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Autoimmune Necrotizing Myopathy

NAM


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]
  • Anti-HMGCR antibodies are associated with necrotizing myopathies and are often present in isolation to other myositis specific or associated antibodies.[inter-medico.com]
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]
  • This weakness often leads to difficulty climbing stairs, rising from a chair or from the floor, turning in bed, lifting, combing hair, brushing teeth, or reaching over the head for something like an item on a shelf.[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]
Pathologist
  • Therefore, you may also see a physical therapist, occupational therapist, and, if you have trouble swallowing (dysphagia), you may also see a speech-language pathologist.[understandingmyositis.org]
Localized Pain
  • Myalgias can present as widespread pain or as localized pain, usually in the lower extremities. Muscle cramps and tendonitis-related pain are commonly reported.[mdedge.com]
Soft Tissue Swelling
Military Personnel
  • The second examines Environmental Risk Factors for Myositis in Military Personnel.[hss.edu]
Neck Muscle Weakness
  • Muscle weakness, which is often severe, can develop over a period of days, weeks, or months.[understandingmyositis.org]
  • In the anti-SRP positive IMNM, neurological symptoms and muscular involvement are present with limb and neck muscle weakness, dysphagia and respiratory failure, symptoms always present and more severe than the form with HMGCR positive [89,108].[journals.seedmedicalpublishers.com]
Ankle Pain
  • Initially, she had minor ankle pain after falling but this progressed to bilateral ankle pain and eventually bilateral shoulder pain. Creatine Kinase (CK) levels were drawn and results showed a level of 30,186.[scopemed.org]
Thigh Weakness
  • Neurological exam revealed symmetrical bilateral proximal shoulder girdle and thigh weakness.[lipid.org]
Neck Swelling
  • Neck swelling and severe dysphagia as the initial symptoms upon presentation has not been previously described.[bmcearnosethroatdisord.biomedcentral.com]
  • A case report of autoimmune necrotizing myositis presenting as dysphagia and neck swelling. BMC Ear Nose Throat Disord 2016; 16: 7; 49. Boyce B. 002. Case report: statin-induced autoimmune necrotizing myopathy with positive HMGCR antibodies.[journals.seedmedicalpublishers.com]

Treatment

  • Medical treatment is mostly based on more frequently arising IIMs. Prednisone treatment is the first choice on an empiric basis [ 16 ].[karger.com]
  • Before treatment, data on clinical features, muscle pathology, and thigh MRIs were obtained. After definitive diagnoses, all 3 patients were treated with intravenous immunoglobulin and corticosteroids, and thigh MRIs were performed.[ncbi.nlm.nih.gov]
  • 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]

Prognosis

  • 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]
  • 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]
  • 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]
  • Data are limited concerning differences among etiologic subgroups and treatment outcomes in NAM.[ncbi.nlm.nih.gov]
  • Though statin-induced autoimmune- mediated necrotizing myonecrosis is rare, it is important to consider it as a possible etiology for severely ill patients and for patients whose muscle symptoms do not resolve with cessation of statin therapy.[lipid.org]
  • Clues to these other etiologies are often apparent in the history.[clinicaladvisor.com]
  • 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]

Epidemiology

  • The present article summarizes the main features of statin-related IMNM, describing diagnosis, classification, epidemiology, treatment, and the main autoantibodies detected.[journals.seedmedicalpublishers.com]
  • 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]
Sex distribution
Age distribution

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]
  • Statin-associated autoimmune myopathies: A pathophysiologic spectrum. Can J Neurol Sci 2014; 41: 638-47; 20. Musset L, Allenbach Y, Benveniste O, et al.[journals.seedmedicalpublishers.com]
  • MD, FAAN National University of Athens Medical School, Department of Pathophysiology,75, Mikras Asias street, 11527 Athens, Greece.[em-consulte.com]
  • Hengstman GJD, Engelen BGM van, Venrooij WJ van (2004) Myositis specific autoantibodies: changing insights in pathophysiology and clinical associations. Curr Opin Rheumatol 16:692–699 PubMed 32.[springermedizin.de]

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]

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