miR-125b, miR-200c Are Correlated with the Severity of Interstitial Lung Disease in Dermatomyositis/Polymyositis

Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM and 23 healthy controls were recruited into current study. Anti-JO-1, anti-SSA, muscle enzymes, the data of chest HRCT and pulmonary function test were collected. 9 consecutive DM/PM-ILD patients underwent bronchoalveolar lavage (BAL). TGF-β1 and surfactant protein D (SP-D) in BAL fluid (BALF) and plasma were detected by ELISA. miR-125b and miR-200c in PBMCs and bronchoalveolar cells were detected by QRT-PCR. All patients were classified into three groups: Mild or non-ILD group, moderate ILD group, and severe ILD group. The correlations between miRNAs and the severity of ILD, the lung damage markers, auto-antibodies, were analyzed. Results: The levels of miR-125b and miR-200c in bronchoalveolar cells were higher than in PBMCs, and the levels of TGF-β1 and SP-D were higher in BALF than in plasma in DM/PM-ILD patients. There were positive correlations between miR-125b, miR-200c in bronchoalveolar cells and SP-D in BALF. The levels of miR-125b and miR-200c in severe ILD group were higher than in mild or non-ILD and moderate ILD groups. There were negative correlations between miR-125b, miR-200c, and FEV1, and between miR-200c and DLCO. The patients with anti-JO-1 antibody had higher levels of miR-125b and miR-200c, and had more severe condition of ILD. Conclusion: miR-125b and miR-200c were positively correlated with the lung damage and severity of ILD in DM/PM, which could be important markers for judgement of disease condition in clinic.

Previous Pulmonary Fibrosis in Dermatomyositis/Polymyositis: A Predictive Factor for Pulmonary and Extra-Pulmonary Tuberculosis

Objective: With scant studies in the literature, little is known about the risk factors for tuberculosis in patients with dermatomyositis/polymyositis. Therefore, the aim of the present study was to analyze the predictive factors for tuberculosis development in dermatomyositis/polymyositis. Methods: This single-center, retrospective, cohort study initially included 290 patients with dermatomyositis/polymyositis, from 2002 to 2016. Tuberculosis (pulmonary and/or extra-pulmonary) was confirmed after dermatomyositis/polymyositis diagnosis in 12 patients (4.1%) (Tuberculosis+ group). For the control group (Tuberculosis−), 24 patients without tuberculosis were arbitrarily selected in the same period and matched for age, ethnicity, gender, age at disease diagnosis, disease duration and type (dermatomyositis or polymyositis). Results: Tuberculosis occurred for a median of 16 months after dermatomyositis/polymyositis diagnosis. Clinical, laboratory and treatment features were similar in Tuberculosis+ and Tuberculosis−groups (P > 0.05). However, previous pulmonary fibrosis in dermatomyositis/polymyositis was more prevalent in the Tuberculosis+ group (41.7 vs. 8.3%;P = 0.029). Moreover, on a multivariate logistic regression model, pulmonary fibrosis was significantly associated with Tuberculosis (Odds ratio: 9.59, 95% confidence interval: 1.17 - 78.82). Tuberculosis affected 3 dermatomyositis cases for every 1 polymyositis case, with predominantly pulmonary followed by extra-pulmonary involvement (pleura, cutaneous, muscular, joint, soft tissue and hematologic). Two or more sites were affected in 41.7% of cases. Conclusions: Previous pulmonary fibrosis in dermatomyositis/polymyositis was a predictive factor associated with tuberculosis development. Further studies are needed to confirm these results.

Anterior segment parameters associated with extramuscular manifestations in polymyositis and dermatomyositis

AIM:To evaluate detailed anterior segment parameters of patients with idiopathic inflammatory myopathies(IIM),including polymyositis(PM),and dermatomyositis(DM),and to clarify the associations between these data and clinical variables of IIM.METHODS:Totally 57 PM,41 DM patients and 62 controls were enrolled in this cross-sectional,observational,case-control study.All study participants underwent Pentacam evaluation.Laboratory investigations consisted of different antibody assays,while extramuscular clinical assessments included Raynaud’s phenomenon,dysphagia,interstitial lung disease,arthritis/arthralgia,and weight loss.Objective signs and subjective symptoms of dry eye disease(DED)were also evaluated.RESULTS:All pachymetric parameters[center,apex,thinnest and maximal keratometry(Kmax)]and corneal volume(CV)of both sides of PM patients proved to be significantly lower.Some pachymetric data were also noticed as significantly decreased compared to those of controls.Several significant differences were traced between anterior segment values and extramuscular manifestations of myositis,largely in case of arthritis/arthralgia and weight loss,whereas associations between anterior segment parameters and antibodies were weak.Objective clinical tests of DED were also significantly decreased in IIM patients.CONCLUSION:The results suggest that all IIM patients have thinner corneas compared with those of controls,and decreased corneal parameters are significantly associated with the occurrence of some extramuscular manifestations.In addition,IIM patients tend to develop objective signs of DED.

Dermatomyositis and polymyositis in total hip arthroplasty

BACKGROUND Idiopathic inflammatory myopathies(IIM)are systemic autoimmune disorders such as dermatomyositis(DM),polymyositis(PM),inclusion body myopathy,and autoimmune necrotizing myopathy that,similar to osteoarthritis,affect quality of life and activities of daily living.Moreover,these patients are often burdened with chronic pain and disability;however,the outcomes and risk of total hip arthroplasty(THA)in this patient population remain unclear.AIM To evaluate 90-d complications and costs in patients with these conditions.METHODS A retrospective case control study was designed by accessing data from the Medicare dataset available on the PearlDiver server.Patients with IIM,here,those with DM and PM were matched based on possible confounding variables to a cohort without these diseases and with the same 10-year risk of mortality as defined by the Charlson Comorbidity Index Score(CCI).Univariate and multivariate analysis were performed to evaluate complications and t-tests to evaluate 90-d Medicare reimbursements as markers of costs after THA.RESULTS The total sample was 1090 patients with each cohort comprised of 545.Females were 74.9%of the population.The mean CCI was 5.89(SD 2.11).Those with IIM had increased rates of pneumonia[odds ratio(OR)1.45,P<0.001]and pulmonary embolism(OR 1.46,P=0.035)and decreased hematoma risks(OR 0.58,P=0.00).90-d costs were on average$1411 greater for those with IIM yet not significantly different(P=0.034).CONCLUSION Patients with IIM have an increased 90-d rate of pneumonia and pulmonary embolism concomitant with a decreased hematoma rate consistent with their procoagulatory state.Further attention to increased resource utilization in these patients is also warranted.

A Survey on TCM Treatment of Polymyositis and Dermatomyositis

Polymyositis is an illness characterized by inflammation of the muscle(s) with uncertain etiological causes. Most of the physicians think that viral infection, disturbances of immune functions and/or pathological vascular changes might be responsible for the condition.

Pulmonary hypertension,nephrotic syndrome,and polymyositis due to hepatitis C virus infection:A case report

BACKGROUND Hepatitis C infection not only damages the liver but also often accompanies many extrahepatic manifestations.Incidences of pulmonary hypertension(PH)caused by hepatitis C are rare,and incidences of concurrent nephrotic syndrome and polymyositis are even rarer.CASE SUMMARY Herein we describe the case of a 57-year-old woman who was admitted to our department for intermittent chest tightness upon exertion for 5 years,aggravated with dyspnea for 10 d.After relevant examinations she was diagnosed with PH,nephrotic syndrome,and polymyositis due to chronic hepatitis C infection.A multi-disciplinary recommendation was that the patient should be treated with sildenafil and macitentan in combination and methylprednisolone.During treatment autoimmune symptoms,liver function,hepatitis C RNA levels,and cardiac parameters of right heart catheterization were monitored closely.The patient showed significant improvement in 6-min walking distance from 100 to 300 m at 3-mo follow-up and pulmonary artery pressure drops to 50 mmHg.Long-term follow-up is needed to confirm further efficacy and safety.CONCLUSION Increasing evidence supports a relationship between hepatitis C infection and diverse extrahepatic manifestations,but it is very rare to have PH,nephrotic syndrome,and polymyositis in a single patient.We conducted a literature review on the management of several specific extrahepatic manifestations of hepatitis C.

Anti-melanoma differentiation-associated gene 5 and anti-Ro52 antibody-dual positive dermatomyositis accompanied by rapidly lung disease:Three case reports

BACKGROUND Clinically amyopathic deramatomyositis was manifested as the various cutaneous dermatomyositis(DM)manifestations without muscle weakness.Anti-melanoma differentiation-associated gene 5(anti-MDA5)and anti-Ro52 antibody-dual positive clinically amyopathic DM patients are at a high risk of developing rapidly progressive interstitial lung disease,and they exhibit an immensely high half-year mortality.CASE SUMMARY We presented three patients with anti-MDA5 and anti-Ro52 antibody-dual positive DM patients and we reviewed the previous studies on the link between anti-MDA5 and anti-Ro52 antibody-dual positive DM.Although we aggressively treated these patients similarly,but they all exhibited different prognoses.We reviewed the importance of clinical cutaneous rashes as well as the pathogenesis and treatment in the dual positive anti-MDA5 and anti-Ro52 associated DM.CONCLUSION Patients with anti-MDA5 anti-Ro52 antibody-dual positive DM should be accurately diagnosed at an early stage and should be treated aggressively,thus,the patient’s prognosis can be significantly modified.

Treatment of refractory anti-melanoma differentiation-associated gene 5 anbibody-positive dermatomyositis complicated by rapidly progressing interstitial pulmonary disease:Two case reports

BACKGROUND Anti-melanoma differentiation-associated gene 5 antibody-positive(anti-MDA5 Ab+)dermatomyositis complicated with rapidly progressive interstitial lung disease(anti-MDA5 Ab+DM-RP-ILD)has an unclear underlying mechanism with no recommended unified treatment plan.Herein,one of the cases that we report(Case 2)was successfully treated with tocilizumab despite having lung infection.CASE SUMMARY Case 1 was a 30-year-old woman who was admitted due to recurrent rash for 5 mo,fever and cough for 1 mo,and chest tightness for 3 d.She was diagnosed with non-myopathic dermatomyositis(anti-MDA5 Ab+)and interstitial pneumonia,and was treated with the combination of hormone therapy and cyclophosphamide followed by oral tacrolimus.Case 2 was a 31-year-old man admitted due to systemic rash accompanied by muscle weakness of limbs for more than 1 mo,and chest tightness and dry cough for 4 d.He was diagnosed with dermatomyositis(anti-MDA5 Ab+)and acute interstitial pneumonia with Pneumocystis jirovecii and Aspergillus fumigatus infections and was treated with hormone therapy(without cyclophosphamide)and the combination of tocilizumab and tacrolimus.The condition of both patients eventually improved and they were discharged and showed clinically stable condition at the latest follow-up.CONCLUSION Tocilizumab could be a salvage treatment for patients with anti-MDA5 Ab+DMRP-ILD who are refractory to intensive immunosuppression.

Hepatitis B virus-related liver cirrhosis complicated with dermatomyositis: A case report

BACKGROUND Twenty percent of patients infected with hepatitis B virus(HBV)develop extrahepatic manifestations with HBV detected in the lymph nodes,spleen,bone marrow,kidneys,and skin.HBV infection has been associated with some autoimmune disorders.Dermatomyositis(DM)is an idiopathic inflammatory myopathy,which involves a viral infection,and DM has been identified in patients infected with HBV,but there is no direct histological evidence for an association between HBV and DM.CASE SUMMARY We describe a familial HBV-infected patient admitted with liver function abnormality,rashes,a movement disorder,and an elevated level of creatine kinase(CK).A computed tomography scan of the lung showed pulmonary fibrosis,and a liver biopsy identified nodular cirrhosis.An electromyogram revealed myogenic damage,and a muscle biopsy showed nuclear migration in local sarcolemma and infiltration of chronic inflammatory cells.Immunohistochemical staining showed negative results for HBsAg and HBcAg.Fluorescence in situ hybridization showed a negative result for HBV DNA.The patient was diagnosed with HBV-related liver cirrhosis complicated with DM and was treated with methylprednisolone,mycophenolate mofetil,and lamivudine.Eight months later,the patient was readmitted for anorexia and fatigue.The blood examination showed elevated levels of aminotransferases and HBV DNA,however,the CK level was within the normal range.The patient developed a virological breakthrough and lamivudine was replaced with tenofovir.CONCLUSION DM in chronic HBV-infected patients does not always associate with HBV.Antiviral and immunosuppressive drugs should be taken into consideration.

Dermatomyositis associated with hepatocellular carcinoma in an elderly female patient with hepatitis C virus-related liver cirrhosis

A 79-year-old female patient with hepatitis C virusrelated liver cirrhosis was diagnosed as having hepatocellular carcinoma （HCC） with a diameter of 2.0 cm. She refused therapy for HCC. Nine months after the diagnosis, she developed dermatomyositis when the HCC enlarged to a diameter of 6.0 cm. She underwent therapy for dermatomyositis, and then transcatheter arterial chemoembolization for HCC. Although the manifestations of dermatomyositis improved and entire tumor necrosis was achieved, she died of pneumonia 2 mo after the treatment of HCC. HCC and/or chronic hepatitis C virus infection might be involved in the pathogenesis of dermatomyositis.

Dermatomyositis associated with gallbladder carcinoma:A case report

Patients with gallbladder carcinoma can present with a variety of paraneoplastic syndromes,including Cushing's syndrome,hypercalcemia,acanthosis nigricans,bullous pemphigoid,dermatomyositis and the sign of Leser-Trélat.Surgical resection of the primary tumor results in resolution of these paraneoplastic syndromes.We present a 67-year old female with facial and cervical erythema who was initially diagnosed with dermatomyositis.However,an abdominal computed tomography(CT)and positron emission tomographyCT scan was suspicious for gallbladder carcinoma with lymph node metastasis.After surgical resection,her dermatomyositis was resolved.This case demonstrates that dermatomyositis may be a manifestation of preexisting gallbladder carcinoma.

Endoscopy is of low yield in the identification of gastrointestinal neoplasia in patients with dermatomyositis: A cross-sectional study

To determine the prevalence of gastrointestinal neoplasia among dermatomyositis patients who underwent an esophagogastroduodenoscopy and/or colonoscopy. METHODSA cross-sectional study examining the results of upper endoscopy and colonoscopy in adults with dermatomyositis at an urban, university hospital over a ten year period was performed. Chart review was performed to confirm the diagnosis of dermatomyositis. Findings on endoscopy were collected and statistical analyses stratified by age and presence of symptoms were performed. RESULTSAmong 373 adult patients identified through a code based search strategy, only 163 patients had dermatomyositis confirmed by chart review. Of the 47 patients who underwent upper endoscopy, two cases of Barrett’s esophagus without dysplasia were identified and there were no cases of malignancy. Of the 67 patients who underwent colonoscopy, no cases of malignancy were identified and an adenoma was identified in 15% of cases. No significant differences were identified in the yield of endoscopy when stratified by age or presence of symptoms. CONCLUSIONThe yield of endoscopy is low in patients with dermatomyositis and is likely similar to the general population; we identified no cases of malignancy. A code based search strategy is inaccurate for the diagnosis of dermatomyositis, calling into question the results of prior population-based studies. Larger studies with rigorously validated search strategies are necessary to understand the risk of gastrointestinal malignancy in patients with dermatomyositis.

Infliximab is effective in the treatment of ulcerative colitis with dermatomyositis:A case report

BACKGROUND Joint,skin,oral cavity,and eye lesions are the most common extraintestinal manifestations of ulcerative colitis that can occur before or after its onset.The cases of ulcerative colitis with dermatomyositis(DM)are rare.In this study,we report a rare case of ulcerative colitis with DM that was effectively treated with infliximab.CASE SUMMARY The patient was a 57-year-old female with a 2-year history of DM.The patient was admitted to hospital with abdominal pain,diarrhea,and blood in stool lasting for more than 2 mo.Colonoscopy revealed multiple erosions and ulcers in the entire colon and rectum.Pathological sections showed chronic inflammatory cell infiltration,especially neutrophil infiltration,in the colonic mucosa;therefore,the patient was diagnosed with ulcerative colitis.Preparations of 5-aminosalicylic acid was added to her treatment based on the original treatment for DM,but its effect was unsatisfactory.The patient’s discomfort was relieved after infliximab treatment.CONCLUSION Infliximab can improve DM in the treatment of ulcerative colitis.Specialists need to raise awareness about patients with inflammatory bowel disease who have rare extraintestinal manifestations.

Symptoms relieved after tumor removal in a case of dermatomyositis associated with hepatocellular carcinoma:case report and literature review

A case of dermatomyositis associated with hepatocellular carcinoma is reported in a 43-year-old man,in which removal of the tumor resulted in great improvement of dermatomyositis.The patient had shown aggravating symptoms of dermatomyositis including manifestations of typical skin rashes and muscle weakness six months before he came to our hospital.He had no history of drug allergy and took no medicine in these days.When first visiting the dermatological clinic,he complained of discomforts in skin and muscle manifestation and was diagnosed as having dermatomyositis.At the same time,A CT scan discovered a resectable liver tumor in the right lobe.Then he came to our hospital and asked for surgical treatment.Removal of the tumor resulted in great improvement of dermatomyositis and recurrence of liver tumor was followed by resumption of dermatomyositis.

Muscular involvement of extranodal natural killer/T cell lymphoma misdiagnosed as polymyositis: A case report and review of literature

BACKGROUND Natural killer(NK)/T cell lymphoma is a rare and highly aggressive malignant tumor,and is a special form of non-Hodgkin's lymphoma.Although extranodal involvement is frequently found in tissues such as the skin,testicular and gastrointestinal tract etc,its presence in skeletal muscle has scarcely been reported in the literature.CASE SUMMARY We report a case of extranodal NK/T cell lymphoma with muscle swelling as the first clinical manifestation.A 42-year-old man,who initially presented with localized swelling in the double lower extremities,demonstrated gradual facial and eyelid swelling,and his imaging results showed multiple sites of muscle damage throughout the body.The final pathological results suggested NK/T cell lymphoma,and immunohistochemistry showed CD20(-),CD3(+),CD30(+),CD56(-),EBER(+),Ki67(60%),TIA-1(+)and CD68(±)staining.The muscle swelling significantly improved after treatment with chemotherapy regimens.CONCLUSION This disease is difficult to diagnose and highly invasive,and should be included in the differential diagnosis of unexplained muscle swelling.

Treatment of gastric cancer with dermatomyositis as the initial symptom:Two case reports and review of literature

BACKGROUND Dermatomyositis(DM) is a rare autoimmune disease involving the connective tissue.The association between DM and gastric cancer remains unclear.Patients with DM have an increased risk of cancer and higher mortality.It requires immunosuppressive therapy,heightened surveillance,and immunologic response to internal malignancy.CASE SUMMARY Two cases of gastric cancer with DM as the first symptom in Zhongshan Hospital,Fudan University(Shanghai,China) were reported.Two patients had a typical skin rash.The rash in the first patient involved mainly bilateral upper limbs and neck,while the second patient manifested rash associated mainly with the face,neck,and back.Both manifested muscle weakness in the extremities and elevated serum creatine kinase.Radical resection of the tumor dramatically improved DMrelated symptoms in the two patients.The literature review showed that gastric cancer is more commonly associated with DM in middle-aged and older male populations.CONCLUSION The findings suggest the need for comprehensive screening for malignant tumors in patients with DM refractory to long-term pharmacotherapy or hormone manipulation.

Pneumocystis jiroveci pneumonia after total hip arthroplasty in a dermatomyositis patient:A case report

BACKGROUND Pneumocystis jiroveci pneumonia(PJP)is a serious opportunistic infection that occurs mostly in patients with immunodeficiency and long-term immunosuppressive therapy.In non-human immunodeficiency virus-infected patients,the most important risk factor for PJP is the use of glucocorticoids in combination with other immunosuppressive treatments.The management of glucocorticoids during the perioperative period in patients with dermatomyositis requires special care.CASE SUMMARY We report a case of PJP in the perioperative period.A 61-year-old woman with a history of anti-melanoma differentiation-associated gene 5(MDA5)-positive dermatomyositis and interstitial pneumonia was administered with long-term oral methylprednisolone and cyclosporine.The patient underwent right total hip arthroplasty in the orthopaedic department for bilateral osteonecrosis of the femoral head.She was given intravenous drip hydrocortisone before anesthesia and on the first day after surgery and resumed oral methylprednisolone on the second postoperative day.On the fifth day after surgery,the patient suddenly developed dyspnea.The computed tomography scan showed diffuse grid shadows and ground glass shadows in both lungs.Polymerase chain reaction testing of bronchoalveolar lavage fluid was positive for Pneumocystis jiroveci.The patient was eventually diagnosed with PJP and was administered with oral trimethoprim-sulfamethoxazole.At the 6-mo review,there was no recurrence or progression.CONCLUSION Continued perioperative glucocorticoid use in patients with anti-MDA5-positive dermatomyositis may increase the risk of PJP.

Safety of Transcranial Direct Current Electrical Stimulation in Dermatomyositis: A Case Report

Transcranial direct current stimulation (tDCS) has emerged as a nonpharmacological tool in physical rehabilitation. There have currently no studies that evaluated the safety and efficacy of tDCS in patients with dermatomyositis. Case-report: Three adult women with dermatomyositis were allocated randomly to intervention (i-tDCS, one patient) or not (sham-tDCS, two patients) of three consecutive days of tDCS and evaluated in four periods: before-tDCS (PRE), 15 minutes after-tDCS (0th POST), 15 days after-tDCS (15th POST), and 30 days after-tDCS (30th POST). The tDCS was safe throughout the protocol, without disease relapsing or adverse effects related to tDCS. Furthermore, the tDCS increased the muscle torque and total work of dominant and non-dominant elbow flexors in the patient with i-tDCS, when compared to two patients with sham-tDCS. Conclusions: The tDCS was safe and appeared to influence long-term strength in the limb of the patient with stable dermatomyo-sitis.

Polymyositis-like syndrome with rhabdomyolysis in association with brucellosis

Diffuse myositis with progression to rhabdomynlysis has been reported in association with wide range of viral infections.We report a case of polvmvosilis-like svndrome complicated by rhabdomyolysis secondary to brucellosis.This case report thus contributes yet another atypical presentation to a disease already infamous for its protean manifestations.

Paraneoplastic Dermatomyositis Accompanying Nasopharyngeal Carcinoma

NASOPHARYNGEAL carcinoma （NPC） is a raredisease in North America and Western Europe,but is common in Asia and North Africa.1,2 Asmall percentage of NPC patients present withparaneoplastic syndrome and less than 0.1% with dermatomyositis.2 No prospective case-control double-blindedstudies of immunosuppressive therapy in dermatomyositishave been performed, let alone in paraneoplastic dermatomyositis.