A case of dermatomyositis complicated with pneumomediastinum

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.     

A case of dermatomyositis complicated with pneumomediastinum successfully treated with cyclosporin A

 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     

Dermatomyositis, complicated with pneumomediastinum, successfully treated with cyclosporine A: a case report and review of literature

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.     

Persistent pneumomediastinum and dermatomyositis: a case report and review of the literature

We describe a 42-year-old man with dermatomyositis and interstitial lung disease who presented with anterior neck pain and dyspnoea. Chest radiographs showed subcutaneous emphysema, pneumomediastinum and diffuse reticulonodular infiltration in both lungs. After the administration of high doses of prednisolone, an improvement of pulmonary function and respiratory symptoms was observed but the pneumomediastinum persists 12 months after diagnosis, and without any complication. We review the cases that have been reported thus far of pneumomediastinum associated with dermatomyositis and discuss the possible mechanisms involved. We conclude that pneumomediastinum is not an uncommon complication of dermatomyositis and that its aetiopathogenesis remains very unclear. Received: 26 July 2000 / Accepted: 14 March 2001     

A case of polymyositis complicated with interstitial pneumonitis and pneumomediastinum

Pneumomediastinum as a complication of interstitial pneumonia with leakage of air into the mediastinum or subcutaneous tissues is a rare complication of dermatomyositis (DM). Herein we report a case of pneumomediastinum complicating polymyositis (PM), which is usually associated with DM. A 61-year-old man was hospitalized in our department because of deterioration of interstitial pneumonia. Treatment with high-dose corticosteroid and cyclosporin A steadily improved his interstitial pneumonia. Two weeks later, he developed subcutaneous emphysema and chest X-ray showed pneumomediastinum. Both subcutaneous emphysema and pneumomediastinum improved gradually without any additional treatment.     

Pneumomediastinum and subcutaneous emphysema secondary to amyopathic dermatomyositis with cryptogenic organizing pneumonia in invasive breast cancer: a case report and review of literature

Amyopathic dermatomyositis (ADM) is recognized as a variant phenotype of dermatomyositis and characterized by typical skin manifestations without evidence of muscular inflammation. While interstitial lung disease (ILD) is occasionally found as one of the lung manifestations in ADM patients, the development of a pneumomediastinum and/or subcutaneous emphysema in this disease entity is one of the extremely rare pulmonary complications. These latter complicated pulmonary manifestations have been usually reported in idiopathic ADM with ILD without any other associated medical conditions. We report a case presented with the spontaneous pneumomediastinum and subcutaneous emphysema in both ADM and cryptogenic organizing pneumonia during adjuvant chemotherapy based on cyclophosphamide for breast cancer.     

Spontaneous pneumomediastinum and dermatomyositis may be a not so rare association: report of a case and review of the literature

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     

Subcutaneous cervical emphysema and pneumomediastinum due to a lower gastrointestinal tract perforation

This case report describes a 69-year-old man presenting with an extensive subcutaneous emphysema in his neck and generalized peritonitis caused by a lower gastrointestinal tract perforation. This case emphasizes that subcutaneous emphysema patients with negative thoracic findings should be scrutinized for signs of retroperitoneal hollow viscus perforation.     

Clinical experience of spontaneous pneumomediastinum: diagnosis and treatment

Background

Spontaneous pneumomediastinum (SPM) is a benign disease with a variety degree of severity but definite treatment modality is not clearly identified with its rarity. The purpose of this study was to review our experience and discuss the management of SPM according to the severity of disease.

Methods

From March 1996 to December 2012, total 64 patients were enrolled and classified as mild, moderate and severe groups and subsequent clinical courses were analyzed retrospectively.

Results

Fifty-one were males and 13 were females (M:F =3.9:1) with a mean age of 18 years old (range: 10-30 years old). Thirty-six patients were in mild, 22 in moderate and 6 in severe group. Chief complaints were chest pain (50 cases; 78.1%), neck pain (35 cases; 54.7%), dyspnea (18 cases; 28.1%), odynophagia (9 cases; 14.1%) and precipitating factors were coughing in 12 cases, feeding problems in 9 cases, and vomiting in 7 cases; however, 34 patients (53.1%) had no precipitating signs. All patients received oxygen therapy (100%), prophylactic antibiotics in 57 patients (89.1%), and pain medications in 47 patients (73.4%). The mean hospital stay was 4.6 days (range: 1-10 days). There was an increased linear trend according to time to visit (P=0.023) but clinical course demonstrated no significant trend between groups.

Conclusions

These data demonstrated that there was no difference in symptom, clinical course and SPM was adequately treated with conservative management regardless of the degree of severity of SPM.     

Severe subcutaneous generalized edema in a patient with dermatomyositis

Subcutaneous generalized edema associated with dermatomyositis (DM)/polymyositis (PM) is extremely rare. Herein we report a case of severe subcutaneous generalized edema complicating DM. A 78-year-old woman was hospitalized in our department because of massive edema in the four limbs. Elevated muscle enzymes, heliotrope rash, results of electromyography, and muscle biopsy confirmed the diagnosis of DM. The absence of other diseases that could cause the symptoms indicated that massive edema was correlated with the pathophysiology of DM. Although myopathy and edema responded well to oral prednisolone, dysphagia persisted. We conclude that subcutaneous generalized edema can occur during the course of DM/PM, and subcutaneous vasculopathy may be involved in the pathogenesis of DM/PM.     

Severe subcutaneous generalized edema in a patient with dermatomyositis

Abstract

Subcutaneous generalized edema associated with dermatomyositis (DM)/polymyositis (PM) is extremely rare. Herein we report a case of severe subcutaneous generalized edema complicating DM. A 78-year-old woman was hospitalized in our department because of massive edema in the four limbs. Elevated muscle enzymes, heliotrope rash, results of electromyography, and muscle biopsy confirmed the diagnosis of DM. The absence of other diseases that could cause the symptoms indicated that massive edema was correlated with the pathophysiology of DM. Although myopathy and edema responded well to oral prednisolone, dysphagia persisted. We conclude that subcutaneous generalized edema can occur during the course of DM/PM, and subcutaneous vasculopathy may be involved in the pathogenesis of DM/PM.     

皮肌炎合并纵隔、皮下气肿临床分析

目的:总结皮肌炎(DM)合并纵隔、皮下气肿的临床相关因素,提高对该并发症的认识。方法:回顾性分析7例皮肌炎患者并发纵隔、皮下气肿的临床特征、治疗及转归。结果:7例患者均存在不同程度的肺间质病变,较明显的皮肤血管炎表现,均合并感染;血肌酸激酶(CK)平均(88±30)U/L,而乳酸脱氢酶(LDH)平均(482±128)U/L、羟丁酸脱氢酶(HBDH)平均(417±164)U/L;2例男性、1例女性死亡,4例存活;均未行大剂量激素冲击治疗,均联合环孢素A或他克莫司等免疫抑制剂治疗。结论:DM患者中肺间质病变和皮肤血管炎可能是并发纵膈、皮下气肿的高危因素,感染亦是引发因素;男性患者较多,Gottron疹、甲周红斑、指端溃疡较突出,肌炎较轻,而LDH、HBDH升高;不推荐大剂量激素冲击治疗,联合钙调磷酸酶抑制剂(CNIs),预后较好。     

A case of dermatomyositis complicated with pneumomediastinum that was successfully treated with cyclosporin A]

We reported the case of a 39-year-old man with dermatomyositis (DM) complicated with subcutaneous emphysema and pneumomediastinum during steroid therapy. The patient had complained of muscle weakness, dyspnea and skin eruption on his anterior chest wall 6 months prior to admission. He was diagnosed as having DM on the basis of an elevation in myogenic enzymes, myogenic changes in electromyography, a skin biopsy and a muscle biopsy. Chest roentgenogram revealed interstitial pneumonia (IP) in the lower lobes of the lungs. The administration of prednisolone (60 mg/day) was initiated, which resulted in improvement of DM. Fifteen days after the initiation of the steroid therapy, the patient developed subcutaneous emphysema and pneumomediastinum. Additional administration of cyclosporin A (CsA) enabled us to rapidly taper the dose of prednisolone without aggravating the diseases. Several reports have shown that vasculitis might be involved in the pathogenesis of pneumomediastinum in DM patients. Infection and tissue fragility due to steroid therapy worsen the outcome of those patients. CsA therapy may improve the outcome through the anti-vasculitic- and steroid sparing-effects.     

慢性乙型肝炎合并重症皮肌炎1例报道

患者，男，42岁。四肢肌肉痛2周加重伴皮疹12天。2周前出现四肢肌肉疼痛，以近端肌肉为著，活动后疼痛加剧，同时伴乏力、纳差，在家自服“活血通络丸”无明显好转，在西安交大二院就诊，做腰椎CT及肌电图均正常。12天前受凉后自觉四肢肌肉疼痛加重，且发现颈、胸部出现大片暗红色皮疹，无瘙痒感，同时伴有咳嗽，咳少许白色黏痰。在私人诊所静滴鱼腥草及抗菌素约1周左右，咳嗽好转，但肌肉痛及皮疹无明显好转。2天前到军工医院化验乙肝系列HBsAg（＋）、HBcAb（＋），肝功：ALT107U／L、AST421U／L。为进一步诊治，遂来我院并收住我科。家族中母亲因乙肝肝硬化并发症而病故。查体T36．3℃，     

Pneumomediastinum in dermatomyositis itself is not a poor prognostic factor: report of a case and review of the literature

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.     

皮肌炎合并颅内肿瘤12例分析

目的提高对皮肌炎(DM)合并颅内肿瘤的认识,探讨其发病机制,总结诊断和治疗此类病例的经验.方法对12例DM合并颅内肿瘤的病例做回顾性分析,并复习23年相关文献.结果p53基因种系突变与DM合并颅内肿瘤有关.DM合并颅内肿瘤相对少见是由于:①脑组织有血脑屏障:②脑组织中淋巴细胞较少,免疫应答及免疫交叉的概率相对较少.由于DM有比较典型的临床表现,结合实验室检查,一般能明确诊断,如在发病或治疗过程中出现了头痛、呕吐、视力减退等高颅压症状,出现了偏瘫、失语等定位症状可做头颅CT或磁共振成像(MRI)即可明确.治疗首选类固醇激素,同时处理肿瘤十分必要.结论DM合并颅内肿瘤是一种少见疾病,应及时明确诊断,恰当选择治疗措施.     

Characteristics and prognosis of interstitial pneumonias complicated with pneumomediastinum

BackgroundThe clinical characteristics and prognostic impact of complication with pneumomediastinum in patients with interstitial pneumonias (IPs) are not well studied due to the relatively limited nature of available reports. The purpose of this study was to clarify the characteristics and prognostic factors of IPs complicated with pneumomediastinum.MethodsConsecutive patients with IPs complicated with pneumomediastinum detected by computed tomography (CT) between July 1, 2011, and April 30, 2014 were retrospectively reviewed. Clinical data including symptoms associated with pneumomediastinum, laboratory data, lung function tests, treatments, and mortality were collected from medical records.ResultsForty-five patients (25 males, 20 females), including 32 with idiopathic IP (IIPs) and 13 connective tissue disease-associated interstitial lung diseases (CTD-ILDs) were identified. The median age of onset of pneumomediastinum was 72 years (interquartile range [IQR] 68–79 years). The most common symptom associated with occurrence of pneumomediastinum was appearance or worsening of dyspnoea. No specific treatment was performed for most (84%) of the cases. The median period between occurrence and improvement of pneumomediastinum was 29 days (IQR 5–69 days). Multivariate analysis revealed that IIPs and no improvement of pneumomediastinum were associated with poor prognosis.ConclusionsPatients with IIPs complicated with pneumomediastinum and those without improvement of pneumomediastinum had poor prognosis.     

An autopsy case with Good's syndrome and dermatomyositis

Summary A 31-year-old male patient complaining of repeated respiratory infections was revealed to be in an immunodeficient state in both humoral and cellular immunity and accompanied by dermatomyositis which was proved by both clinical and histological findings. Persistent diarrhea followed by colonic bleeding due to multiple ulcers and a severe liver dysfunction seemed to bring him to his death. The autopsy revealed the presence of thymoma of spindel cell type, depleted cells in the systemic lymphatic systems and inflammatory lesions in the muscle, liver, lung, kidney and gastrointestinal tract. This is the first report of Good's syndrome accompanied by dermatomyositis.     

A case of dermatomyositis with rhabdomyolysis,rescued by intravenous immunoglobulin

Abstract

We describe a case of severe dermatomyositis (DM) complicated by rhabdomyolysis, acute tubular necrosis, and hemophagocytosis. The case failed to respond to corticosteroids, but showed rapid and significant improvement after the addition of intravenous immunoglobulin (IVIG). While the prognosis of DM is poor when it is complicated by rhabdomyolysis, the early administration of IVIG has the potential to be the cornerstone of its management.     

Clinical study of 10 cases of acute or subacute interstitial pneumonia associated with dermatomyositis

The prognosis for dermatomyositis (DM) with acute interstitial pneumonia (IP) is very poor. In the past 5 years, we have treated 10 DM patients with acute or subacute IP. Six cases were of acute-type IP, and 4 were of subacute-type IP. The treatment was a combination therapy of methylprednisolone (m-PSL) pulse therapy, cyclophosphamide (CPA) pulse therapy, oral cyclosporine A (CsA), and oral PSL. The outcome was 5 deaths and 5 survivals. All 5 cases of death had acute-type IP, four of which were complicated with pneumomediastinum, and these patients died within 40 days of IP onset. Furthermore, 4 of the 5 death cases were diagnosed with amyopathic DM, and one had hypomyopathic DM. The survivors comprised one case of acute-type IP with marked myositis, and 4 subacute cases. These results suggested that the prognosis for DM with IP might be dependent on the type of IP, the severity of the myositis, and the existence of pneumomediastinum. The rapid establishment of a more useful diagnostic technique and therapy for early-phase DM with acute IP is hoped for.