Objective:To evaluate the predictive value of the neutrophil⁃to⁃lymphocyte ratio(NLR)and the systemic immune⁃inflammation index(SII)in predicting patients with anti⁃melanoma differentiation⁃associated gene 5⁃positive(...Objective:To evaluate the predictive value of the neutrophil⁃to⁃lymphocyte ratio(NLR)and the systemic immune⁃inflammation index(SII)in predicting patients with anti⁃melanoma differentiation⁃associated gene 5⁃positive(anti⁃MDA5+)dermatomyositis(DM)develop into the rapidly progressive interstitial lung disease(RPILD).Methods:We retrospectively analyzed the clinical and laboratory data of 124 anti⁃MDA5+DM patients from the First Affiliated Hospital of Nanjing Medical University between March 2019 and September 2023.We identified independent risk factors associated with the development and mortality of RPILD with the Cox regression analysis,and determined the optimal cut⁃off values for predicting adverse outcomes with the receiver operating characteristic(ROC)curve analysis.Results:Among the 124 patients,36 patients(29.03%)developed RPILD,and 39 patients(31.45%)died during the follow⁃up period.The results of multivariate Cox regression analysis showed that the elevated NLR was an independent risk factor for RPILD development,while the elevated SII expression was independently associated with the increased mortality of RPILD.Based on the ROC curve analysis,NLR>6.12 was a predictor for RPILD,and SII>875.79 was associated with increased mortality risk of RPILD.Conclusion:Both NLR and SII are accessible,cost⁃effective,and reliable prognostic indicators for the prognosis of patients with anti⁃MDA5^(+)DM,providing a valuable guidance for clinical management and risk stratification of the disease.展开更多
Dermatomyositis(DM)is an idiopathic inflammatory myopathy characterized by prominent skin lesions,muscle weakness,and clinically heterogeneous systemic manifestations.Among patients with DM,the male-to-female ratio is...Dermatomyositis(DM)is an idiopathic inflammatory myopathy characterized by prominent skin lesions,muscle weakness,and clinically heterogeneous systemic manifestations.Among patients with DM,the male-to-female ratio is 2:1.Juvenile dermatomyositis(JDM)is most prevalent between the ages of 4 and 14 years,while adult-onset dermatomyositis typically occurs between the ages of 40 and 60 years.In a population-based study conducted in Olmsted County,Minnesota,USA,the risk of DM was found to increase with age across different age groups stratified by decade.The incidence rate of DM in individuals aged≥80 years was 3.2 per 100,000 person-years.Dermatomyositis in elderly patients is characterized by unique clinical manifestations,pathogenic mechanisms,and therapeutic approaches.However,discussions regarding geriatric dermatomyositis are currently limited.Therefore,this article aims to review the epidemiology,clinical features,histopathology,and pathogenesis of dermatomyositis,with a particular focus on the unique clinical characteristics of geriatric dermatomyositis.展开更多
In this manuscript, we present a case report of a child with 16-year-old previously healthy Hispanic male who presented for progressive proximal muscle weakness, rash, and dysphagia. He was admitted to the acute care ...In this manuscript, we present a case report of a child with 16-year-old previously healthy Hispanic male who presented for progressive proximal muscle weakness, rash, and dysphagia. He was admitted to the acute care floor and diagnosed with juvenile dermatomyositis and found to be positive for anti-Mi-2 alpha, anti-Mi-2 beta, and anti-MDA-5 antibodies. He gradually improved with a combination of steroid, immunomodulatory treatment, and physical therapy. This case outlines the clinical course of a patient with this rare disorder as well as the importance of understanding the role of associated antibodies to manage potential long-term sequelae.展开更多
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 diagnos...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.展开更多
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(...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.展开更多
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 ...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.展开更多
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-infe...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.展开更多
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 en...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.展开更多
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-T...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.展开更多
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 mo...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.展开更多
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 symp...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.展开更多
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 an...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.展开更多
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 derma...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.展开更多
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%...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.展开更多
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 vasc...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.展开更多
Dermatomyositis is known to be associated with neoplastic disorders,however the presentation of carcinoma of unknown primary as dermatomyositis is rare. We describe a case index of 50-year-old female who presented wit...Dermatomyositis is known to be associated with neoplastic disorders,however the presentation of carcinoma of unknown primary as dermatomyositis is rare. We describe a case index of 50-year-old female who presented with enlarged inguinal lymph nodes accompanied with symmetric proximal muscle weakness and erythematous plaques. Conventional basic work-up did not reveal the diagnosis,however,positron emission tomography-computed tomography and re-staining of the pathology specimen suggested the ovaries as the primary site. Chemotherapy including carboplatin paclitaxel and bevacizumab led to complete response of disease and improvement in the dermatomyositis. The present case emphasizes the importance of a thorough directed evaluation for the underlying cancer in patients with carcinoma of unknown primary presenting as dermatomyositis. We further provide an up-to-date detailed review of published data describing these clinical entities.展开更多
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 c...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.展开更多
A 51-year old male who presented at our hospital for recurrent palpitation for several months was diagnosed dermatomyositis ten years ago and had interstitial lung disease since two years ago. Recently, he was admitte...A 51-year old male who presented at our hospital for recurrent palpitation for several months was diagnosed dermatomyositis ten years ago and had interstitial lung disease since two years ago. Recently, he was admitted for atypical hepatitis, and received maintenance treatment of oral corticosteroids.展开更多
BACKGROUND Macrophage activation syndrome(MAS)can be a fatal complication of rheumatic disorders,which occurs most commonly in patients with systemic juvenile idiopathic arthritis or systemic lupus erythematosus.It ha...BACKGROUND Macrophage activation syndrome(MAS)can be a fatal complication of rheumatic disorders,which occurs most commonly in patients with systemic juvenile idiopathic arthritis or systemic lupus erythematosus.It has rarely been reported in patients with dermatomyositis.Here,we describe a fatal case of MAS that developed in an adult patient with dermatomyositis.CASE SUMMARY A 44-year-old woman was admitted to our hospital with fever,generalized rash and muscle weakness.Fifteen days later,the fever persisted after the use of antibiotics,and repeat blood culture was negative.The patient then exhibited a typical Gottron sign and diffuse erythema on the face and neck,which were consistent with a diagnosis of dermatomyositis.The patient exhibited limb muscle strength of 2,and electromyography was suggestive of muscle-derived damage,which also supported a diagnosis of dermatomyositis.In addition,the patient exhibited high serum ferritin level,cytopenia,liver dysfunction,coagulopathy,enlarged spleen and hypertriglyceridemia,all of which are typical manifestations of MAS.The patient was diagnosed with dermatomyositis complicated by MAS.Although a high dose of methylprednisolone was administered for 15 d,the patient’s condition continued to deteriorate and central nervous system symptoms developed.Eventually,treatment was discontinued,and the patient died.CONCLUSION MAS is an important,potentially fatal,complication of dermatomyositis.Although MAS is rare in dermatomyositis,it should be considered in the differential diagnosis of an unexplained change of hemoglobin,platelet,fibrinogen,ferritin and triglyceride,which may complicate dermatomyositis.展开更多
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 osteoarthrit...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.展开更多
文摘Objective:To evaluate the predictive value of the neutrophil⁃to⁃lymphocyte ratio(NLR)and the systemic immune⁃inflammation index(SII)in predicting patients with anti⁃melanoma differentiation⁃associated gene 5⁃positive(anti⁃MDA5+)dermatomyositis(DM)develop into the rapidly progressive interstitial lung disease(RPILD).Methods:We retrospectively analyzed the clinical and laboratory data of 124 anti⁃MDA5+DM patients from the First Affiliated Hospital of Nanjing Medical University between March 2019 and September 2023.We identified independent risk factors associated with the development and mortality of RPILD with the Cox regression analysis,and determined the optimal cut⁃off values for predicting adverse outcomes with the receiver operating characteristic(ROC)curve analysis.Results:Among the 124 patients,36 patients(29.03%)developed RPILD,and 39 patients(31.45%)died during the follow⁃up period.The results of multivariate Cox regression analysis showed that the elevated NLR was an independent risk factor for RPILD development,while the elevated SII expression was independently associated with the increased mortality of RPILD.Based on the ROC curve analysis,NLR>6.12 was a predictor for RPILD,and SII>875.79 was associated with increased mortality risk of RPILD.Conclusion:Both NLR and SII are accessible,cost⁃effective,and reliable prognostic indicators for the prognosis of patients with anti⁃MDA5^(+)DM,providing a valuable guidance for clinical management and risk stratification of the disease.
文摘Dermatomyositis(DM)is an idiopathic inflammatory myopathy characterized by prominent skin lesions,muscle weakness,and clinically heterogeneous systemic manifestations.Among patients with DM,the male-to-female ratio is 2:1.Juvenile dermatomyositis(JDM)is most prevalent between the ages of 4 and 14 years,while adult-onset dermatomyositis typically occurs between the ages of 40 and 60 years.In a population-based study conducted in Olmsted County,Minnesota,USA,the risk of DM was found to increase with age across different age groups stratified by decade.The incidence rate of DM in individuals aged≥80 years was 3.2 per 100,000 person-years.Dermatomyositis in elderly patients is characterized by unique clinical manifestations,pathogenic mechanisms,and therapeutic approaches.However,discussions regarding geriatric dermatomyositis are currently limited.Therefore,this article aims to review the epidemiology,clinical features,histopathology,and pathogenesis of dermatomyositis,with a particular focus on the unique clinical characteristics of geriatric dermatomyositis.
文摘In this manuscript, we present a case report of a child with 16-year-old previously healthy Hispanic male who presented for progressive proximal muscle weakness, rash, and dysphagia. He was admitted to the acute care floor and diagnosed with juvenile dermatomyositis and found to be positive for anti-Mi-2 alpha, anti-Mi-2 beta, and anti-MDA-5 antibodies. He gradually improved with a combination of steroid, immunomodulatory treatment, and physical therapy. This case outlines the clinical course of a patient with this rare disorder as well as the importance of understanding the role of associated antibodies to manage potential long-term sequelae.
文摘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.
基金the National Natural Science Foundation of China,No.81860104 and 81460114the Natural Science Foundation of Guangxi Zhuang Autonomous Region,No.2017GXNSFAA198299+2 种基金the Development and Application of Medical and Health Appropriate Technology Project in Guangxi Zhuang Autonomous Region,No.S2018049the Youth Science Foundation of Guangxi Medical University,No.GXMUYSF201913 and GXMUYSF201908the Self-financing Project of Health Commission of Guangxi Zhuang Autonomous Region,No.Z20200398。
文摘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.
基金Supported by Natural Science Foundation of Jilin Science and Technology Department,No.20190201065JC
文摘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.
基金Supported by National Natural Science Foundation of China,No. 81673776, and No. 82072494
文摘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.
基金Supported by National Cancer Institute,No.K07CA212057American Gastroenterological Association
文摘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.
文摘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.
基金Supported by the National Natural Science Foundation of China,No. 81773080。
文摘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.
文摘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.
文摘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.
基金Fundacao de Amparo a Pesquisa do Estado de Sao Paulo(FAPESP)#2019/12155-5 to RGMConselho Nacional de Desenvolvimento Cientifico e Tecnologico(CNPq)303379/2018-9 to SKSFaculdade de Medicina da USP to SKS.
文摘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.
文摘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.
文摘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.
文摘Dermatomyositis is known to be associated with neoplastic disorders,however the presentation of carcinoma of unknown primary as dermatomyositis is rare. We describe a case index of 50-year-old female who presented with enlarged inguinal lymph nodes accompanied with symmetric proximal muscle weakness and erythematous plaques. Conventional basic work-up did not reveal the diagnosis,however,positron emission tomography-computed tomography and re-staining of the pathology specimen suggested the ovaries as the primary site. Chemotherapy including carboplatin paclitaxel and bevacizumab led to complete response of disease and improvement in the dermatomyositis. The present case emphasizes the importance of a thorough directed evaluation for the underlying cancer in patients with carcinoma of unknown primary presenting as dermatomyositis. We further provide an up-to-date detailed review of published data describing these clinical entities.
文摘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.
文摘A 51-year old male who presented at our hospital for recurrent palpitation for several months was diagnosed dermatomyositis ten years ago and had interstitial lung disease since two years ago. Recently, he was admitted for atypical hepatitis, and received maintenance treatment of oral corticosteroids.
文摘BACKGROUND Macrophage activation syndrome(MAS)can be a fatal complication of rheumatic disorders,which occurs most commonly in patients with systemic juvenile idiopathic arthritis or systemic lupus erythematosus.It has rarely been reported in patients with dermatomyositis.Here,we describe a fatal case of MAS that developed in an adult patient with dermatomyositis.CASE SUMMARY A 44-year-old woman was admitted to our hospital with fever,generalized rash and muscle weakness.Fifteen days later,the fever persisted after the use of antibiotics,and repeat blood culture was negative.The patient then exhibited a typical Gottron sign and diffuse erythema on the face and neck,which were consistent with a diagnosis of dermatomyositis.The patient exhibited limb muscle strength of 2,and electromyography was suggestive of muscle-derived damage,which also supported a diagnosis of dermatomyositis.In addition,the patient exhibited high serum ferritin level,cytopenia,liver dysfunction,coagulopathy,enlarged spleen and hypertriglyceridemia,all of which are typical manifestations of MAS.The patient was diagnosed with dermatomyositis complicated by MAS.Although a high dose of methylprednisolone was administered for 15 d,the patient’s condition continued to deteriorate and central nervous system symptoms developed.Eventually,treatment was discontinued,and the patient died.CONCLUSION MAS is an important,potentially fatal,complication of dermatomyositis.Although MAS is rare in dermatomyositis,it should be considered in the differential diagnosis of an unexplained change of hemoglobin,platelet,fibrinogen,ferritin and triglyceride,which may complicate dermatomyositis.
文摘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.
