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1.
We describe a 38-year-old man who presented with proximal muscle weakness, myalgia, polyarthralgia, and skin rash and was diagnosed as having dermatomyositis (DM). The patient's symptoms improved with prednisolone therapy. However, myopathy relapsed and pneumomediastinum with subcutaneous emphysema developed. Pneumomediastinum with subcutaneous emphysema rapidly disappeared by the administration of ciclosporin. We reviewed the present case and previously reported cases regarding the clinical characteristics. All of the reported death cases were accompanied by interstitial lung disease (ILD). Although it has been reported that pneumomediastinum in DM can be fatal, the direct cause of patient's death was due to respiratory failure resulting from progressive ILD. Pneumomediastinum without ILD shows a good prognosis. 相似文献
2.
Dermatomyositis (DM) is rare during the reproductive period, but when it does occur most reports have noted that it has an
adverse effect on fetal outcome. Conversely, there is little information concerning the contribution of pregnancy to the development
and course of DM. We describe here a patient with DM that developed after delivery of an infant and summarize previously documented
cases of postpartum-onset DM. This case suggests that pregnancy could be a trigger for the development of DM.
Received: January 21, 2002 / Accepted: May 23, 2002
Correspondence to:W.H. Yoo 相似文献
3.
We report a rare case of patient with dermatomyositis (DM) who developed spontaneous pneumomediastinum (PnM) and subcutaneous
emphysema. She was successfully treated with oral prednisolone and cyclosporine A (CsA). We reviewed the cases of PnM in patients
with DM treated with CsA. A review of four previously reported cases revealed that treatment with systemic glucocorticoid
and CsA was effective for the DM and PnM. We indicate that initial and early treatment of the patients with DM and PnM with
CsA enabled rapid tapering of the dose of glucocorticoid and improved the disease. 相似文献
4.
Terao M Ozawa K Inui S Murota H Yokomi A Itami S 《Modern rheumatology / the Japan Rheumatism Association》2007,17(2):156-159
We herein describe a 16-year-old boy with pneumomediastinum, pneumothorax, and subcutaneous emphysema as the initial symptoms
of dermatomyositis (DM). His pneumomediastinum disappeared after strict bed rest and he was thereafter successfully treated
with oral prednisolone and cyclosporine A. His condition was further complicated with mild interstitial lung disease, arrhythmia,
and skin ulcers on his fingertips, right elbow, ear, and sacral region. Pneumomediastinum is a rare complication of DM and
its pathogenesis remains unclear. We review the literature and discuss the possible mechanism of this disease. 相似文献
5.
A case of dermatomyositis complicated with pneumomediastinum successfully treated with cyclosporin A
Kuroda T Morikawa H Satou T Tanabe Y Murakami S Ito S Nakan M Gejyo F 《Clinical rheumatology》2003,22(1):45-48
We describe a rare case of a 46-year-old Japanese man with dermatomyositis (DM) and interstitial lung disease who developed
spontaneous pneumomediastinum and subcutaneous emphysema. Relatively mild myositis, mild elevation of CK values and the absence
of anti-Jo-1 antibody were observed and the case was similar to amyopathic DM. Treatment of this patient with oral prednisolone
and cyclosporin A (CsA) was effective for the myositis and interstitial lung disease. The administration of CsA enabled rapid
tapering of the dose of prednisolone without aggravating the disease. Pneumomediastinum and subcutaneous emphysema disappeared
5 months later without recurrence. The serum levels of KL-6 were monitored every 2 weeks to help determine whether this may
have contributed to the recurrence of interstitial pneumonitis. This is a rare case of pneumomediastinum in a patient with
DM.
Received: 6 May 2002 / Accepted: 26 August 2002 相似文献
6.
《Modern rheumatology / the Japan Rheumatism Association》2013,23(2):156-159
AbstractWe herein describe a 16-year-old boy with pneumomediastinum, pneumothorax, and subcutaneous emphysema as the initial symptoms of dermatomyositis (DM). His pneumomediastinum disappeared after strict bed rest and he was thereafter successfully treated with oral prednisolone and cyclosporine A. His condition was further complicated with mild interstitial lung disease, arrhythmia, and skin ulcers on his fingertips, right elbow, ear, and sacral region. Pneumomediastinum is a rare complication of DM and its pathogenesis remains unclear. We review the literature and discuss the possible mechanism of this disease. 相似文献
7.
《Modern rheumatology / the Japan Rheumatism Association》2013,23(4):362-365
AbstractDermatomyositis (DM) is rare during the reproductive period, but when it does occur most reports have noted that it has an adverse effect on fetal outcome. Conversely, there is little information concerning the contribution of pregnancy to the development and course of DM. We describe here a patient with DM that developed after delivery of an infant and summarize previously documented cases of postpartum-onset DM. This case suggests that pregnancy could be a trigger for the development of DM. 相似文献
8.
Neves Fde S de Souza Neves F Shinjo SK Carvalho JF Levy-Neto M Borges CT 《Clinical rheumatology》2007,26(1):138-107
One patient with dermatomyositis (DM) who developed spontaneous pneumomediastinum (SPnM) is described. A review of 15 previously
reported cases shows that this is a complication of DM but not of polymyositis. It occurs in young patients with recent onset
of disease characterized by cutaneous vasculopathy, and patients treated with immunosuppressive drugs have, in general, better
outcomes. We suggest that the onset of SPnM in DM patients must alert to possible vasculitic activity and that immunosuppressive
agents with progressive tapering of corticosteroids are part of the most reasonable approach in these cases.
An erratum to this article can be found at 相似文献
9.
肿瘤相关皮肌炎合并多发神经根神经病一例并文献复习 总被引:1,自引:0,他引:1
目的 探讨肿瘤相关皮肌炎与其他神经系统副肿瘤综合征的区别及诊治.方法 分析1例肿瘤相关皮肌炎同时合并多发神经根神经病患者的临床特点,并复习相关文献.结果 本患者皮肌炎临床表现典型,同时发现合并肺癌,经手术及放化疗后皮肌炎缓解,4年后出现神经系统副肿瘤综合征.皮肌炎与多发神经根神经病在临床表现上具有相似之处,罕见情况下可同时出现在恶性肿瘤患者身上,需详加鉴别.肿瘤相关皮肌炎与神经系统副肿瘤综合征的发病机制相似,治疗均以控制肿瘤为主,且均可使用免疫治疗.结论 皮肌炎患者需警惕恶性肿瘤.肿瘤可同时合并多种副肿瘤综合征,不能只满足于某一个诊断.Abstract: Objective To investigate the difference and treatment strategy of malignancy-associated dermatomyositis and other para-neoplastic neurological syndromes (PNS).Methods The clinical characteristics of a patient with malignancy-associated dermatomyositis and poly radiculoneuropathy was reported and the relevant literature was reviewed.Results Patients with dermatomyositis had increased risk of malignancies,and should routinely screened.Dermatomyositis and polyradiculoneuropathy was clinically similar,but could rarely be seen in the same malignant patient.Malignancy -associated dermatomyositis and PNS had similar pathogenesis.The treatment strategy of both was similar.Malignancy specific treatment should be initiated and immune suppressive agents should be prescribed concurrently.Conclusion Rheumatolgists should aware the association between dermatomyositis and potential underlying malignancies.Multiple para-neoplastic syndromes could be seen in the same patient,but the diagnosis should be considered as one. 相似文献
10.
以急进性肺间质病变为突出表现的无肌炎的皮肌炎 总被引:3,自引:0,他引:3
目的了解以急进性肺间质病变为突出表现的临床无肌炎的皮肌炎的临床特点转归和治疗。方法回顾分析本院1998—2005年住院的成人特发性皮肌炎/多发性肌炎(DM/PM)202例,按临床无肌炎的皮肌炎(CADM)、典型DM、PM进行分组,有无合并临床肺间质病变(ILD)进行分层。对随访的145例DM/PM行生存分析和COX回归,分析预后和危险因素。横断面研究用Logistic回归分析ILD相关的预测因子。结果①存在临床ILD是DM/PM预后的最重要危险因素,OR为4.826(95%CI为1.60~14.56.P=0.005);而亚临床ILD与无ILD的DM/PM生存曲线差异无统计学意义。②CADM、典型DM、PM之合并临床ILD存在不同的病情经过。CADM-ILD呈急进性ILD的临床模式,其6个月存活率仅为40.8%,中位数存活时间仅为10.2个月;而典型的DM-ILD呈现“次急进性”(sub-rapid progressive)的临床模式,5年存活率为54.0%,中位数存活时间为90个月;PM-ILD则为慢性经过,5年和10年存活率分别为72.4%和60.3%,中位数存活时间可达10年,部分DM-ILD和个别PM-ILD患者也可呈现急进性ILD的疾病模式。③Logistic回归显示:四肢近端肌力OR 3.374(95%CI 1.729~6.583,P<0.01)和血沉OR 1.025 (95%CI 1.002~1.049,P=0.032)与临床ILD呈正相关,血白蛋白水平OR 0.877(95? 0.792~0.970,P= 0.011)则与临床ILD呈负相关。④随访CADM(28例)的病程为3~80个月,其中CADM-ILD超过半数在1年内死亡(12/21);而病程>24个月的CADM(8/28例)预后良好。对于无ILD的CADM,主要予中小剂量的激素(<0.5~1mg·kg~(-1)·d~(-1))治疗,而CADM-ILD则多接受较大剂量的激素(≥1~2mg·kg~(-1)·d~(-1)),且多联用包括硫唑嘌呤、环磷酰胺、环孢素、霉酚酸酯在内的细胞毒药物,但对呈急进性ILD者缺乏疗效。⑤DM/PM随访病例的病死率为24.8%(36/145例),ILD及其并发症作为直接死因的占69.4%(25/36例),与ILD无直接相关的感染占25.0%(9/36例)。纵隔(皮下)气肿,气胸发生率占所有DM/PM-ILD的9.3%(8/86例),也是提示预后不良指征之一。结论ILD是DM/PM的常见并发症,也是最重要危险因素。本文提出了一个新的DM/PM疾病分类模式:即DM/PM是皮损、肌炎和ILD三个维度上相组合的立体疾病谱。CADM是该疾病谱的组成部分,CADM-ILD可呈急进性,是疾病谱中特殊的临床表型。 相似文献
11.
Ye S Chen XX Lu XY Wu MF Deng Y Huang WQ Guo Q Yang CD Gu YY Bao CD Chen SL 《Clinical rheumatology》2007,26(10):1647-1654
The aim of the study was to investigate the characteristics of adult clinically amyopathic dermatomyositis (CADM) with rapid progressive interstitial lung disease (ILD). Hospitalized patients with dermatomyositis (DM) and polymyositis (PM) between 1998 and 2005 in the Shanghai Renji Hospital were retrospectively studied. One hundred and forty-five patients were classified into CADM, classic DM or PM according to the modified Sontheimer's definition or Bohan-Peter's classification criteria. They were further stratified based on the presence or absence of clinical ILD. The Kaplan-Meier survival analysis and COX regression were performed. The predictive factors for ILD and other clinical properties of CADM-ILD were explored. The presence of clinical ILD was a significant risk factor for the poor outcome of DM/PM (OR = 4.237, CI 95%: 1.239-14.49, p = 0.021). Other risk factors are the presence of rashes and elevated urea nitrogen. Patients with DM/PM complicated by ILD had different clinical courses. Patients with CADM-ILD showed a rapidly progressive pattern with 6-month survival rate of 40.8%. The DM-ILD manifested a progressive pattern with a 5-year survival rate of 54%, while PM-ILD was chronic with 5- and 10-year survival rate of 72.4% and 60.3%, respectively. Better preserved muscle strength, elevated erythrocyte sedimentation rate, and hypoalbuminemia may herald ILD in DM/PM. Patients with CADM-ILD who later died had lower PO(2), higher lactate dehydrogenase, and prominent arthritis/arthralgia compared with those who survived. The presence of antinuclear antibody seems to be protective. Rapid progressive CADM-ILD is refractory to conventional treatment. ILD is a common complication in over 40% of our hospitalized DM/PM cohort and is also a prominent prognostic indicator. CADM is a special phenotype of DM/PM. CADM-ILD, which is usually rapidly progressive and fatal, requires further investigation. 相似文献
12.
A 77-year-old man developed pneumonitis while on chlorambucil therapy for chronic lymphocytic leukemia, with a cumulative
dose of 2700 mg. The condition improved promptly with the discontinuation of the drug and initiation of steroids. A case report
and review of the literature are presented in this paper.
Received: September 9, 1997 / Accepted: March 31, 1998 相似文献
13.
I-Jung Chen Yeong-Jian Jan Wu Cho-Wei Lin Kang-Wei Fan Shue-Fen Luo Huei-Huang Ho Lieh-Bang Liou Wen-Pin Tsai Ji-Yih Chen Chung-Han Yang Chang-Fu Kuo Kuang-Hui Yu 《Clinical rheumatology》2009,28(6):639-646
The aim of the study was to estimate the prevalence, characteristics, and prognostic factors of interstitial lung disease
(ILD) in patients with polymyositis (PM) and dermatomyositis (DM). The medical records of 151 PM/DM patients treated at Chang
Gung Memorial Hospital between January, 2000 and June, 2007 were retrospectively reviewed. Thirty of 151 (19.9%) PM/DM patients
had developed ILD. Older age at PM/DM onset, anti-Jo-1 antibody, and arthritis/arthralgia were associated with the presence
of ILD (p = 0.004, p = 0.008, and p = 0.026, respectively). Anti-Jo-1 was initially excluded from the multivariate analysis because only 80 patients underwent
the test. An older age at onset above 45 years (odds ratio 3.28, 95% confidence interval (CI) 1.15–9.34, p = 0.026) and arthritis/arthralgia at onset (odds ratio (OR) 2.57, 95% CI 1.09–6.08, p = 0.032) were the two independent risk factors for developing ILD. If anti-Jo-1 was included in the multivariate analysis
(n = 80), then an older age at onset above 45 years (OR 7.30, 95% CI 1.70–31.40, p = 0.008) and anti-Jo-1 positive (OR 7.89, 95% CI 1.18–52.87, p = 0.033) were associated with ILD, while arthritis/arthralgia was no longer significant (OR 2.64, 95% CI 0.70–10.01, p = 0.153). Of the 30 ILD patients, 16 (53.3%) died. The survival time was significantly shorter in ILD patients than in patients
without ILD (p < 0.001). Poor survival in ILD patients was associated with male gender (p = 0.039), a Hamman–Rich-like presentation (p = 0.039), and a clinical diagnosis of acute interstitial pneumonia (p = 0.007). 相似文献
14.
Two patients with rheumatoid arthritis and empyematous pleural effusion were treated with repeated drainage and intrapleural
corticosteroids. One patient with active joint disease improved within 3 months without sequelae, probably because of the
systemic therapy. The other patient, with non-active joint disease, had persistent pleural effusion which resulted in pleural
thickening and symptomatic restrictive disturbance. It appears that early intervention intended to prevent the accumulation
of empyematous pleural effusion could also prevent pleural thickening and fibrosis. Therapeutic options are discussed.
Received: 3 August 2000 / Accepted: 9 September 2001 相似文献
15.
Altiparmak MR Pamuk ON Pamuk GE Apaydin S Ataman R Serdengeçti K 《Clinical rheumatology》2002,21(6):497-500
Familial Mediterranean fever (FMF) is a hereditary disease, the most threatening complication of which is systemic amyloidosis. The thyroid gland may be asymptomatically involved in most patients with systemic amyloidosis secondary to FMF. However, clinically detectable thyroid goitre is quite rare, and until now only nine cases of thyroid goitre secondary to amyloid deposition in FMF have been reported. Of 1,100 FMF patients regularly followed up at our centre, thyroid goitre due to the accumulation of amyloid substance could be detected in only three (0.27%). In this report, we summarise the clinical and laboratory features of these patients. All three patients were euthyroid. Total thyroidectomy was performed for compressive symptoms in one patient and for aesthetic purposes in the other two. In countries with a high prevalence of FMF, such as Turkey, secondary amyloidosis of the thyroid gland should be borne in mind in long-standing FMF patients. 相似文献
16.
A 58-year-old woman presented with inflammatory demyelinating polyradiculoneuropathy accompanied by sensory and motor disturbance
and interstitial lung disease. Corticosteroid therapy led to a marked amelioration of both the neuropathy and the lung disease.
We suggest that a demyelinating neuropathy is associated with an interstitial lung disease.
Received: 20 April 1999 / Accepted: 1 December 1999 相似文献
17.
Regional migratory osteoporosis (RMO) is a migrating arthralgia of the weight-bearing joints of the lower limb which mainly
affects middle-aged males. Its aetiology is unknown. The association of RMO with generalised osteoporosis has recently been
reported. A concurrent systemic osteoporosis was also reported in some cases of transient osteoporosis of the hip (TOH), a
disorder closely related to RMO. In its turn, TOH is considered a reversible stage of avascular necrosis of the hip (AVN),
and the aetiopathogenesis of both of them remains strongly debated. We report three cases of RMO associated with generalised
severe idiopathic osteoporosis. Three men, in the fourth and fifth decades of life, complained of at least four episodes of
arthralgia in the lower limbs, with a migratory pattern, radiographic focal osteoporosis and final clinical resolution. The
most striking common feature of these patients was the presence of a severe systemic osteoporosis with a prevailing trabecular
involvement. We suggest that a prolonged or exaggerated activation of regional acceleratory phenomena (RAP) is the cause of
transient osteoporosis. Bone tissue microdamage due to osteoporosis may be the most frequent noxious stimulus that turns RAP
on, and, bone tissue microfracture is the most prevalent consequence. When this pathogenetic pathway is activated, the progression
from focal osteoporosis and bone marrow oedema to avascular necrosis is associated with the amount of structural damage.
Received: 17 February 2002 / Accepted: 21 May 2002 相似文献
18.
Yamaguchi Koichi Yamaguchi Aya Uchida Megumi Kono Shunichi Suzuki Masafumi Masubuchi Hiroaki 《Modern rheumatology / the Japan Rheumatism Association》2017,27(3):536-540
Rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) associated with antibodies to melanoma differentiation-associated gene5 (MDA5) results in a high mortality rate.
We experienced a case of anti-MDA5-positive RP-ILD of CADM which showed a response to rituximab, although there was no significant effect due to standard immunosuppressive treatment. This case suggests that rituximab has the potential to offer an effective agent for the treatment of anti-MDA5-positive RP-ILD of CADM. 相似文献
19.
目的 探讨幼年皮肌炎的发病原因、诊治方法及预后.方法 分析我院35例幼年皮肌炎的临床特点、病理、实验室检查、诊治过程及预后情况,并结合文献复习.结果 幼年皮肌炎合并肿瘤及引起重要内脏器官损伤少见.治疗首选激素,疗效明显.对于病情控制不理想或泼尼松减量困难的病例,可加用免疫抑制剂.4例重者加用静脉注射人免疫球蛋白(IVIG)等.35例患儿中25例痊愈,10例有效,后者经维持治疗,最终停药.一般用药时间为2~3年或更长.8例患儿在停药后1~4年病情反复,再给予治疗,仍有效.本组患儿酶学指标在治疗3~5周后降低或正常,肌力在1~6个月(平均2.6个月)恢复,肌力恢复从4~5级需1.5~3个月,从3~5级需6~12个月.结论 幼年皮肌炎早期诊断、早期治疗,预后良好. 相似文献
20.
Osteopoikilosis is an uncommon, benign sclerosing bone dysplasia characterised by typical roentgenographic findings and usually
seen in patients with dermatological problems. We report a case of osteopoikilosis and discoid lupus erythematosus presenting
with skin and mucosal involvement, an association that has never previously been reported. We also discuss the differential
diagnosis and the clinical pathologies accompanying osteopoikilosis in the literature.
Received: 30 July 2001 / Accepted: 11 February 2002 相似文献