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Abstract

Introduction

Idiopathic inflammatory myopathies are a group of rheumatologic disorders presenting with progressive muscle weakness and the presence of inflammatory infiltrates in muscle tissue on histopathology. Inclusion body myositis classically has an insidious onset and slow progression and affects the older population, most commonly men. Muscle weakness is usually asymmetric and involves the distal upper extremity muscle groups.

Case Presentation

This case describes a 59-year-old man presenting with worsening symmetrical upper and lower extremity proximal muscle weakness and disabling muscle pain in his shoulders and hips. Further, weakly positive antinuclear antibodies were also observed. The creatinine phosphokinase was also remarkably elevated, uncharacteristic of both inclusion body myositis and polymyalgia rheumatica. He was initially thought to have polymyalgia rheumatica, but given the time frame and the presence of muscle pain, a musclebiopsy was done, which confirmed inclusion body myositis.

Conclusion

This case underscores the challenges in diagnosing inclusion body myositis due to its slow progression and overlapping features with other conditions, highlighting the importance of recognizing its distinguishing characteristics.

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2025-06-27
2025-12-06

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References

  1. Milone M. Diagnosis and management of immune-mediated myopathies. Mayo Clin. Proc. 2017 92 5 826 837 10.1016/j.mayocp.2016.12.025 28473041
    [Google Scholar]
  2. Weihl C.C. Mammen A.L. Sporadic inclusion body myositis – a myodegenerative disease or an inflammatory myopathy. Neuropathol. Appl. Neurobiol. 2017 43 1 82 91 10.1111/nan.12384 28111778
    [Google Scholar]
  3. Greenberg S.A. Inclusion body myositis: Clinical features and pathogenesis. Nat. Rev. Rheumatol. 2019 15 5 257 272 10.1038/s41584‑019‑0186‑x 30837708
    [Google Scholar]
  4. Murata K.Y. Ito H. The etiology and pathogenesis of sporadic inclusion body myositis. Brain Nerve 2014 66 11 1385 1394 25407074
    [Google Scholar]
  5. Naddaf E. Barohn R.J. Dimachkie M.M. Inclusion body myositis: Update on pathogenesis and treatment. Neurotherapeutics 2018 15 4 995 1005 10.1007/s13311‑018‑0658‑8 30136253
    [Google Scholar]
  6. Mastaglia F.L. Needham M. Scott A. James I. Zilko P. Day T. Kiers L. Corbett A. Witt C.S. Allcock R. Laing N. Garlepp M. Christiansen F.T. Sporadic inclusion body myositis: HLA-DRB1 allele interactions influence disease risk and clinical phenotype. Neuromuscul. Disord. 2009 19 11 763 765 10.1016/j.nmd.2009.07.015 19720533
    [Google Scholar]
  7. Hedberg-Oldfors C. Lindgren U. Basu S. Visuttijai K. Lindberg C. Falkenberg M. Larsson Lekholm E. Oldfors A. Mitochondrial DNA variants in inclusion body myositis characterized by deep sequencing. Brain Pathol. 2021 31 3 12931 10.1111/bpa.12931 33354847
    [Google Scholar]
  8. Barohn R. Dimachkie M. Inclusion body myositis. Semin. Neurol. 2012 32 3 237 245 10.1055/s‑0032‑1329197 23117948
    [Google Scholar]
  9. Gazeley D.J. Cronin M.E. Diagnosis and treatment of the idiopathic inflammatory myopathies. Ther. Adv. Musculoskelet. Dis. 2011 3 6 315 324 10.1177/1759720X11415306 22870489
    [Google Scholar]
  10. Alfano L. Lowes L.P. Emerging therapeutic options for sporadic inclusion body myositis. Ther. Clin. Risk Manag. 2015 11 1459 1467 10.2147/TCRM.S65368 26445546
    [Google Scholar]
  11. Shigeyama M. Nishio N. Yokoi S. Mukoyama N. Wada A. Maruo T. Noda S. Murakami A. Tsuboi T. Katsuno M. Fujimoto Y. Sone M. Efficacy of endoscopic cricopharyngeal myotomy using a curved rigid laryngoscope in patients with sporadic inclusion body myositis: Four retrospective case reviews. Nagoya J. Med. Sci. 2023 85 4 866 874 38155617
    [Google Scholar]
  12. Lotz B.P. Engel A.G. Nishino H. Stevens J.C. Litchy W.J. Inclusion body myositis. Observations in 40 patients. Brain 1989 112 3 727 747 10.1093/brain/112.3.727 2543478
    [Google Scholar]
  13. Greenberg S.A. Cytoplasmic 5′-nucleotidase autoantibodies in inclusion body myositis: Isotypes and diagnostic utility. Muscle Nerve 2014 50 4 488 492 10.1002/mus.24199 24752512
    [Google Scholar]
  14. Blijham P.J. Hengstman G.J.D. Hama-Amin A.D. van Engelen B.G.M. Zwarts M.J. Needle electromyographic findings in 98 patients with myositis. Eur. Neurol. 2006 55 4 183 188 10.1159/000093866 16772711
    [Google Scholar]
  15. Dion E. Cherin P. Payan C. Fournet J.C. Papo T. Maisonobe T. Auberton E. Chosidow O. Godeau P. Piette J.C. Herson S. Grenier P. Magnetic resonance imaging criteria for distinguishing between inclusion body myositis and polymyositis. J. Rheumatol. 2002 29 9 1897 1906 12233884
    [Google Scholar]
  16. Rose M.R. 188th ENMC international workshop: Inclusion body myositis, 2–4 december 2011, naarden, the netherlands. Neuromuscul. Disord. 2013 23 12 1044 1055 10.1016/j.nmd.2013.08.007 24268584
    [Google Scholar]
  17. Kula R.W. Sawchak J.A. Sher J.H. Inclusion body myositis. Curr. Opin. Rheumatol. 1989 1 4 460 467 10.1097/00002281‑198901040‑00007 2562009
    [Google Scholar]
  18. Dimachkie MM Barohn RJ Inclusion body myositis. Neurol. Clin. 2014 32 3 629 646 10.1016/j.ncl.2014.04.001
    [Google Scholar]
  19. Dasgupta B. Cimmino M.A. Maradit-Kremers H. Schmidt W.A. Schirmer M. Salvarani C. Bachta A. Dejaco C. Duftner C. Jensen H.S. Duhaut P. Poór G. Kaposi N.P. Mandl P. Balint P.V. Schmidt Z. Iagnocco A. Nannini C. Cantini F. Macchioni P. Pipitone N. Amo M.D. Espígol-Frigolé G. Cid M.C. Martínez-Taboada V.M. Nordborg E. Direskeneli H. Aydin S.Z. Ahmed K. Hazleman B. Silverman B. Pease C. Wakefield R.J. Luqmani R. Abril A. Michet C.J. Marcus R. Gonter N.J. Maz M. Carter R.E. Crowson C.S. Matteson E.L. 2012 provisional classification criteria for polymyalgia rheumatica: A European League Against Rheumatism/American College of Rheumatology collaborative initiative. Ann. Rheum. Dis. 2012 71 4 484 492 10.1136/annrheumdis‑2011‑200329 22388996
    [Google Scholar]
  20. Dalakas M.C. Therapeutic advances and future prospects in immune-mediated inflammatory myopathies. Ther. Adv. Neurol. Disord. 2008 1 3 157 166 10.1177/1756285608097463 21180574
    [Google Scholar]
  21. Pawlitzki M. Nelke C. Korsen M. Meuth S.G. Ruck T. Sirolimus leads to rapid and sustained clinical improvement of motor deficits in a patient with inclusion body myositis. Eur. J. Neurol. 2022 29 4 1284 1287 10.1111/ene.15231 35253967
    [Google Scholar]
  22. Machado P.M. McDermott M.P. Blaettler T. Sundgreen C. Amato A.A. Ciafaloni E. Freimer M. Gibson S.B. Jones S.M. Levine T.D. Lloyd T.E. Mozaffar T. Shaibani A.I. Wicklund M. Rosholm A. Carstensen T.D. Bonefeld K. Jørgensen A.N. Phonekeo K. Heim A.J. Herbelin L. Barohn R.J. Hanna M.G. Dimachkie M.M. Dimachkie M. Statland J. Pasnoor M. Jawdat O. Heim A. Ciersdorff A. Sasidharan S. Currence M. Levine T. Otutoa R. Cooper A. Mozaffar T. Habib A. Cauchi J. Ung S. Mathew V. Hernandez I. Gibson S. Bromberg M. Mahoney K. Neate C. Janecki T. Papadakis M. Freimer M. Kaschalk M.K. Heintzman S. Wicklund M. Baines B. Vareldzis A. Hyslop E. Blume B. Ciafaloni E. Luebbe E. Eichinger K. Martens W. Gregory S. Janciuras J. Amato A. Doughty C. Roe K. Flynn P. Russo E. Lloyd T. Albayda J. Tiniakou E. Thomas S. Jones S. Solorzano G. Elliott M. Burns T. Crowell A. Eggleston D. Wagoner M. Shaibani A. Oates C. Machado P. Hanna M. Greensmith L. Ahmed M. Vivekanandam V. Appleby M. Ransley G. Eshun E.E. Skorupinska I. Germain L. Laxa A.M. Pontes J.R. Bellin A. Anifowoshe D. Safety and efficacy of arimoclomol for inclusion body myositis: A multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2023 22 10 900 911 10.1016/S1474‑4422(23)00275‑2 37739573
    [Google Scholar]
  23. Amato A.A. Sivakumar K. Goyal N. David W.S. Salajegheh M. Praestgaard J. Lach-Trifilieff E. Trendelenburg A.U. Laurent D. Glass D.J. Roubenoff R. Tseng B.S. Greenberg S.A. Treatment of sporadic inclusion body myositis with bimagrumab. Neurology 2014 83 24 2239 2246 10.1212/WNL.0000000000001070 25381300
    [Google Scholar]
  24. Nogalska A. D’Agostino C. Terracciano C. Engel W.K. Askanas V. Impaired autophagy in sporadic inclusion-body myositis and in endoplasmic reticulum stress-provoked cultured human muscle fibers. Am. J. Pathol. 2010 177 3 1377 1387 10.2353/ajpath.2010.100050 20616343
    [Google Scholar]
  25. Lindgren U. Pullerits R. Lindberg C. Oldfors A. Epidemiology, survival, and clinical characteristics of inclusion body myositis. Ann. Neurol. 2022 92 2 201 212 10.1002/ana.26412 35596584
    [Google Scholar]
  26. Amlani A. Choi M.Y. Tarnopolsky M. Brady L. Clarke A.E. Garcia-De La Torre I. Mahler M. Schmeling H. Barber C.E. Jung M. Fritzler M.J. Anti-NT5c1A autoantibodies as biomarkers in inclusion body myositis. Front. Immunol. 2019 10 745 10.3389/fimmu.2019.00745 31024569
    [Google Scholar]
  27. Warman-Chardon J. Breiner A. Bourque P.R. Inclusion body myositis. CMAJ 2024 196 14 E486 10.1503/cmaj.231815 38621777
    [Google Scholar]
/content/journals/crr/10.2174/0115733971361017250619105000
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Case  Report  of  an  Atypical  Presentation  of  Inclusion Body Myositis Masquerading as Polymyalgia Rheumatica
Bentham Science Publishers ; https://doi.org/10.2174/0115733971361017250619105000
/content/journals/crr/10.2174/0115733971361017250619105000
/content/journals/crr/10.2174/0115733971361017250619105000
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  • Article Type:     Case Report
Keywords: inclusion body myositis ; dermatomyositis ; polymyositis ; necrotizing autoimmune myopathy ; myopathy ; Idiopathic inflammatory myopathies

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