Presentation
Case Presentation: A 90 year old woman presented with weakness for one month. Atorvastatin 80mg was newly prescribed for a myocardial infarction. On exam proximal muscle strength on all extremities was 3/5. [shmabstracts.com]
An inflammatory exudate was present in perivascular regions in perimysium especially where the necrotic fibers were present. Myositis specific antibodies including Jo-1, Mi-2, PL-7, PL-12, SRP, EJ and OJ were negative. [bmcearnosethroatdisord.biomedcentral.com]
Abstract A 73-year-old male presented with jaundice and severe muscle weakness. He was diagnosed with distal cholangiocarcinoma and paraneoplastic necrotizing autoimmune myopathy (NAM). [karger.com]
All patients presented with painless proximal weakness of varying severity. Investigations. Investigation findings demonstrated elevated CK in all patients. These results ranged from 2,700 to 16,200 IU/L at presentation. [nn.neurology.org]
The case presented is that of a 51-year-old female patient who developed progressive, proximal muscle weakness with bulbar involvement after treatment with statin. [neurologyindia.com]
Entire Body System
- Difficulty Climbing Stairs
climbing stairs and standing up from a chair Difficulty lifting arms over the head Falling and difficulty getting up from a fall A general feeling of tiredness [myositis.org]
Weakness in other muscles, including breathing and swallowing muscles, and distal muscles (furthest from the body’s core) Muscle pain (myalgia) Inability or difficulty climbing stairs, getting into a car and rising from a seated position Difficulty washing [understandingmyositis.org]
Five months later, she developed painless progressive shoulder weakness and difficulty climbing stairs, suggestive of proximal myopathy. There were no features of systemic illness. [nn.neurology.org]
Cardiovascular
- Heart Failure
The effect of spironolactone on morbidity and mortality in patients with severe heart failure. [books.google.de]
Patients may have cardiac involvement most often in the form of rhythm disturbances. 2 More rarely, they may experience conduction abnormalities, pericarditis, myocarditis, cardiac arrest, and congestive heart failure. 2,3 All of the preceding are often [clinicaladvisor.com]
Musculoskeletal
- Osteoporosis
Steroids have many side effects including weight gain and redistribution of body fat to the face, neck, and abdomen; elevated blood sugars, mood swings, thinning of the skin, cataracts, and osteoporosis. [understandingmyositis.org]
However, I began to see a loss of thigh muscle and was being diagnosed with osteoporosis. “I mentioned my leg problems to the rheumatologist. [peoplespharmacy.com]
Psychiatrical
- Withdrawn
This suggests that once the autoimmune muscle disease process is initiated by statin use, high levels of HMGCR in regenerating muscle cells continue to fuel the aggressive and painful autoimmune response, even after statins are withdrawn. [hopkinsmedicine.org]
Treatment
Medical treatment is mostly based on more frequently arising IIMs. Prednisone treatment is the first choice on an empiric basis [ 16 ]. [karger.com]
Some IMNM patients respond very well to treatment, while others may not. Finding the right treatment, or a combination of treatments can take months-to-years. Long-term steroid use may be a major source of morbidity. [understandingmyositis.org]
Muscle Nerve Myopathywithantibodiestothesignalrecognition particle: clinicalandpathological features AlfughamNB,Mahler M,MiloneM(2015)Clinical features and treatment outcomes of Necrotizing AutoimmuneMyopathy Clinical features and treatment outcomes of [semanticscholar.org]
This book provides state of the art information on the pathogenesis, diagnosis, and treatment of a range of inflammatory, autoimmune, and idiopathic neuromuscular disorders. [books.google.de]
SUMMARY: It is important to recognize and distinguish NAM from other causes of myocyte necrosis, because it has the potential of being amenable to treatment. [ncbi.nlm.nih.gov]
Prognosis
Progressive dysphagia is associated with a poorer prognosis and a poorer quality of life. [patient.info]
Scientists continue to learn more about how these autoantibodies shape pathophysiology, diagnosis, disease monitoring, prognosis and optimum treatment. [the-rheumatologist.org]
Correct subtype identification and the distinction of these conditions from other diseases that have similar characteristics are fundamental in differing prognosis and differing responses to therapy. [pathologicallyspeaking.blogspot.com]
IMNM Prognosis The prognosis of Immune-Mediated Necrotizing Myopathy depends on many varying factors including patient response to treatment, the severity of disease and its manifestations, the presence of pulmonary or cardiac involvement, and associated [understandingmyositis.org]
The Message Aside from the distinctive biopsy and antibody findings with immune-mediated necrotizing myopathy, the prognosis also differs from that of the more typical statin-associated myopathy. [medpagetoday.com]
Etiology
A muscle biopsy was performed and revealed a necrotizing myopathy with high probability of autoimmune etiology. [scopemed.org]
Clues to these other etiologies are often apparent in the history. [clinicaladvisor.com]
Data are limited concerning differences among etiologic subgroups and treatment outcomes in NAM. [ncbi.nlm.nih.gov]
Etiology The disease is thought to be related to an immune response possibly triggered by drug therapy (statins), connective tissue diseases, or cancer. [orpha.net]
100,000 ( Ann Rheum Dis 2009;68:1192 ), but only 3.8 per 100,000 in US ( Muscle Nerve 2012;45:676 ) Peak incidence is in older patients, with a mean age of 56 years and a male to female ratio of 1.6 : 1 Extremely rare in younger patients and childhood Etiology [pathologyoutlines.com]
Epidemiology
Summary Epidemiology The prevalence and annual incidence of NAM are not known but the disorder is very rare. About 300 cases have been reported to date. Clinical description Age of onset ranges from 30 to 70 years of age in reported cases. [orpha.net]
Skeletal muscle shows myopathic changes with evidence of myofiber degeneration and regeneration with lymphocytes invading non necrotic myofibers Predominant inflammatory cell is the CD8+ T lymphocyte Terminology Polymyositis or idiopathic polymyositis Epidemiology [pathologyoutlines.com]
Interest has been stimulated by the finding that substances deposited in muscle are similar to those found in the brain in Alzheimer's disease - eg, amyloid precursor proteins. [ 7, 8 ] Epidemiology [ 1 ] Most cases present in patients aged over 50 years [patient.info]
The present article summarizes the main features of statin-related IMNM, describing diagnosis, classification, epidemiology, treatment, and the main autoantibodies detected. [journals.seedmedicalpublishers.com]
Epidemiology of adult idiopathic inflammatory myopathies in a U.S. Managed care plan. Muscle Nerve 2012; 45 (5):676-83. [ Links ] 4. Needham M, Corbett A, Day T, Christiansen F, Fabian V, Mastaglia F. [scielo.cl]
Pathophysiology
Clinical features, pathophysiology, and treatment of polymyositis and dermatomyositis; inclusion body myositis; metabolic myopathies; drug-induced myopathies; muscular dystrophies and neurologic diseases; laboratory testing and imaging; electrophysiological [books.google.de]
Scientists continue to learn more about how these autoantibodies shape pathophysiology, diagnosis, disease monitoring, prognosis and optimum treatment. [the-rheumatologist.org]
In most subjects with statin-associated musculoskeletal side effects, discontinuing the offending medication will halt the pathophysiologic process, and the subsequent regeneration of myofibers will restore muscle function. [neurologyindia.com]
MD, FAAN National University of Athens Medical School, Department of Pathophysiology,75, Mikras Asias street, 11527 Athens, Greece. [em-consulte.com]
Statin-associated autoimmune myopathies: A pathophysiologic spectrum. Can J Neurol Sci 2014; 41: 638-47; https://doi.org/10.1017/cjn.2014.22 20. Musset L, Allenbach Y, Benveniste O, et al. [journals.seedmedicalpublishers.com]
Prevention
Proper recognition of this ominous complication is important to prevent further damage from statin use. [mdedge.com]
Discussion Statins are among the most prescribed medications in the world because of the excellent evidence demonstrating efficacy in primary and secondary prevention of cardiovascular events. [lipid.org]
We want to prevent this autoimmune disease.” Although statins are tolerated by most patients, about 5 percent who take them experience muscle pain and/or weakness severe enough to warrant stopping the medication. [hopkinsmedicine.org]
Some treat newly diagnosed patients with immunosuppression on the basis that early suppression of the inflammatory cascade may prevent downstream effects leading to muscle degeneration. [patient.info]
From the American Heart Association: 2013 ACC/AHA Guideline on the Treatment of Blood Cholesterol to Reduce Atherosclerotic Cardiovascular Risk in Adults Clinical Practice Guidelines for Prevention Vencovsky and colleagues reported no financial disclosures [medpagetoday.com]