生存率有极大帮助。皮肌炎的异质性强, 临床表现多种多样, 本文论述皮肌炎合并急性肺间质性纤维化、发生吞咽困难、合并恶性肿瘤的高风险因素, 总结出"皮疹+甲皱血管+肌炎抗体"的联合模式有助于早期诊断皮肌炎, 早期识别重要内脏损害风险和肿瘤, 早期干预, 提高患者的整体生存率。     

Dermatomiositis paraneoplásica: estudio de 12 casos

Introduction and objectivesAdult dermatomyositis presents as a paraneoplastic syndrome in up to 25% of cases, but no clinical, histologic, or laboratory markers completely specific for paraneoplastic disease in dermatomyositis have been identified to date. Furthermore, studies on adult dermatomyositis do not usually report the frequency of cutaneous features of dermatomyositis in patients with associated cancer. Our aim was to review the characteristics of paraneoplastic dermatomyositis in patients seen at our hospital.Material and methodsWe studied 12 cases of paraneoplastic dermatomyositis and recorded patient age and sex, associated cancer, time between onset of dermatomyositis and cancer, emergent cutaneous manifestations, muscle involvement, dysphagia, lung disease, and levels of creatine phosphokinase and circulating autoantibodies.ResultsThe mean age of the patients was 61 years and the 2 most common malignancies were ovarian cancer and bladder cancer. The mean time between the diagnosis of cancer and dermatomyositis was 7 months and in most cases, the cancer was diagnosed first. Seven patients had amyopathic dermatomyositis. The most common cutaneous signs were a violaceous photodistributed rash sparing the interscapular area and a heliotrope rash, followed by Gottron papules and cuticle involvement. Superficial cutaneous necrosis was observed in 3 cases. Myositis-specific autoantibodies were not detected in any of the 6 patients who underwent this test.ConclusionsParaneoplastic dermatomyositis is often amyopathic. There are no specific cutaneous markers for malignancy in dermatomyositis. Myositis-specific antibodies are not associated with paraneoplastic dermatomyositis.     

Juvenile dermatomyositis: a retrospective review of a 30-year experience.

BACKGROUND: Children with juvenile dermatomyositis (JDMS) have variable initial presentations. OBJECTIVE: Our purpose was to evaluate the epidemiology trends, presenting clinical features, laboratory data, and outcome of patients with JDMS. METHODS: A total of 16 patients were identified at Geisinger Medical Center by a 30-year retrospective chart review. RESULTS: Sex ratio, age at diagnosis, and outcome were similar to data published in previous studies. However, certain trends were noted. The most common initial physical examination findings were an extremity rash (94%) and periungual erythema (75%). New associations of JDMS that were uncovered included the findings of pruritus (38%) and a psoriasiform scalp dermatitis (25%). Nonspecific laboratory elevations were the most common initial laboratory changes (erythrocyte sedimentation rate, lactate dehydrogenase, and aspartate aminotransferase). Tubuloreticular inclusions as found on electron microscopy of muscle biopsy specimens were present in all 3 patients tested. One patient with tubuloreticular inclusions had otherwise normal muscle biopsy findings on hematoxylin-and-eosin staining. Two of the 16 patients had cutaneous findings of JDMS but did not exhibit muscle involvement after long-term follow-up at 4 and 5 years. CONCLUSION: Our study confirms that the initial physical and laboratory findings in patients with JDMS may be nonspecific. The heliotrope rash and Gottron papules classically associated with dermatomyositis appeared less commonly than an extremity rash and periungual erythema. Creatinine kinase and aldolase levels may not be elevated on initial presentation. Pruritus, a psoriasiform scalp dermatitis, and tubuloreticular inclusions found on muscle biopsy electron microscopy should be additional factors to consider. The long-term follow-up in 2 patients without muscle involvement lends support to the existence of amyopathic dermatomyositis.     

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.     

Predicting factors of interstitial lung disease in dermatomyositis and polymyositis

The aim of this study was to define the predicting factors and evaluate the prognosis of interstitial lung disease in dermatomyositis/polymyositis. For the period 1995-2005, we retrospectively reviewed the clinical information and laboratory data of 56 patients who were diagnosed as definite and probable dermatomyositis and polymyositis. Interstitial lung disease is common (41.9%) in these patients. Dyspnoea and cough were the two most common initial presentations. Anti-Jo1 antibody was more common in those with interstitial lung disease. Univariate and multivariate analyses identified primary idiopathic dermatomyositis subtype, cough and dyspnoea at onset to be the three independent clinical predicting factors of interstitial lung disease. High serum lactate dehydrogenase level (>400 U/l) was inversely associated with development of interstitial lung disease (OR 0.088, p=0.031). Serum lactate dehydrogenase level and presence of anti-Jo1 antibody can serve as laboratory indicators of lung complications. Patients with malignancy and older age at onset (more than 60 years) had poorer prognosis for dermatomyositis/polymyositis (p=0.047 and p=0.035, respectively). Interstitial lung disease did not affect the survival of dermatomyositis/polymyositis patients.