PATIENT FACT SHEET Myopathies

Total Page:16

File Type:pdf, Size:1020Kb

PATIENT FACT SHEET Myopathies Inflammatory PATIENT FACT SHEET Myopathies Inflammatory myopathies are muscle diseases caused skin rashes also. Muscle pain is not a common symptom. by inflammation. They are autoimmune diseases where Some people can have breathing problems. the body’s immune system attacks its own muscles by People of all ages and races may get inflammatory mistake. The most common inflammatory myopathies are myopathies, but they’re rare. Children usually get them polymyositis and dermatomyositis. between ages 5 and 10. Adults usually get these diseases CONDITION Inflammatory myopathies cause muscle weakness, usually between 40 and 50. Women get inflammatory myopathies DESCRIPTION in the neck, shoulders and hips. Dermatomyositis causes twice as often as men. The most common sign of inflammatory myopathies is • Shortness of breath weakness in the large muscles of the shoulders, neck or • Cough hips. Inflammation damages tissue so you lose strength Dermatomyositis causes skin rashes that look like red or in these muscles. Inflammatory myopathies may cause purple spots on the eyelids, or scaly, red bumps on the problems like these: elbows, knuckles or knees. Children may also have white • Trouble climbing stairs, lifting objects over your head spots on their skin called calcinosis or vasculitis, a blood or getting out of a seat vessel inflammation that causes skin lesions. SIGNS/ • Choking while eating or intake of food into the lungs SYMPTOMS Diagnosing inflammatory myopathies starts with (Deltasone, Orasone), to reduce inflammation. Muscle a muscle strength exam. A rheumatologist may also enzymes usually return to normal at 4 to 6 weeks, and do blood tests to measure certain muscle enzymes or strength returns in 2 to 3 months. myositis-specific antibodies. An electromyogram (EMG) Your doctor may add methotrexate (Rheumatrex, Trexall, can measure electrical activity in the muscles. You may Orastrup, Rasuvo) or azathioprine (Imuran) to your need a muscle biopsy or an MRI to look for muscle damage. treatment plan for long-term disease control. Some These diseases may be linked to cancer, so tests to rule out patients with severe disease may need intravenous cancer may be needed too. COMMON immunoglobulin, cyclosporine (Neoral, Sandimmune), TREATMENTS The first treatment for inflammatory myopathies is tacrolimus (Prograf), mycophenolate mofetil (CellCept) a highdose, oral corticosteroid, such as prednisone or rituximab (Rituxan). Good health habits are important for long-term swallowing, eat soft foods or puree solid foods in a management of inflammatory myopathies. Eat a blender. To prevent choking if you are bedbound, sit healthy, well-balanced diet, get regular exercise and stay up in bed to eat. at a healthy weight. Corticosteroids can cause serious side effects like high People with dermatomyositis should use sun protection blood pressure, weight gain, thinning skin or bruising, CARE/ so rashes don’t worsen. Limit your time outdoors and cataracts or osteoporosis. Get regular exams to watch MANAGEMENT use sunscreen when you go outside. If you have trouble for or treat these problems. TIPS Updated March 2019 by Vaneet Sandhu, MD, and reviewed by the American College of Rheumatology © 2019 American College of Rheumatology Committee on Communications and Marketing. This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition. www.ACRPatientInfo.org.
Recommended publications
  • Myositis Center Myositis Center
    About The Johns Hopkins The Johns Hopkins Myositis Center Myositis Center he Johns Hopkins Myositis Center, one Tof the first multidisciplinary centers of its kind that focuses on the diagnosis and management of myositis, combines the ex - pertise of rheumatologists, neurologists and pulmonologists who are committed to the treatment of this rare disease. The Center is conveniently located at Johns Hopkins Bayview Medical Center in Baltimore, Maryland. Patients referred to the Myositis Center can expect: • Multidisciplinary Care: Johns Hopkins Myositis Center specialists make a diagno - sis after evaluating each patient and re - viewing results of tests that include muscle enzyme levels, electromyography, muscle biopsy, pulmonary function and MRI. The Center brings together not only physicians with extensive experience in di - agnosing, researching and treating myosi - tis, but nutritionists and physical and occupational therapists as well. • Convenience: Same-day testing and appointments with multiple specialists are The Johns Hopkins typically scheduled to minimize doctor Myositis Center visits and avoid delays in diagnosis and Johns Hopkins Bayview Medical Center treatment. Mason F. Lord Building, Center Tower 5200 Eastern Avenue, Suite 4500 • Community: Because myositis is so rare, Baltimore, MD 21224 the Center provides a much-needed oppor - tunity for patients to meet other myositis patients, learn more about the disease and Physician and Patient Referrals: 410-550-6962 be continually updated on breakthroughs Fax: 410-550-3542 regarding treatment options. www.hopkinsmedicine.org/myositis The Johns Hopkins Myositis Center THE CENTER BRINGS TOGETHER Team NOT ONLY PHYSICIANS WITH EXTENSIVE EXPERIENCE IN DIAGNOSING , RESEARCHING AND Lisa Christophe r-Stine, TREATING MYOSITIS , BUT M.D., M.P.H.
    [Show full text]
  • Focal Eosinophilic Myositis Presenting with Leg Pain and Tenderness
    CASE REPORT Ann Clin Neurophysiol 2020;22(2):125-128 https://doi.org/10.14253/acn.2020.22.2.125 ANNALS OF CLINICAL NEUROPHYSIOLOGY Focal eosinophilic myositis presenting with leg pain and tenderness Jin-Hong Shin1,2, Dae-Seong Kim1,2 1Department of Neurology, Research Institute for Convergence of Biomedical Research, Pusan National University Yangsan Hospital, Yangsan, Korea 2Department of Neurology, Pusan National University School of Medicine, Yangsan, Korea Focal eosinophilic myositis (FEM) is the most limited form of eosinophilic myositis that com- Received: September 11, 2020 monly affects the muscles of the lower leg without systemic manifestations. We report a Revised: September 29, 2020 patient with FEM who was studied by magnetic resonance imaging and muscle biopsy with Accepted: September 29, 2020 a review of the literature. Key words: Myositis; Eosinophils; Magnetic resonance imaging Correspondence to Dae-Seong Kim Eosinophilic myositis (EM) is defined as a group of idiopathic inflammatory myopathies Department of Neurology, Pusan National associated with peripheral and/or intramuscular eosinophilia.1 Focal eosinophilic myositis Univeristy School of Medicine, 20 Geu- mo-ro, Mulgeum-eup, Yangsan 50612, (FEM) is the most limited form of EM and is considered a benign disorder without systemic 2 Korea manifestations. Here, we report a patient with localized leg pain and tenderness who was Tel: +82-55-360-2450 diagnosed as FEM based on laboratory findings, magnetic resonance imaging (MRI), and Fax: +82-55-360-2152 muscle biopsy. E-mail: [email protected] ORCID CASE Jin-Hong Shin https://orcid.org/0000-0002-5174-286X A 26-year-old otherwise healthy man visited our outpatient clinic with leg pain for Dae-Seong Kim 3 months.
    [Show full text]
  • Dermatomyositis: Observations on the Use of Cairo-Glasgow Study Group
    Postgrad Med J: first published as 10.1136/pgmj.54.634.516 on 1 August 1978. Downloaded from Postgraduate Medical Journal (August 1978) 54, 516-527. Dermatomyositis: observations on the use of immunosuppressive therapy and review of literature Cairo-Glasgow Study Group AHMED EL- GHOBAREY GEZA BALINT M.R.C.P. M.D. (Budapest) KAREL DE CEULAER W. CARSON DICK M.D. (Leuven) M.D., M.R.C.P. W. W. BUCHANAN M.D., F.R.C.P. TAHSIN HADIDI* T. A. HASSAN* F.R.C.P. M.R.C.P. The Centre for Rheumatic Diseases, University Department ofMedicine, Royal Infirmary, Protected by copyright. Glasgow, Scotland, and *Maadi Armed Forces Hospital, and Azhar University, Cairo, Egypt Summary The response to therapy and the subsequent Seven young adults, six of whom were male, all clinical course are briefly described as follows. suffering from dermatomyositis unassociated with Patient N.D. was treated with corticosteroids. The malignancy are described. These patients were not course of therapy, clinical response and changes in adequately controlled with high doses of corti- serum muscle enzymes are summarized in Fig. 1. costeroids but all responded when immunosuppressive Intravenous dexamethasone was discontinued at the therapy was also given. nineteenth week and by the end of the twenty-first week the dose of prednisolone was reduced to 10 mg/ day. Introduction http://pmj.bmj.com/ Dermatomyositis (as the name suggests) is a The patient was discharged at the end of the syndrome consisting of polymyositis associ- twenty-fourth week. clinical One year later, the patient had a relapse, the ated with skin lesions (Pearson, 1966a; Currie and subsequent course and response to treatment are Walton, 1971).
    [Show full text]
  • The Impact of Hypermobility Spectrum Disorders on Musculoskeletal Tissue Stiffness: an Exploration Using Strain Elastography
    Clinical Rheumatology (2019) 38:85–95 https://doi.org/10.1007/s10067-018-4193-0 ORIGINAL ARTICLE The impact of hypermobility spectrum disorders on musculoskeletal tissue stiffness: an exploration using strain elastography Najla Alsiri1 & Saud Al-Obaidi2 & Akram Asbeutah2 & Mariam Almandeel1 & Shea Palmer3 Received: 24 January 2018 /Revised: 13 June 2018 /Accepted: 26 June 2018 /Published online: 3 July 2018 # International League of Associations for Rheumatology (ILAR) 2018 Abstract Hypermobility spectrum disorders (HSDs) are conditions associated with chronic joint pain and laxity. HSD’s diagnostic approach is highly subjective, its validity is not well studied, and it does not consider many of the most commonly affected joints. Strain elastography (SEL) reflects musculoskeletal elasticity with sonographic images. The study explored the impact of HSD on musculoskeletal elasticity using SEL. A cross-sectional design compared 21 participants with HSD against 22 controls. SEL was used to assess the elasticity of the deltoid, biceps brachii, brachioradialis, rectus femoris, and gastrocnemius muscles, and the patellar and Achilles tendon. SEL images were analyzed using strain index, strain ratio, and color pixels. Mean strain index (standard deviation) was significantly reduced in the HSD group compared to the control group in the brachioradialis muscle 0.43 (0.10) vs. 0.59 (0.24), patellar 0.30 (0.10) vs. 0.44 (0.11), and Achilles tendons 0.24 (0.06) vs. 0.49 (0.13). Brachioradialis muscle and patellar tendon’s strain ratios were significantly lower in the HSD group compared to the control group, 6.02 (2.11) vs. 8.68 (2.67) and 5.18 (1.67) vs.
    [Show full text]
  • Collagen Vascular Diseases: SLE, Dermatomyositis, Scleroderma, and MCTD
    Collagen Vascular Diseases: SLE, Dermatomyositis, Scleroderma, and MCTD Richard K. Vehe, MD,* Mona M. Riskalla, MD* *Division of Pediatric Rheumatology, Department of Pediatrics, University of Minnesota and the University of Minnesota Masonic Children’s Hospital, Minneapolis, MN Practice Gap Timely and accurate recognition of collagen vascular disorders (CVDs), and implementation of effective screening and referral processes for patients suspected of having a CVD, remain a challenge for many physicians. The result, too often, is unnecessary testing and referrals, and in some cases unnecessary anxiety for physicians, patients, and parents. Objectives After completing this article, readers should be able to: 1. Recognize the common clinical symptoms and signs of systemic lupus erythematosus, dermatomyositis, and scleroderma, and their distinction from common infectious mimics. 2. Recognize the testing that can clarify the likelihood of whether a child has a rheumatic disease, including the limited utility of early serologic testing for autoantibodies. 3. Recognize the prognosis and management objectives for these often- chronic disorders, and practical steps to ensure excellent outcomes. AUTHOR DISCLOSURE Drs Vehe and Riskalla have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/ INTRODUCTION investigative use of a commercial product/ device. Timely and accurate recognition of collagen vascular diseases (CVDs), and ABBREVIATIONS implementation of effective screening and referral processes for patients sus- ANA antinuclear antibody pected of having a CVD, remain challenging for many physicians. The result, too CTD connective tissue disease often, is unnecessary testing with questionable results, leading in some cases to CVD collagen vascular disease unnecessary referrals and anxiety for physicians, patients, and parents.
    [Show full text]
  • Beneath the Surface: Derm Clues to Underlying Disorders
    Christian R. Halvorson, MD; Richard Colgan, MD Department of Family and Beneath the surface: Derm clues Community Medicine, University of Maryland School of Medicine, Baltimore to underlying disorders [email protected] Dermatologic fi ndings are frequent indicators of The authors reported no potential confl ict of interest connective tissue disorders. Here’s what to look for. relevant to this article. any systemic conditions are accompanied by skin PRACTICE manifestations. Th is is especially true for connec- RECOMMENDATIONS Mtive tissue disorders, for which dermatologic fi nd- › When evaluating patients ings are often the key to diagnosis. with suspected cutaneous In this review, we describe the dermatologic fi ndings of lupus erythematosus, use some well-known connective tissue disorders. Th e text and multiple criteria—including photographs in the pages that follow will help you hone your histologic and immuno- diagnostic skills, leading to earlier treatment and, possibly, fl uorescent biopsy fi ndings better outcomes. and American College of Rheumatology criteria—to rule out systemic disease. C Lupus erythematosus: Cutaneous › Cancer screening with a and systemic disease often overlap careful history and physi- Lupus erythematosus (LE), a chronic, infl ammatory autoim- cal examination is recom- mended for all adult patients mune condition that primarily aff ects women in their 20s and whom you suspect of having 30s, may initially present as a systemic disease or in a purely dermatomyositis. C cutaneous form. However, most patients with systemic LE have some skin manifestations, and those with cutaneous › Suspect mixed connective LE often have—or subsequently develop—systemic involve- tissue disease in patients 1 with skin fi ndings charac- ment.
    [Show full text]
  • Dermatomyositis
    DERMATOMYOSITIS http://www.aocd.org Dermatomyositis (DM) is a rare inflammatory muscle disease that affects both the muscles as well as the skin. DM can affect people of all races, sex and age. Although it affects both males and females equally in childhood, it is more common in females in adults. The exact cause of DM is unknown but it is believed to result from an immune-mediated process triggered by outside factors (e.g. malignancy, drugs, and infectious agents) in genetically predisposed individuals. Dermatomyositis can occur with other connective tissue disorders such as systemic lupus erythematosus, rheumatoid arthritis, scleroderma, Sjogren’s syndrome, and mixed connective tissue disease. Patients with dermatomyositis usually present with complaints of tiredness and loss of energy. The skin changes occur before the onset of muscle disease in most patients. The earliest signs of skin manifestations may begin with a red to bluish-purple rash most commonly in the sun exposed areas (face, neck, shoulders, upper chest and back). The eyelids may get the typical purple rash known as the heliotrope rash. Early clinicians thought that this violaceous rash around the eyes reminded them of the color of a heliotrope flower, and thus referred to this as the ‘heliotrope sign’. The knuckles may have purple spots known as ‘Gottron’s papules’. The most important diagnostic feature of skin eruption of DM is poikiloderma, which is the pale, thin skin with blood vessels and dark spots located in the sun exposed areas. Hardened deposits of yellow to white lumps under the skin can develop especially in children and adolescents known as calcinosis.
    [Show full text]
  • Scleroderma, Myositis and Related Syndromes [4] Giordano J, Khung S, Duhamel A, Hossein-Foucher C, Bellèvre D, Lam- Blin N, Et Al
    Scientific Abstracts 1229 Ann Rheum Dis: first published as 10.1136/annrheumdis-2021-eular.75 on 19 May 2021. Downloaded from Scleroderma, myositis and related syndromes [4] Giordano J, Khung S, Duhamel A, Hossein-Foucher C, Bellèvre D, Lam- blin N, et al. Lung perfusion characteristics in pulmonary arterial hyper- tension and peripheral forms of chronic thromboembolic pulmonary AB0401 CAN DUAL-ENERGY CT LUNG PERFUSION hypertension: Dual-energy CT experience in 31 patients. Eur Radiol. 2017 DETECT ABNORMALITIES AT THE LEVEL OF LUNG Apr;27(4):1631–9. CIRCULATION IN SYSTEMIC SCLEROSIS (SSC)? Disclosure of Interests: None declared PRELIMINARY EXPERIENCE IN 101 PATIENTS DOI: 10.1136/annrheumdis-2021-eular.69 V. Koether1,2, A. Dupont3, J. Labreuche4, P. Felloni3, T. Perez3, P. Degroote5, E. Hachulla1,2,6, J. Remy3, M. Remy-Jardin3, D. Launay1,2,6. 1Lille, CHU Lille, AB0402 SELF-ASSESSMENT OF SCLERODERMA SKIN Service de Médecine Interne et Immunologie Clinique, Centre de référence THICKNESS: DEVELOPMENT AND VALIDATION OF des maladies autoimmunes systémiques rares du Nord et Nord-Ouest de THE PASTUL QUESTIONNAIRE 2 France (CeRAINO), Lille, France; Lille, Université de Lille, U1286 - INFINITE 1,2 1 1 1 J. Spierings , V. Ong , C. Denton . Royal Free and University College - Institute for Translational Research in Inflammation, Lille, France; 3Lille, Medical School, University College London, Division of Medicine, Department From the Department of Thoracic Imaging, Hôpital Calmette, Lille, France; 4 of Inflammation, Centre for Rheumatology and Connective
    [Show full text]
  • Diagnosis and Treatment of Dermatomyositis-Systemic Lupus
    Diagnosis and Treatment of Dermatomyositis-Systemic Lupus Erythematosus Overlap Syndrome Preston Williams1; Benjamin McKinney, MD2 1Texas A&M College of Medicine; 2Baylor University Medical Center Family Medicine Residency Introduction Case Description Discussion Dermatomyositis is an autoimmune condition classically A punch biopsy of her rash showed atrophic epithelium with This case of overlap syndrome between dermatomyositis and characterized by symmetric proximal muscle weakness, dyskeratotic keratinocytes, vacuolar interface changes, superficial systemic lupus erythematosus presents a rare but important inflammatory muscle changes, and dermatologic abnormalities.1 perivascular and lichenoid inflammation, and pigment challenge to the primary care physician. Our patient presented Several studies have shown that the inflammatory myopathies, incontinence consistent with systemic lupus erythematosus initially with arthralgias and fatigue, symptoms more such as dermatomyositis, commonly overlap with other (SLE). The patient was started on prednisone 40 mg daily for a 2- characteristic of systemic lupus erythematosus. However, these connective tissue disorders, significantly complicating the week taper to 10 mg and hydroxychloroquine 200 mg daily with symptoms were followed by a facial rash that involved the diagnosis.2 The reported incidence of overlap syndrome ranges marked improvement in symptoms. Further lab work-up was nasolabial folds and periorbital regions more in line with from 11% to 40% in patients diagnosed with significant
    [Show full text]
  • Myositis 101
    MYOSITIS 101 Your guide to understanding myositis Patients who are informed, who seek out other patients, and who develop helpful ways of communicating with their doctors have better outcomes. Because the disease is so rare, TMA seeks to provide as much information as possible to myositis patients so they can understand the challenges of their disease as well as the options for treating it. The opinions expressed in this publication are not necessarily those of The Myositis Association. We do not endorse any product or treatment we report. We ask that you always check any treatment with your physician. Copyright 2012 by TMA, Inc. TABLE OF CONTENTS contents Myositis basics ...........................................................1 Diagnosis ....................................................................5 Blood tests .............................................................. 11 Common questions ................................................. 15 Treatment ................................................................ 19 Disease management.............................................. 25 Be an informed patient ............................................ 29 Glossary of terms .................................................... 33 1 MYOSITIS BASICS “Myositis” means general inflammation or swelling of the muscle. There are many causes: infection, muscle injury from medications, inherited diseases, disorders of electrolyte levels, and thyroid disease. Exercise can cause temporary muscle inflammation that improves after rest. myositis
    [Show full text]
  • Inflammatory Myopathies B
    Current Treatment Options in Neurology DOI 10.1007/s11940-010-0111-8 Neuromuscular Disorders Inflammatory Myopathies B. Jane Distad, MD*,1 Anthony A. Amato, MD 2 Michael D. Weiss, MD 1 Address *,1Department of Neurology, Neuromuscular Division, University of Washing- ton, School of Medicine, Seattle, WA 98195, USA E-mail: [email protected] E-mail: [email protected] 2Department of Neurology, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA 02115, USA E-mail: [email protected] * Springer Science+Business Media, LLC 2011 Opinion statement The mainstay of treatment for the idiopathic inflammatory myopathies currently and traditionally has been therapeutics aimed at suppressing or modifying the immune system. Most therapies being used are directed towards polymyositis (PM) and dermatomyositis (DM), as there is yet to be efficacious treatment of any kind for inclusion body myositis (IBM), However, there are few randomized controlled studies supporting the use of such therapies even in PM and DM. Even in the absence of controlled studies, oral corticosteroids (in particular high-dose prednisone) continue to be the first-line medications used to manage these conditions. Second-line therapies include the addition of chronic, steroid-sparing immunosuppressive drugs such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, and mycophenolate mofetil. These drugs are typically added when patients are on corticosteroids for an extended period or when the disease is refractory. Such medications often allow corticosteroid dosages to be reduced, but monitoring is required for their own side effects, such as bone marrow suppression, kidney dysfunction, and respiratory concerns. Small controlled studies also support the role of intravenous immunoglobulin therapy as an alternative therapy, particularly for DM, though the cost of this treatment is sometimes prohibitive.
    [Show full text]
  • Multiple Myeloma Associated With
    ISSN: 2469-5696 Islam and Kotoucek. Int J Blood Res Disord 2018, 5:029 DOI: 10.23937/2469-5696/1410029 International Journal of Volume 5 | Issue 1 Open Access Blood Research and Disorders RESEARCH ARTICLE Multiple Myeloma Associated with Dermatomyositis: A Short Report and Mini-Review Md Serajul Islam1,2* and Pavel Kotoucek2 Check for 1Department of Haematology, Guys’ & St Thomas’ Hospital, London, UK updates 2Department of Haematology, Broomfield Hospital, Chelmsford, UK *Corresponding author: Md Serajul Islam, Department of Haematology, Guys’ & St Thomas’ Hospital, London, UK; De- partment of Haematology, Broomfield Hospital, Chelmsford, UK, Tel: +44-7769580452, Fax: +44-1245-51-6669, E-mail: [email protected] amyloid deposits in various tissues [3]. The American Abstract Cancer Society has estimated that 30,770 new MM Multiple myeloma (MM) is characterized by the neoplastic proliferation of plasma cell clones that produce monoclo- cases will occur in the United States in 2018, with an nal immunoglobulin. Dermatomyositis (DM), and to a lesser estimated 12,770 deaths [4]. Idiopathic inflammatory extent polymyositis (PM), carry a higher risk of cancer than myopathies (IIM), a heterogeneous group of disorders that of the general population as demonstrated by several characterised by weakness and inflammation of skele- studies with the prevalence being 32% and 15% for DM and PM respectively. The mechanism underlying the association tal muscle, are often associated with malignancies [5]. between idiopathic inflammatory myopathies (IIM) and ma- Polymyositis (PM) and dermatomyositis (DM) are clas- lignancies remains unclear. Both conditions predominantly sified as IIM, with these conditions predominantly af- affect adults. About 15% of DM is refractory to conventional fecting adults [6].
    [Show full text]