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

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

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

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

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

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

Dermatomyositis associated with gallbladder carcinoma:A case report

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

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

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.

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

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

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

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

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

有丙肝的一个 79 岁的女病人病毒相关的肝肝硬化作为与 2.0 厘米的一条直径有肝细胞癌(HCC ) 被诊断。她为 HCC 拒绝了治疗。在诊断以后的九个月，当 HCC 扩大了到 6.0 厘米的一条直径时，她开发了皮肤肌炎。她为皮肤肌炎，然后 transcatheter 经历了治疗为 HCC 的动脉的 chemoembolization。尽管皮肤肌炎的表明改善了，全部肿瘤坏死被完成，她在 HCC 的治疗以后死于肺病 2 瞬间。HCC 或长期的丙肝病毒感染可能涉及皮肤肌炎的致病。

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

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

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

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

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

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

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

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

Paraneoplastic Dermatomyositis Accompanying Nasopharyngeal Carcinoma

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

Carcinoma of unknown primary and paraneoplastic dermatomyositis

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.

Tofacitinib as a Treatment for Refractory Dermatomyositis: A Case Report

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.

Effectiveness of Rituximab Therapy on Severe Calcinosis in 4 Children with Juvenile Dermatomyositis

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.

Transcranial Direct Current Stimulation Is Safe and Relieves Post-Herpetic Neuralgia in Patient with Dermatomyositis: A Case Report

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.

Use of Rituximab in Refractory Dermatomyositis: A Case Report

We report a case of a 56-year-old female with a confirmed diagnosis of Dermatomyositis on muscle biopsy, which was refractory to 5 different disease modifying drugs as well as intravenous immunoglobulins and symptoms improved dramatically with a single course of Rituximab. We hereby wish to highlight the importance of Rituximab in this highly resistant case and that anti CD 20 biologic drugs can be considered as standard treatment protocol in refractory Dermatomyositis.

Anterior segment parameters associated with extramuscular manifestations in polymyositis and dermatomyositis

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

Dermatomyositis Associated with Docetaxel Use in the Treatment for Ovarian Cancer

A 50-year-old woman presented with a 1-week history of rash on the face and hands and difficulty when rising from a sitting position. She underwent an exploratory laparotomy for ovarian cancer 8 months ago. Three months ago, she underwent total hysterectomy, adnexectomy, and retroperitoneal lymph node dissection. Docetaxel and carboplatin were started and administered twice. Physical examination revealed periorbital edema with a purplish appearance;Erythematous lesions on radiodorsal aspects of the proximal interphalangeal and metacarpophalangeal joints were seen. We diagnosed as dermatomyositis on the basis of clinical findings. Interview demonstrated that such rashes were enhanced 1 week after chemotherapy, and got better through following one week. Cutaneous and muscular symptoms got worse two months after her initial visit. Myotonia was found in electromyography, and high density areas on both femoral regions and lower thighs were observed by MRI of skeletal muscle. Scleroderma-like lesions are known as an adverse effect to taxane. In addition, three cases of dermatomyositis due to taxane were reported in Japan. Since dermatomyositis was regarded as one of paraneoplastic syndrome generally, it was not considered as drug-induced. However, clinical course of our case suggests that docetaxel may play more important role in activity of dermatomyositis than ovarian cancer. Seeing patients treated with taxane, therefore, we have to be careful for symptoms suggestive of autoimmune diseases: not only scleroderma but dermatomyositis.