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Steroid myopathy

P Devic, L Gallay, N Streichenberger, P Petiot
Amongst the heterogeneous group of inflammatory myopathies, focal myositis (FM) stands as a rare and benign dysimmune disease. Although FM can be associated with root and/or nerve lesions, traumatic muscle lesions and autoimmune diseases, its triggering factors remain poorly understood. Defined as an isolated inflammatory pseudotumour usually restricted to one skeletal muscle, clinical presentation of FM is that of a rapidly growing solitary mass within a single muscle, usually in the lower limbs. Electromyography shows spontaneous activity associated with a myopathic pattern...
August 26, 2016: Neuromuscular Disorders: NMD
Rajiv D Reddy, Gayle R Spill
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Catherine Ashton, Reimar Junckerstorff, Chris Bundell, Peter Hollingsworth, Merrilee Needham
Necrotising Autoimmune Myopathy (NAM) presents as a subacute proximal myopathy with high creatine kinase levels. It is associated with statin exposure, 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody, connective tissue diseases, signal recognition particle (SRP) antibody and malignancy. This case series presents our Western Australian NAM patient cohort: comparing the subgroup presentations, biopsy appearance and treatment outcomes. We retrospectively collected data on patients diagnosed with NAM at the Western Australian Neuroscience Research Institute between the years 2000 and 2015...
September 3, 2016: Neuromuscular Disorders: NMD
Aarat Patel, Georgia Seely, Rohit Aggarwal
Idiopathic inflammatory myopathies are a group of systemic autoimmune diseases that involve inflammation of skeletal muscle. The two most common forms are dermatomyositis and polymyositis, the former of which entails a skin component. There are few approved therapeutics available for treatment of this group of diseases and the first-line therapy is usually corticosteroid treatment. Considering that a large proportion of patients do not respond to or cannot tolerate corticosteroids, additional treatments are required...
2016: Case Reports in Rheumatology
Cameron C Wick, Joseph Zachariah, Sunil Manjila, William C Brown, Prerna Malla, Bashar Katirji, Mark Cohen, Cliff A Megerian
IgG4-related disease (IgG4-RD) is increasingly being recognized as an entity effecting the head and neck region. Although most commonly seen with salivary gland or paranasal sinus involvement, IgG4-RD may also involve the temporal bone and skull base. We report a rare care of a 61-year-old female with IgG4-RD presenting as synchronous lesions of the middle ear and middle cranial fossa with polyneuropathy of cranial nerves II, VI, and VII. Initial histopathological evaluation of her resected ear mass suggested a benign inflammatory process but no specific diagnosis...
August 18, 2016: American Journal of Otolaryngology
Marta Amaral Silva, Estefania Cogollo, David A Isenberg
OBJECTIVES: Causes of death in inflammatory myopathies have rarely been studied. We have assessed a cohort of myositis patients followed in a single centre over a 37-year period, reviewing the mortality rate, causes of death and predictors of poor prognosis. METHODS: We performed a single-centre, retrospective study on patients aged ≥16 years fulfilling 3 or 4 of the Bohan and Peter criteria, noting their demographic data, clinical features, serology, treatment and outcome...
September 2016: Clinical and Experimental Rheumatology
Seo-Hyun Kim, I-Nae Park
Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome...
July 2016: Tuberculosis and Respiratory Diseases
Ilyas Arshad, Desmond Barton
A 60-year-old woman diagnosed with papillary serous ovarian cancer had Klean-Prep and MRI contrast preoperatively. Afterwards, she developed swelling and an urticarial rash around her eye as she proceeded to have planned debulking surgery. Postoperatively the swelling and rash had spread over her face, neck, back and chest. Dermatology advised a possible allergy to Klean-Prep and MRI contrast. Subsequently over the next few months, the patient became severely debilitated from proximal myopathy of the upper and lower limbs, suffered severe pain restricting mobility and small bowel obstruction...
2016: BMJ Case Reports
Ken-Ya Murata, Kumiko Nakatani, Mika Yananeki, Ichiro Nakanishi, Hidefumi Ito
BACKGROUND: The discovery of myositis-specific autoantibodies and myositis-associated autoantibodies has led to a new serological classification. Human U3 RNP, which consists of the U3 small nucleolar RNA and anti-U3 RNP antibody, is directed against one of the subunits. Anti-U3 RNP antibodies have been detected in 5-8 % of patients with systemic sclerosis, and antibody-positive patients with systemic sclerosis have shown more frequent skeletal muscle involvement than that of antibody-negative patients with systemic sclerosis...
2016: Journal of Medical Case Reports
A Pazderska, S Crowther, P Govender, K C Conlon, M Sherlock, J Gibney
UNLABELLED: Avascular necrosis (AVN) is a rare presenting feature of endogenous hypercortisolism. If left untreated, complete collapse of the femoral head may ensue, necessitating hip replacement in up to 70% of patients. The majority of the described patients with AVN due to endogenous hypercortisolaemia required surgical intervention. A 36-year-old female, investigated for right leg pain, reported rapid weight gain, bruising and secondary amenorrhoea. She had abdominal adiposity with violaceous striae, facial plethora and hirsutism, atrophic skin, ecchymosis and proximal myopathy...
2016: Endocrinology, Diabetes & Metabolism Case Reports
Sarah Tansley, Gavin Shaddick, Lisa Christopher-Stine, Charlotte Sharp, Lyubomir Dourmishev, Britta Maurer, Hector Chinoy, Neil McHugh
OBJECTIVES: The evidence base for treatment of the idiopathic inflammatory myopathies is extremely limited. The rarity and heterogeneity of these diseases has hampered the development of good quality clinical trials and while a range of immunomodulatory treatments are commonly used in clinical practice, as yet there are no clear guidelines directing their use. We aimed to establish current prescribing regimens used to treat adults with myositis internationally. METHODS: An electronic survey based on different clinical scenarios was distributed internationally to clinicians involved in the treatment of patients with myositis...
May 31, 2016: Clinical and Experimental Rheumatology
Felicity M Davis, Eugenia H Goulding, Diane M D'Agostin, Kyathanahalli S Janardhan, Connie A Cummings, Gary S Bird, Edward M Eddy, James W Putney
Store-operated calcium entry (SOCE) is an important Ca(2+) influx pathway in somatic cells. In addition to maintaining endoplasmic reticulum (ER) Ca(2+) stores, Ca(2+) entry through store-operated channels regulates essential signaling pathways in numerous cell types. Patients with mutations in the store-operated channel subunit ORAI1 exhibit defects in store-operated Ca(2+) influx, along with severe immunodeficiency, congenital myopathy and ectodermal dysplasia. However, little is known about the functional role of ORAI1 in germ cells and reproductive function in mice, or in men, since men with loss-of-function or null mutations in ORAI1 rarely survive to reproductive age...
April 2016: Cell Calcium
Jaime de Inocencio, Eugenia Enríquez-Merayo, Rocío Casado, Luis Ignacio González-Granado
Juvenile dermatomyositis (JDM) is the most common form of juvenile idiopathic inflammatory myopathy. We report a child with steroid-dependent JDM refractory to hydroxychloroquine and subcutaneous methotrexate who experienced systemic reactions to intravenous immunoglobulin and was successfully treated with subcutaneous immunoglobulin. This form of therapy has been shown to be safe, has a very low rate of adverse effects, does not require hospital admission, reduces the number of missed school days, and decreases the costs associated with treatment...
April 2016: Pediatrics
Ramanamma Kalluru, Hamish Hart, Michael Corkill, Kristine P Ng
BACKGROUND: Idiopathic inflammatory myopathies (IIMs) are an uncommon group of diseases that can be associated with significant morbidity and mortality related to systemic involvement or treatment-related complications. AIM: This study reports the concomitant diseases, extent of organ involvement, immunosuppressive use, treatment-related complications and damage outcome in a cohort of adult IIM. METHODS: All patients with IIM fulfilling at least 3 of the 4 Bohan and Peter criteria were identified...
January 29, 2016: New Zealand Medical Journal
Serena Fasano, Sara Custódio Alves, David A Isenberg
The idiopathic inflammatory myopathies are uncommon and heterogeneous disorders. Their classification is based on distinct clinicopathologic features. Although idiopathic inflammatory myopathies share some similarities, different subtypes may have variable responses to therapy, so it is very important to distinguish the correct subtype. There are few randomised, double blind placebo controlled studies to support the current treatment. High dose corticosteroids continue to be the first-line therapy and other immunosupressive drugs are used in refractory cases, as well as steroid-sparing agents...
February 6, 2016: Expert Review of Clinical Pharmacology
Shanthini Kasturi, Lisa R Sammaritano
Corticosteroids are the mainstay of treatment of systemic lupus erythematosus, with most patients receiving them at some point in the course of their disease. Corticosteroid use is associated with significant side effects, including infections, hypertension, hyperglycemia, osteoporosis, avascular necrosis, myopathy, cataracts, and glaucoma. Dosing regimens are based on limited data, with the goal of adequately controlling inflammatory symptoms while minimizing steroid exposure in order to reduce adverse effects...
February 2016: Rheumatic Diseases Clinics of North America
Merrilee Needham, Frank L Mastaglia
Until recently, the treatment of immune-mediated inflammatory myopathies has largely been empirical with glucocorticoids, steroid-sparing immunosuppressive drugs, and intravenous immunoglobulin. However, a proportion of patients are only partially responsive to these therapies, and there has been a need to consider alternative treatment approaches. In particular, patients with inclusion body myositis are resistant to conventional immunotherapies or show only a transient response, and remain a major challenge...
January 2016: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Antonella LoMauro, Maria Grazia D'Angelo, Andrea Aliverti
Duchenne muscular dystrophy (DMD) is an X-linked myopathy resulting in progressive weakness and wasting of all the striated muscles including the respiratory muscles. The consequences are loss of ambulation before teen ages, cardiac involvement and breathing difficulties, the main cause of death. A cure for DMD is not currently available. In the last decades the survival of patients with DMD has improved because the natural history of the disease can be changed thanks to a more comprehensive therapeutic approach...
2015: Therapeutics and Clinical Risk Management
Minoru Satoh, Shin Tanaka, Angela Ceribelli, S John Calise, Edward K L Chan
Autoantibodies specific for idiopathic inflammatory myopathy (myositis-specific autoantibodies (MSAs)) are clinically useful biomarkers to help the diagnosis of polymyositis/dermatomyositis (PM/DM). Many of these are also associated with a unique clinical subset of PM/DM, making them useful in predicting and monitoring certain clinical manifestations. Classic MSAs known for over 30 years include antibodies to Jo-1 (histidyl transfer RNA (tRNA) synthetase) and other aminoacyl tRNA synthetases (ARS), anti-Mi-2, and anti-signal recognition particle (SRP)...
September 30, 2015: Clinical Reviews in Allergy & Immunology
Guy J Ben Simon, Gabriel Katz, Ofira Zloto, Hana Leiba, Beatrix Hadas, Ruth Huna-Baron
PURPOSE: To examine the clinical differences in manifestation, course, treatment, and prognosis of thyroid eye disease (TED) in patients younger than 40 years of age at diagnosis compared to older patients. METHODS: Medical record review of 131 TED patients was performed. The patients were divided into two age groups, Group 1 ≤ 40 years (23 patients) and Group 2 > 40 years (108 patients). RESULTS: Younger patients had more eyelid retraction and proptosis at initial presentation, whereas older patients were more likely to have diplopia (P = 0...
December 2015: Graefe's Archive for Clinical and Experimental Ophthalmology
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