Management Of Polymyositis Research Articles

Overview on Diagnosis and Management of Polymyositis

Polymyositis (PM) is an autoimmune disorder; result from abnormal activation of cytotoxic T lymphocytes (CD8 cells) and macrophages against muscular antigens as well as the strong extrafusal muscular expression of major histocompatibility complex class 1 causing damage to the endomysium of the skeletal muscles. Polymyositis develops over the months as compared to inclusion body myositis (IBM), which is a slowly progressive chronic myopathy developing in older individuals over a period of months to years with more severe symptoms. Many patients require treatment for many years. Polymyositis affects the distal musculature of the esophagus in the late stage of disease in up to 70% of the patients leading to the inability to swallow, as well as regurgitation problems that can cause aspiration pneumonia. The principal goals of therapy are to improve strength and improve physical functioning. Many patients require treatment for several years. The 5-year survival rate for treated patients is in the order of 95%. Up to one-third of PM patients may be left with some degree of residual muscle weakness.

The efficacy of tacrolimus in patients with refractory dermatomyositis/polymyositis: a systematic review.

The purpose of this study is to examine the efficacy and safety of tacrolimus (FK506) in the management of polymyositis (PM)/dermatomyositis (DM). The Cochrane Central Register of Controlled Trials (CENTRAL), PubMed, Embase, and China National Knowledge Infrastructure (CNKI) were searched to find articles published between May 1980 and April 2015 concerning tacrolimus therapy in PM/DM. The initial search yielded 107 articles. In the end, eight studies met our inclusion criteria and involved a total of 134 patients who received tacrolimus therapy for DM/PM. All studies were non-randomized. Oral tacrolimus of 0.075 mg/kg/day or 1.0-3.5 mg/d was administered twice daily or once daily together with glucocorticoids (GCs). According to comprehensive analysis of the studies, 93.3 % (42/45) and 64.7 % (11/17) of patients showed improvement in muscle strength and physical function status. The creatine kinase (CK) levels of 100 % (68/68) of patients decreased. The average dosage of GCs was reduced from 33.8 to 11.5 mg/day in PM/DM patients after the addition of tacrolimus. In the subject population, 65 patients had interstitial lung disease (ILD). After treatment, the forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) improved or stabilized in 89.3 % (25/28) and 81.3 % (13/16) of patients, respectively. The commonly adverse events were nephrotoxicity, hypomagnesemia, tremors, and hypertension, but they were slight among these patients. Current evidence appears to support the use of tacrolimus in refractory PM/DM and PM/DM-ILD patients. Tacrolimus seems to be a safe drug that improves both muscle strength and lung function, and it is well tolerated by patients. However, this conclusion should be confirmed by large-sample, randomized controlled studies.

THU0496 [18F] Fdg Uptake in Proximal Muscles Assessed by Pet/Ct Reflects Both Global and Local Muscular Inflammation and Provides Useful Information in the Management of Patients with Polymyositis/Dermatomyositis

BackgroundAlthough the utility of [18F] fluoro-deoxy-glucose-positron emission tomography/computed tomography ([18F] FDG-PET/CT) in the management of rheumatic or inflammatory diseases has been increasingly reported, its applications and benefits in the management...

Polymyositis and myocarditis after chemotherapy for advanced thymoma

Abstract Polymyositis and myocarditis very rarely develop during chemotherapy for thymoma. Most reported cases of myocarditis and polymyositis associated with thymoma were found at autopsy of patients who died of acute progression of myocarditis. We describe our experience with a 64-year-old man who had recurrent thymoma accompanied by polymyositis and myocarditis. Lower-extremity myalgia and palpitations developed on day 25 of chemotherapy with weekly paclitaxel. Steroid pulse therapy was effective for the management of polymyositis and myocarditis associated with thymoma. Polymyositis and myocarditis after paclitaxel monotherapy have not been documented previously. Whether paclitaxel induced polymyositis and myocarditis is unclear and these symptoms might have been a paraneoplastic phenomenon associated with thymoma. However, our experience suggested that patients with thymoma who received paclitaxel-based chemotherapy should be carefully observed for polymyositis and myocarditis. If such patients have high serum creatine phosphokinase and troponin levels, steroid pulse therapy should be considered without delay.

Evaluation and management of polymyositis

Polymyositis (PM) is one of the inflammatory myopathies, disorders characterized pathologically by the presence of inflammatory infiltrates in striated muscle. The principal clinical manifestation of PM is proximal muscle weakness. The cause of PM is unknown, but current evidence suggests that it is an autoimmune disorder. PM can affect people of any age, but most commonly presents between the ages of 50 to 70. PM is rarely seen in people younger than 18 years of age, and is twice as common among females than males. PM is more common in blacks than in whites. The overall prevalence of PM is 1 per 100,000. Muscle weakness may develop suddenly or more insidiously over a period of weeks to months. The classic symptom of PM is proximal weakness, which may manifest as difficulty holding the arms over the head, climbing stairs, or rising from a chair. Weakness of the striated muscle of the upper esophagus may result in dysphagia, dysphonia, and aspiration. The chest wall muscles may be affected, leading to ventilatory compromises. Involvement of cardiac muscle may lead to arrhythmias and congestive heart failure. Dermatomyositis (DM) is closely related to PM, and both are distinguished primarily by the occurrence of characteristic skin abnormalities in the former. PM and DM may be associated with a variety of malignancies. PM may also occur as part of the spectrum of other rheumatic diseases like systemic lupus erythematosus and mixed connective tissue disease. Moreover, inflammatory myopathy may be caused by some drugs (procainamide, D-penicillamine), and viruses, most notably the retroviruses. Corticosteroids and immunosuppressive agents are the mainstays of therapy for PM. The principal goals of therapy are to improve strength and improve physical functioning. Many patients require treatment for several years. The 5-year survival rate for treated patients is in the order of 95%. Up to one-third of PM patients may be left with some degree of residual muscle weakness.

Cyclosporin in the management of polymyositis and dermatomyositis.

Three patients with polymyositis refractory to conventional steroid and immunosuppressive treatment, but responsive to cyclosporin A, are described. In a fourth patient cyclosporin A was used as a first line drug in combination with steroids in the treatment of life-threatening dermatomyositis. Cyclosporin A in the management of polymyositis/dermatomyositis requires formal assessment of its costs and benefits compared with conventional treatments.

Current management of polymyositis and dermatomyositis.

This article reviews the clinical features and emphasises the treatment of polymyositis and dermatomyositis. Pharmacological intervention with corticosteroids and immunosuppressive agents is discussed. In addition, strategies regarding the initiation and subsequent tapering of these drugs are provided, and an algorithmic approach to the management of myositis is provided. Therapeutic modalities for patients with refractory disease are considered, and the potential adverse effects of such treatment are discussed. Since patients with myositis often have disease-related complaints other than muscle weakness, a practical treatment approach for these problems is also outlined.

13 - Management of Polymyositis and Dermatomyositis

The overall prognosis for polymyositis and dermatomyositis has improved with the judicious use of corticosteroids, while azathioprine and other cytotoxic agents are of value in improving functional ability and decreasing the total steroid requirement. However, the duration of therapy and the relative risks of it must be under constant surveillance. Problems such as respiratory or cardiac failure in the course of the disease are of serious importance and require particular care in management . Finally, the possibility of neoplasm underlying the disease or occurring as a sequel to cytotoxic therapy should always be borne in mind.

Evaluation of laboratory tests in diagnosis and management of polymyositis.

Evaluation of laboratory tests in diagnosis and management of polymyositis.