Polymyositis Patient Research Articles

A Case-report of Concurrent Pulmonary and Cerebral Lesions in a Patient with Polymyositis: Invasive Aspergillosis or Astrocytoma?

Introduction: Polymyositis is an idiopathic inflammatory myopathy that mainly manifests itself with muscle weakness. Patients with polymyositis have a higher risk of developing infections and malignancies. We report concurrent pulmonary and cerebral lesions in a polymyositis patient with many diagnostic challenges. Case Presentation: A 56-year-old woman complained of a productive cough and dyspnea from two weeks ago. Her symptoms gradually progressed until a sudden loss of consciousness occurred. She was a known case of polymyositis and was treated with oral prednisolone. Imaging revealed concurrent pulmonary and cerebral lesions. Initially, the patient underwent empirical therapy. However, the patient underwent a bronchoscopy because she did not respond to treatment. Specimens obtained from respiratory secretions revealed branched septate hyphae, and the culture was positive for Aspergillus fumigatus. She was diagnosed with invasive aspergillosis, so we replaced the therapy with voriconazole. After three months, the lung lesions improved, but the number and extent of cerebral lesions increased. Finally, after a stereotactic biopsy, the patient was diagnosed with astrocytoma and became a candidate for radiotherapy. Conclusions: Patients with polymyositis are prone to contracting opportunistic infections and malignancies. Both of them can mimic each other and present diagnostic challenges to physicians. Thus, they should think about them for early diagnosis and timely treatment.

Increased Incidence of Amyotrophic Lateral Sclerosis in Polymyositis: A Nationwide Cohort Study.

Past studies have shown common pathologic characteristics and shared immunologic features between polymyositis (PM) and amyotrophic lateral sclerosis (ALS). To explore the potential relationship between the 2 diseases, we performed a nationwide cohort study. We identified all newly diagnosed patients with PM from Taiwan's Registry of Catastrophic Illness Database between January 1, 1998 and December 31, 2011. Each PM patient was matched to ≤5 control patients from the National Health Insurance Research Database by sex, age, and entry date. Cumulative incidence of ALS was calculated by the Kaplan-Meier method and compared using the log rank test. Cox hazard regression was used to calculate the hazard ratio of ALS. A total of 1,778 PM patients and 8,124 control patients were enrolled. PM patients had a higher cumulative incidence of ALS (P < 0.001). There was a positive correlation in being diagnosed with ALS in patients previously diagnosed with PM when stratified by sex. Consistent trends were conserved across different age strata. The strength of this association remained statistically significant after adjusting for sex, age, and concomitant autoimmune diseases (hazard ratio 25.72 [95% confidence interval 2.95-224.58]; P = 0.003). This study demonstrates that a diagnosis of PM increased the likelihood of a subsequent ALS diagnosis, independent of sex, age, and concomitant autoimmune diseases. Future studies are warranted to clarify the underlying biologic mechanisms and to translate them into clinical therapeutic options.

A Case of Polymyositis Presenting as Bent Spine Syndrome

Polymyositis (PM) is a subset of idiopathic inflammatory myopathies. The muscles involved with PM are typically proximal and distal limb muscles, but paraspinal muscles are rarely affected. The primary PM clinical symptom is gradual proximal muscle weakness but unusually abnormal trunk posture. Bent spine syndrome (BSS), also referred to camptocormia, is defined as an abnormal flexion of the trunk, appearing in standing position. An idiopathic axial myopathy is the most common cause of primary BSS. A few cases of inflammatory myopathy, a secondary BSS, have been reported. We describe a 59-year–old polymyositis patient with normal finding on an magnetic resonance imaging femur scan who presented with BSS only, myopathic findings on electromyography and elevation of muscle enzymes. (J Rheum Dis 2016;23:261-265)

AB0205 Expression of Inflammasomes is Different in Muscle of Dermatomyositis and Polymyositis Patients

BackgroundActivation of innate immune plays important role in the pathogenesis of autoimmune disease. Nod-like receptors (NLRs) are cytosol-expressed pattern recognition receptors that can sense endogenous cellular products associated with tissue...

THU0449 Utility of Ultrasound (US) in Assessing Skeletal Muscle Architecture in Idiopathic Inflammatory Myopathies (IIM)

BackgroundIIM are systemic autoimmune diseases characterized by chronic inflammation in skeletal muscle leading to proximal muscle weakness. In rheumatoid arthritis, muscle weakness has been linked to specific changes in muscle...

Symptoms and signs of polymositis, systematic lupus erythematosus and antiphospholipid syndrome follow consecutively

Polymyositis (PM) and dermatomyositis (DM) are very rare connective tissue disorders which only in exceptional circumstances affect white men. The present paper describes the case of an obese 55-years-old man in whom no muscular-skeletal system symptoms were found during the period of 2 years before the onset of arthritis, and who was treated because of cardiac involvement (pericarditis, PAF), pleuritis, malaise and fever. Only the occurrence of non erosive arthritis decided on the connective tissue disorder as a cause of his complaints. Previously the diagnostics was based on the exclusion of malignant diseases and bacterial and viral infections. The determination of antinuclear antibodies in high titer without any specific, typical of individual disease entities antinuclear antibodies allowed only the diagnose of undifferentiated connective tissue disease. Glucocorticosteroid treatment was initiated, however six months later despite treatment with metyloprednisolone the symptoms and signs associated with PM accompanied by high level of creatinophosphokinase and elevated transaminase were found. The muscle biopsy revealed myositis, but no specific antibodies, especially anti-Jo-1, were detected. It is very interesting that cyclophosphamide + glucocorticoids pulse therapy failed to prevent the development of antiphospholipide syndrome and interstitial pulmonary fibrosis in the PM patient. Overlapping of immunological tissue disorders is a well-known phenomenon, but in the case of fulminating and distinct symptoms and signs the lack of detection of specific antibodies is quite uncommon.

P2.47 A polymyositis patient with anti-signal recognition particle (SRP) antibody refractory to corticosteroid treatment

P2.47 A polymyositis patient with anti-signal recognition particle (SRP) antibody refractory to corticosteroid treatment

Treatment-resistant inflammatory myopathy

When confronted with a dermatomyositis or polymyositis patient not responding to immunosuppressive treatment, physicians must first ask whether the original diagnosis was correct. In this review, we provide a guide to the clinical features and ancillary tests, which might be helpful in the differential diagnosis of myositis. Particular attention is paid to the role of autoantibody detection, as some of them are not only relatively specific for the disease but also associated with unique clinical features including resistance to treatment. Subsequently, other possible explanations of treatment resistance are listed and a short overview of treatment options for resistant myositis patients is given.

An unusual association: anti-Jo1 and anti-SRP antibodies in the serum of a patient with polymyositis

The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic inflammation, resulting in the progressive weakness of the proximal muscles. Myositis-specific or myositis-associated autoantibodies can often be found in serum of polymyositis and dermatomyositis patients. This autoantibody presence may play a significant role in patient diagnosis and classification. We present a female polymyositis patient characterized with serious muscle weakness and lung involvement. Anti-Jo1 antibodies were detected in the patient's serum at the time of diagnosis. After 5 years of treatment and surveillance, recent laboratory analysis showed the presence anti-SRP antibody in her serum.

Thoracic Epidural Anesthesia for a Polymyositis Patient Undergoing Awake Mini-thoracotomy and Unroofing of a Huge Pulmonary Bulla

General anesthesia with one-lung ventilation is a conventional anesthetic strategy for most chest surgery, including resection of pulmonary bullae. However, this anesthetic management may cause alveolar barotrauma, hemodynamic instability, pulmonary atelectasis and long-term ventilator dependency. Here, we report a 64-year-old female with polymyositis and bronchiolitis obliterans organizing pneumonia who was scheduled for surgical intervention for a huge pulmonary bulla over the right upper lung. Under thoracic epidural anesthesia, with the patient maintaining clear consciousness and spontaneous breathing, a mini-thoracotomy was accomplished to unroof and partially resect the bulla. There were no perioperative complications, and the patient was satisfied with the anesthetic care. Pulmonary function tests and daily physical performance also improved postoperatively.

An Analysis of a Severe Neck Pain Experienced in a Polymyositis Patient

Following a twisting strain with immediate pain in the neck of a polymyositis patient we detail the analysis of a specific range of data gathered before and after the event. The results reinforce that in this case that the creatine kinase isoenzyme and cardiac Troponin T cannot be used as an indicator of myocardial disease.

A method for extracting muscle information from serum creatine kinase measurements – Its potential value in the monitoring and management of inflammatory muscle disease

A major problem in treating inflammatory myopathies is the very limited information available about the processes taking place. For instance serum creatine kinase levels are often dismissed as a means of gaining an understanding of what is happening or a means of monitoring a patient. This paper shall show how serum creatine kinase levels may be used to explore the processes taking place that create muscle inflammation. This is achieved by a detailed longitudinal study. Firstly, considerable laboratory data is gathered such as serum creatine kinase levels and blood pressure information. Secondly, the data is quantified using a scientific model. We shall illustrate the approach through an extensive nine-year study of a particular polymyositis patient. We identify three basic processes that may contribute to muscle inflammation and show how they may be interpreted from specific patient data. Furthermore, details are given for controlling and monitoring the disease to maximise the reduction in the muscle attack while reducing significantly the muscle inflammation and keeping the drug concentration at a minimum to ensure minimum side-effects and how to identify and handle drug-drug, drug-natural product and adverse drug interactions. Examples are given for a natural product, azathioprine and trandolapril.

Rhabdomyolysis and acute renal failure in a polymyositis patient

Rhabdomyolysis and acute renal failure in a polymyositis patient

Polymyositis—a further investigation

In essence, this paper describes the model-based interpretation of measurements in order to gain insight into physiological processes. The paper extends the mathematical model developed previously to control and monitor a polymyositis patient. The extension considers two further yet different types of muscle attack. The model is applied to simulate, first, the total serum creatine kinase levels, which then are matched to the observed values. The outcomes of this approach yield considerable information about the reactions taking place including various concentration levels over time such as prednisolone. The changes in the muscle attack are estimated. Examples illustrate how the model may be used to assess, for a single patient, the maximum reduction of the muscle attack whilst keeping the prednisolone concentration as low as possible. The model has been successfully used to interpret variations in daily heart rate measurements of a polymyositis patient. Consequently, it is shown how daily heart rate measurements may now be used to complement the control and monitoring of the polymyositis patient. The model is used to explore multi-patient statistics. It shows the dangers of using such an approach. The model illustrates the need for longitudinal studies.

Candida pneumonia in a case of polymyositis complicated with interstitial pneumonitis

We report a polymyositis patient who developedCandida pneumonia, whose clinical manifestations such as dry cough, dyspnea, fever and elevated LDH looked like a recurrence of interstitial pneumonitis (IP) which she had had at admission. The distribution of the lesions in high resolution computed tomography scan, the high serum level of β-d-glucan and positiveCandida antigen test led to make a diagnosis ofCandida pneumonia rather than IP, and fluconazole therapy was successful.

Candidapneumonia in a case of polymyositis complicated with interstitial pneumonitis

We report a polymyositis patient who developed Candida pneumonia, whose clinical manifestations such as dry cough, dyspnea, fever and elevated LDH looked like a recurrence of interstitial pneumonitis (IP) which she had had at admission. The distribution of the lesions in high resolution computed tomography scan, the high serum level of β-d-glucan and positive Candida antigen test led to make a diagnosis of Candida pneumonia rather than IP, and fluconazole therapy was successful.

Wide cross-species aminoacyl-tRNA synthetase replacement in vivo: yeast cytoplasmic alanine enzyme replaced by human polymyositis serum antigen.

Because of variations in tRNA sequences in evolution, tRNA synthetases either do not acylate their cognate tRNAs from other organisms or execute misacylations which can be deleterious in vivo. We report here the cloning and primary sequence of a 958-aa Saccharomyces cerevisiae alanyl-tRNA synthetase. The enzyme is a close homologue of the human and Escherichia coli enzymes, particularly in the region of the primary structure needed for aminoacylation of RNA duplex substrates based on alanine tRNA acceptor stems with a G3.U70 base pair. An ala1 disrupted allele demonstrated that the gene is essential and that, therefore, ALA1 encodes an enzyme required for cytoplasmic protein synthesis. Growth of cells harboring the ala1 disrupted allele was restored by a cDNA clone encoding human alanyl-tRNA synthetase, which is a serum antigen for many polymyositis-afflicted individuals. The human enzyme in extracts from rescued yeast was detected with autoimmune antibodies from a polymyositis patient. We conclude that, in spite of substantial differences between human and yeast tRNA sequences in evolution, strong conservation of the G3.U70 system of recognition is sufficient to yield accurate aminoacylation in vivo across wide species distances.

こう原病患者に対する血しょう交換療法

Plasma exchange was performed in a total 18 patients of 11 rheumatoid arthritis (RA), 3 progressive systemic sclerosis (PSS), 1 polymyositis (PM), 1 dermatomyositis (DM) and 2 mixed connective tissue disease (MCTD) . Serum levels of immunoglobulins and complement and also numbers of lymphocyte subsets and its function were measure before and after treatment. Plasma separator AP-05-H (ASAHI Medical Co.) with menbrane filters was used as substitute for the exchanged plasma of 2, 000 to 3, 000ml in each trial.The treatment seemed to be effective in 9 of 11 RA patients and two of which showed a disappearance of their subcutaneous nodules. The Skin ulcers in the legs found in 2 PSS patients decreased in their size and sclerosis of the skin found in one PSS patient relieved. Respiratory disorder found in one PM patient improved. Muscle weakness and histologic finding of myositis found in one DM patient improved. Severe myalgia and muscle weakness in one MCTD patient improved.In laboratory data, serum levels of total protein, γ-globulin, immunoglobulin class of IgG, IgA and IgM, C3 and C4 decreased significantly. Decrease of numbers of OKT-8 positive cells and increase of the ratio of OKT-4/OKT-8 positive cell were significant.

Coxsackievirus Aggregates in Muscle Cells of a Polymyositis Patient

Muscle biopsy specimens of a patient with polymyositis showed crystalline structures resembling picornavirus aggregates within muscle cells. The patient's serum reacted in an indirect immunofluorescence assay with autologous muscle cells. A strongly positive immunofluorescence staining was also noted when a section of muscle tissue was reacted with coxsackievirus A9 antiserum, and a weakly positive reaction was noted with coxsackievirus B2 antiserum. No staining was observed after treatment with antiserum to poliovirus type 1 or echovirus types 11 and 22.