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-Ro...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.展开更多
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 underlyin...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.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
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
AIM To determine the prevalence of gastrointestinal neoplasia among dermatomyositis patients who underwent an esophagogastroduodenoscopy and/or colonoscopy.METHODS A cross-sectional study examining the results of uppe...AIM To determine the prevalence of gastrointestinal neoplasia among dermatomyositis patients who underwent an esophagogastroduodenoscopy and/or colonoscopy.METHODS A 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.RESULTS Among 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.CONCLUSION The 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
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
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
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
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
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
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
BACKGROUND Juvenile dermatomyositis(JDM)is an idiopathic inflammatory myopathy that occurs in childhood.It is characterized by muscle weakness and a characteristic rash.Previous literature reports have rarely describe...BACKGROUND Juvenile dermatomyositis(JDM)is an idiopathic inflammatory myopathy that occurs in childhood.It is characterized by muscle weakness and a characteristic rash.Previous literature reports have rarely described JDM with severe skin ulcers and infections.CASE SUMMARY Herein,we describe a case of a 2-year-old female patient who suffered from JDM,whose myositis-specific autoantibodies were positive for anti-nuclear matrix protein 2 antibody,with progressively worsening skin ulcers and severe infections.The patient was treated with glucocorticoids and various immunosuppressants.Nevertheless,further progression of the disease and the combination of primary disease and severe infection in the later period were fatal.CONCLUSION In children,anti-nuclear matrix protein 2+JDM combined with skin ulcers often indicates severe disease.In such cases,personalized treatment for the primary disease and infection prevention and control are essential.展开更多
In very scarce case reports and case series, tofacitinib has been a therapeutic alternative for dermatomyositis. To corroborate the literature, we described a refractory dermatomyositis that had a good outcome with to...In very scarce case reports and case series, tofacitinib has been a therapeutic alternative for dermatomyositis. To corroborate the literature, we described a refractory dermatomyositis that had a good outcome with tofacitinib. Case Report: An adult female patient presented with definite dermatomyositis and with refractoriness to high doses of intravenous and oral glucocorticoids, intravenous human immunoglobulin, several immunosuppressive drugs (methotrexate, azathioprine, and leflunomide) and two previous immunobiological drugs (rituximab and abatacept). However, the patient had a good outcome with tofacitinib. Conclusions: Tofacitinib appears to be a promising alternative therapy for refractory dermatomyositis.展开更多
Objectives: To correlate the precise specificity of autoantibodies in Japanese dermatomyositis (DM) patients with their clinical phenotypes. Methods: Serum samples from 94 adult DM patients (67 with classical DM and 2...Objectives: To correlate the precise specificity of autoantibodies in Japanese dermatomyositis (DM) patients with their clinical phenotypes. Methods: Serum samples from 94 adult DM patients (67 with classical DM and 27 with clinically amyopathic dermatomyositis, CADM) were screened for autoantibodies using immunoprecipitation assays. Patients with antibodies against aminoacyl transfer RNA synthetase (ARS), Mi-2 or who had other autoantibodies were assessed for clinical symptoms and laboratory findings. Results: Sera from 27 of 94 DM patients (29%) were found to have anti-ARS antibodies. Nineteen (20%) had anti-CADM-140/MDA5, 5 (5%) had anti-Mi-2, and 8 (6%) had anti-p155/TIF1-γ. Anti-MJ/NXP-2 was not found in our series of adult DM. Seventeen patients with anti-ARS had fever and 22 had arthritis and interstitial lung disease (ILD), compatible with a diagnosis of anti-ARS syndrome. Seventeen of 19 (89%) with anti-CADM-140/MDA5 had ILD, 16 (84%) of whom developed rapidly progressive ILD (RP-ILD). Four of 5 (80%) with anti-Mi-2 had heliotrope rash and/or Gottron’s sign/papules, and 2 (40%) had V-sign and/or shawl-sign rash, whereas no ILD or malignancy was detected. As seen with anti-Mi-2-positive patients, a low frequency of ILD (13%) was found in patients with anti-p155/TIF1-γ but 6 of 8 (75%) had malignancy during their course. The frequency of ILD was significantly higher in patients with anti-ARS or anti-CADM-140/MDA5 compared with anti-Mi-2 or anti-p155/TIF1-γ (81% and 89%, respectively). It should be noted that anti-CADM-140/MDA5-positive patients suffered significantly more RP-ILD compared to patients with anti-ARS (84% vs. 7%, P < 0.0001). On the other hand, anti-p155/TIF1-γ positive patients had a significantly higher rate of malignancy compared with anti-ARS-, anti-CADM-140/MDA5-and anti-Mi-2-positive patients (75% vs. 7%: P = 0.0004, 5%: P = 0.0006, 0%: P = 0.02, respectively). Conclusions: These results indicate that in addition to antibodies previously identified as specific for DM, autoantibodies newly found in these patients are useful for stratifying them into clinical subgroups.展开更多
To the best of our knowledge, this is the first report in the literature showing the efficacy of transcranial direct current stimulation (tDCS) in treating refractory post-herpetic neuralgia in a patient with dermatom...To the best of our knowledge, this is the first report in the literature showing the efficacy of transcranial direct current stimulation (tDCS) in treating refractory post-herpetic neuralgia in a patient with dermatomyositis. In addition, our results showed that tDCS sessions are safe and do not lead to disease relapse. Therefore, despite the limitations of being a case report, this study provides encouraging results that need to be widely explored in studies with a representative number of patients and with an appropriate design for patients with this systemic rheumatic autoimmune disease.展开更多
Background: Calcinosis is an important sequela of JDM which may cause significant morbidity and mortality. There is no standard curative treatment for calcinosis but different agents were used with variable efficacy. ...Background: Calcinosis is an important sequela of JDM which may cause significant morbidity and mortality. There is no standard curative treatment for calcinosis but different agents were used with variable efficacy. We report the favorable outcome of rituximab on severe calcinosis in 4 JDM patients and present their clinical data. Patients and Methods: A retrospective chart review of 4 children with JDM and severe calcinosis who received rituximab for relapsing or polycyclic JDM course. Diagnosis and follow up of calcinosis was clinically and by X-ray. Review data included: age of patients at onset of JDM symptoms and diagnosis, clinical and laboratory criteria at diagnosis, disease course and duration of follow up. Data about calcinosis onset, sites, severity and its progression were also included. Further data about rituximab therapy included: dosage, side effects, other treatment used before, during or after this drug and outcome and duration of follow up of calcinosis after therapy. Results: 4 patients (2 male, 2 female), interval between onset of symptoms and diagnosis was 6 - 12 months, course of JDM was polycyclic or relapsing, duration of follow up was 5 - 7 years. Calcinosis was severe causing ulceration, recurrent skin infections and joint limitation. It was not improving despite treatment with different DMARDs and/or bisphosphonates, colchicine and warfarin. Reason to start rituximab was inadequate disease control with conventional DMARDs. All patients received steroids and more than one DMARD before starting rituximab and were continued thereafter, follow up after rituximab was 3 to 5 years. All patients had improvement in disease activity and frequency of admission especially due to complications of calcinosis. One patient had complete clearance of calcinosis for the last 5 years. Others had significant improvement in calcinosis with no new lesions, decreased sites and density and decreased calcinosis related contractures. There were no serious side effects to rituximab. Conclusion: Our study showed the favorable effect of rituximab in treatment of calcinosis in 4 patients with JDM-associated severe calcinosis when it was used with other conventional DMARDs.展开更多
文摘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.
文摘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.
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.
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 National Cancer Institute,No.K07CA212057American Gastroenterological Association
文摘AIM To determine the prevalence of gastrointestinal neoplasia among dermatomyositis patients who underwent an esophagogastroduodenoscopy and/or colonoscopy.METHODS A 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.RESULTS Among 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.CONCLUSION The 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.
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.
文摘BACKGROUND Juvenile dermatomyositis(JDM)is an idiopathic inflammatory myopathy that occurs in childhood.It is characterized by muscle weakness and a characteristic rash.Previous literature reports have rarely described JDM with severe skin ulcers and infections.CASE SUMMARY Herein,we describe a case of a 2-year-old female patient who suffered from JDM,whose myositis-specific autoantibodies were positive for anti-nuclear matrix protein 2 antibody,with progressively worsening skin ulcers and severe infections.The patient was treated with glucocorticoids and various immunosuppressants.Nevertheless,further progression of the disease and the combination of primary disease and severe infection in the later period were fatal.CONCLUSION In children,anti-nuclear matrix protein 2+JDM combined with skin ulcers often indicates severe disease.In such cases,personalized treatment for the primary disease and infection prevention and control are essential.
文摘In very scarce case reports and case series, tofacitinib has been a therapeutic alternative for dermatomyositis. To corroborate the literature, we described a refractory dermatomyositis that had a good outcome with tofacitinib. Case Report: An adult female patient presented with definite dermatomyositis and with refractoriness to high doses of intravenous and oral glucocorticoids, intravenous human immunoglobulin, several immunosuppressive drugs (methotrexate, azathioprine, and leflunomide) and two previous immunobiological drugs (rituximab and abatacept). However, the patient had a good outcome with tofacitinib. Conclusions: Tofacitinib appears to be a promising alternative therapy for refractory dermatomyositis.
文摘Objectives: To correlate the precise specificity of autoantibodies in Japanese dermatomyositis (DM) patients with their clinical phenotypes. Methods: Serum samples from 94 adult DM patients (67 with classical DM and 27 with clinically amyopathic dermatomyositis, CADM) were screened for autoantibodies using immunoprecipitation assays. Patients with antibodies against aminoacyl transfer RNA synthetase (ARS), Mi-2 or who had other autoantibodies were assessed for clinical symptoms and laboratory findings. Results: Sera from 27 of 94 DM patients (29%) were found to have anti-ARS antibodies. Nineteen (20%) had anti-CADM-140/MDA5, 5 (5%) had anti-Mi-2, and 8 (6%) had anti-p155/TIF1-γ. Anti-MJ/NXP-2 was not found in our series of adult DM. Seventeen patients with anti-ARS had fever and 22 had arthritis and interstitial lung disease (ILD), compatible with a diagnosis of anti-ARS syndrome. Seventeen of 19 (89%) with anti-CADM-140/MDA5 had ILD, 16 (84%) of whom developed rapidly progressive ILD (RP-ILD). Four of 5 (80%) with anti-Mi-2 had heliotrope rash and/or Gottron’s sign/papules, and 2 (40%) had V-sign and/or shawl-sign rash, whereas no ILD or malignancy was detected. As seen with anti-Mi-2-positive patients, a low frequency of ILD (13%) was found in patients with anti-p155/TIF1-γ but 6 of 8 (75%) had malignancy during their course. The frequency of ILD was significantly higher in patients with anti-ARS or anti-CADM-140/MDA5 compared with anti-Mi-2 or anti-p155/TIF1-γ (81% and 89%, respectively). It should be noted that anti-CADM-140/MDA5-positive patients suffered significantly more RP-ILD compared to patients with anti-ARS (84% vs. 7%, P < 0.0001). On the other hand, anti-p155/TIF1-γ positive patients had a significantly higher rate of malignancy compared with anti-ARS-, anti-CADM-140/MDA5-and anti-Mi-2-positive patients (75% vs. 7%: P = 0.0004, 5%: P = 0.0006, 0%: P = 0.02, respectively). Conclusions: These results indicate that in addition to antibodies previously identified as specific for DM, autoantibodies newly found in these patients are useful for stratifying them into clinical subgroups.
文摘To the best of our knowledge, this is the first report in the literature showing the efficacy of transcranial direct current stimulation (tDCS) in treating refractory post-herpetic neuralgia in a patient with dermatomyositis. In addition, our results showed that tDCS sessions are safe and do not lead to disease relapse. Therefore, despite the limitations of being a case report, this study provides encouraging results that need to be widely explored in studies with a representative number of patients and with an appropriate design for patients with this systemic rheumatic autoimmune disease.
文摘Background: Calcinosis is an important sequela of JDM which may cause significant morbidity and mortality. There is no standard curative treatment for calcinosis but different agents were used with variable efficacy. We report the favorable outcome of rituximab on severe calcinosis in 4 JDM patients and present their clinical data. Patients and Methods: A retrospective chart review of 4 children with JDM and severe calcinosis who received rituximab for relapsing or polycyclic JDM course. Diagnosis and follow up of calcinosis was clinically and by X-ray. Review data included: age of patients at onset of JDM symptoms and diagnosis, clinical and laboratory criteria at diagnosis, disease course and duration of follow up. Data about calcinosis onset, sites, severity and its progression were also included. Further data about rituximab therapy included: dosage, side effects, other treatment used before, during or after this drug and outcome and duration of follow up of calcinosis after therapy. Results: 4 patients (2 male, 2 female), interval between onset of symptoms and diagnosis was 6 - 12 months, course of JDM was polycyclic or relapsing, duration of follow up was 5 - 7 years. Calcinosis was severe causing ulceration, recurrent skin infections and joint limitation. It was not improving despite treatment with different DMARDs and/or bisphosphonates, colchicine and warfarin. Reason to start rituximab was inadequate disease control with conventional DMARDs. All patients received steroids and more than one DMARD before starting rituximab and were continued thereafter, follow up after rituximab was 3 to 5 years. All patients had improvement in disease activity and frequency of admission especially due to complications of calcinosis. One patient had complete clearance of calcinosis for the last 5 years. Others had significant improvement in calcinosis with no new lesions, decreased sites and density and decreased calcinosis related contractures. There were no serious side effects to rituximab. Conclusion: Our study showed the favorable effect of rituximab in treatment of calcinosis in 4 patients with JDM-associated severe calcinosis when it was used with other conventional DMARDs.
