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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Muscle biopsy from this case&#46;</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Modified trichrome Gomori &#40;TMG&#41; staining&#46; Close-up 200&#215; TMG&#44; staining demonstrated muscle fibres of varying size&#44; ballooning&#44; aggregates of inflammatory cells with focal invasion of some muscle fibres&#44; and substantial proliferation of connective tissue &#40;A&#41;&#46; Close-up 400&#215; TMG&#44; with obvious nuclear centralisation &#40;arrow&#41; &#40;B&#41;&#46; Close-up 400&#215; MTG Vacuoles rimmed with granular material and filaments &#40;star&#41; classically observed in this pathology &#40;C&#41;&#46;</p> <p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Haematoxylin and eosin &#40;H&#38;E&#41; staining&#46; Close-up 100&#215;&#44; panoramic view of the biopsy displaying a major deviation from the general architecture&#44; with enlarged spaces and substitution by connective tissue&#44; fibre morphology with bulging and inflammatory infiltrate &#40;D&#41;&#46; Close-up at 400&#215; &#40;H&#38;E&#41; illustrates the inflammatory infiltrate surrounding the muscle fibre in greater detail &#40;E&#41;&#44; as well as abundant connective tissue&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Inclusion body myositis &#40;IBM&#41; is an immune-mediated disease affecting muscles and internal organs&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Prevalence rates vary from 24&#46;8 to 45&#46;6 per million&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> It affects individuals over the age of 50<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and debuts with musculoskeletal damage to the volar aspect of the forearm&#44; flexors of the fingers and quadriceps&#44; resulting in asymmetrical muscle weakness and inclusion bodies on biopsy&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Biochemically&#44; they are accompanied by elevated creatine phosphokinase &#40;CPK&#41;&#44; the presence of anti-cytosolic 50-nucleotidase cN1A antibody in up to 30&#37; of cases&#44; which&#44; though non-specific&#44; are associated with increased severity and mortality&#44; and biopsy reveals an endomysial inflammatory exudate with infiltration of inflammatory cells in non-necrotic muscle fibres and vacuoles rimmed by membranous cytoplasmic material&#44; atrophic fibres&#44; and intra- or extravacuolar congophilic inclusions&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a female patient diagnosed with polymyositis &#40;PM&#41; on the basis of elevated CPK&#44; positive AC-1 antibodies&#44; electromyography &#40;EMG&#41; with myopathic pattern&#44; and muscle biopsy with inflammatory infiltrate&#44; with poor response to steroid treatment&#46; A new biopsy was therefore carried out that demonstrated IBM&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case Report</span><p id="par0015" class="elsevierStylePara elsevierViewall">An 82-year-old female presented difficulty climbing stairs and swallowing&#44; diminished muscle strength in the shoulder girdle and pelvis&#44; elevated CPK levels&#44; and was positive for AC-1 antibodies at the age of 66&#46; Anti-Jo1 immunospecificity was subsequently assessed and was negative &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46; An EMG examination was conducted that evidenced myopathic pattern and a biopsy of the left deltoid muscle was performed with the patient&#8217;s informed consent and endomysial lymphocytic inflammatory infiltrate was noted&#46; The woman was diagnosed with MP and treatment was instituted with prednisone and methotrexate&#44; with a subsequent change to mycophenolate mofetil&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Her disease progressed and she required the use of a cane and wheelchair&#59; she experienced difficulty in extending the fingers of the left hand&#44; atrophy of the long flexor muscles of the fingers&#44; predominantly the left one&#44; and quadriceps&#46; Muscle testing &#40;MMT8&#47; MMT26 manual muscle assessment procedures&#41; yielded a score of 116 of 150 and 194 of 260&#44; respectively&#44; at the expense of distal muscle involvement&#46; The new muscle biopsy indicated the presence of an inflammatory infiltrate with rimmed vacuoles and IBM was diagnosed &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">This case initially featured symmetrical proximal muscle weakness&#44; dysphagia&#44; and elevated CPK&#44; as well as an endomysial lymphocytic infiltrate&#44; characteristic of PM&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Nonetheless&#44; recurrence of elevated CPK values&#44; increased proximal muscle weakness and distal involvement&#44; asymmetric atrophy of the hand and quadriceps muscles&#44; deterioration of the patient&#8217;s dysphagia&#44; and her poor response to treatment led to IBM2 being suspected&#46; Alamr et al&#46; reported that 14&#37; of 367 patients with IBM had an atypical onset and of them&#44; 6&#37; displayed proximal arm weakness at the time of onset&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> In a clinical case series of IBM with no rimmed vacuoles on biopsy&#44; diagnosis was delayed for up to eight years following symptom debut<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a>&#59; however&#44; this feature may be absent in 20&#37; of all cases&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> The condition that imitates IBM most closely is PM&#44; inasmuch as its serology is similar and antinuclear antibodies &#40;ANA&#41; are positive in 15-19&#37; in IBM and 60&#37; in PM&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> A retrospective study comparing several subtypes of inflammatory myopathies failed exhibit any significant difference between PM and IBM with respect to follow up&#44; disease duration&#44; clinical characteristics&#44; and CPK values&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> In fact&#44; the presence of PM has currently been called into question&#44; as most individuals diagnosed with PM are re-classified as suffering from anti-synthetase syndrome having no rash&#44; immune-mediated necrotising myopathy&#44; or IBM with or without rimmed vacuoles ribeteadas&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;11</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The clinical course in this case evidences the challenge posed by the differential diagnosis between PM and IBM&#44; which entails a delay in establishing the diagnosis&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0035" class="elsevierStylePara elsevierViewall">The clinical evolution with asymmetrical muscle involvement and poor response to immunosuppressants made it possible to reformulate the diagnosis as IBM&#44; with a slowly progressive course as well as histopathological changes in muscle biopsies&#46; Treatment with corticosteroids and immunosuppressants failed to stem the progression of the disease&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Funding</span><p id="par0040" class="elsevierStylePara elsevierViewall">No external funding was necessary&#46; The resources of the Hospital de Especialidades Centro M&#233;dico Nacional&#44; La Raza were used&#46;</p></span></span>"
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          "identificador" => "xpalclavsec1900103"
          "palabras" => array:5 [
            0 => "Miositis por cuerpos de inclusi&#243;n"
            1 => "Miopat&#237;a inflamatoria idiop&#225;tica"
            2 => "Debilidad muscular"
            3 => "Disfagia"
            4 => "Vacuolas ribeteadas"
          ]
        ]
      ]
    ]
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    "resumen" => array:2 [
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Inclusion body myositis is a idiopathic inflammatory myopathy characterized by muscle weakness and dysphagia&#44; with muscle biopsy showing inflammation and rimmed vacuoles&#46; We present the case of a patient who was diagnosed with polymyositis but due to lack of response to treatment&#44; a new biopsy revealed inclusion body myositis&#46;</p></span>"
      ]
      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">La miositis por cuerpos de inclusi&#243;n es una miopat&#237;a inflamatoria idiop&#225;tica caracterizada por debilidad muscular&#44; disfagia y biopsia muscular con inflamaci&#243;n y vacuolas ribeteadas&#46; Presentamos el caso de una paciente a la que se le diagnostic&#243; polimiositis pero que por falta de respuesta al tratamiento se le realiz&#243; una nueva biopsia que revel&#243; miositis por cuerpos de inclusi&#243;n&#46;</p></span>"
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      0 => array:1 [
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          0 => array:4 [
            "apendice" => "<p id="par0055" class="elsevierStylePara elsevierViewall">The following is Supplementary data to this article&#58;<elsevierMultimedia ident="upi0005"></elsevierMultimedia></p>"
            "etiqueta" => "Appendix A"
            "titulo" => "Supplementary data"
            "identificador" => "sec0035"
          ]
        ]
      ]
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        "descripcion" => array:1 [
          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Muscle biopsy from this case&#46;</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Modified trichrome Gomori &#40;TMG&#41; staining&#46; Close-up 200&#215; TMG&#44; staining demonstrated muscle fibres of varying size&#44; ballooning&#44; aggregates of inflammatory cells with focal invasion of some muscle fibres&#44; and substantial proliferation of connective tissue &#40;A&#41;&#46; Close-up 400&#215; TMG&#44; with obvious nuclear centralisation &#40;arrow&#41; &#40;B&#41;&#46; Close-up 400&#215; MTG Vacuoles rimmed with granular material and filaments &#40;star&#41; classically observed in this pathology &#40;C&#41;&#46;</p> <p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Haematoxylin and eosin &#40;H&#38;E&#41; staining&#46; Close-up 100&#215;&#44; panoramic view of the biopsy displaying a major deviation from the general architecture&#44; with enlarged spaces and substitution by connective tissue&#44; fibre morphology with bulging and inflammatory infiltrate &#40;D&#41;&#46; Close-up at 400&#215; &#40;H&#38;E&#41; illustrates the inflammatory infiltrate surrounding the muscle fibre in greater detail &#40;E&#41;&#44; as well as abundant connective tissue&#46;</p>"
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      "titulo" => "References"
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          "identificador" => "bibs0005"
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Current classification and management of inflammatory myopathies"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "J&#46; Schmidt"
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                    0 => array:2 [
                      "doi" => "10.3233/JND-180308"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Neuromuscul Dis"
                        "fecha" => "2018"
                        "volumen" => "5"
                        "paginaInicial" => "109"
                        "paginaFinal" => "129"
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                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/29865091"
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              "identificador" => "bib0010"
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                0 => array:2 [
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                    0 => array:2 [
                      "titulo" => "Inclusion body myositis&#58; update on pathogenesis and treatment"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "E&#46; Naddaf"
                            1 => "R&#46;J&#46; Barohn"
                            2 => "M&#46;M&#46; Dimachkie"
                          ]
                        ]
                      ]
                    ]
                  ]
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                      "doi" => "10.1007/s13311-018-0658-8"
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                        "tituloSerie" => "Neurotherapeutics"
                        "fecha" => "2018"
                        "volumen" => "15"
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                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/30136253"
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                    0 => array:2 [
                      "titulo" => "Classification and management of adult inflammatory myopathies"
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                          "autores" => array:6 [
                            0 => "A&#46; Selva-O&#8217;Callaghan"
                            1 => "I&#46; Pinal-Fernandez"
                            2 => "E&#46; Trallero-Aragu&#225;s"
                            3 => "J&#46;C&#46; Milisenda"
                            4 => "J&#46;M&#46; Grau-Junyent"
                            5 => "A&#46;L&#46; Mammen"
                          ]
                        ]
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                      "doi" => "10.1016/S1474-4422(18)30254-0"
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                        "tituloSerie" => "Lancet Neurol"
                        "fecha" => "2018"
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                      "titulo" => "Pitfalls in the diagnosis of myositis"
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                            0 => "H Chinoy"
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                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:3 [
                        "tituloSerie" => "Best Pract Res Clin Rheumatol"
                        "fecha" => "2020"
                        "volumen" => "34"
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                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Polymyositis and dermatomyositis &#8211; challenges in diagnosis and management"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "S&#46;H&#46; Yang"
                            1 => "C&#46; Chang"
                            2 => "Z&#46;X&#46; Lian"
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Case Report
Available online 29 October 2024
Inclusion Body Myositis: A Late Diagnosis Case Report
Miositis por cuerpos de inclusión: informe de un caso de diagnóstico tardío
Deysi Andrea Hernández-Riveroa, Lisette Bazán-Rodríguezb, María del Pilar Cruz-Domínguezc, Gabriela Medinad, Ana Lilia Peralta Amaroe, Olga Vera-Lastrae,
Corresponding author
olgavera62@yahoo.com.mx

Corresponding author.
a División Académica Multidisciplinaria de Comalcalco, Universidad Juárez Autónoma de Tabasco, Comalcalco, Tabasco, Mexico
b Departamento de Enfermedades Neuromusculares, Hospital de Especialidades «Dr. Antonio Fraga Mouret», Centro Médico Nacional «La Raza», Mexico City, Mexico
c Dirección de Investigación y Educación, Hospital de Especialidades «Dr. Antonio Fraga Mouret», Centro Médico Nacional «La Raza», Mexico City, Mexico
d Unidad de Investigación Traslacional, Hospital de Especialidades «Dr. Antonio Fraga Mouret», Centro Médico Nacional «La Raza», Mexico City, Mexico
e Departamento de Medicina Interna, Hospital de Especialidades «Dr. Antonio Fraga Mouret», Centro Médico Nacional «La Raza», Mexico City, Mexico
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Table 1. Baseline laboratory values of the patient with inclusion body myositis.
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Abstract

Inclusion body myositis is a idiopathic inflammatory myopathy characterized by muscle weakness and dysphagia, with muscle biopsy showing inflammation and rimmed vacuoles. We present the case of a patient who was diagnosed with polymyositis but due to lack of response to treatment, a new biopsy revealed inclusion body myositis.

Keywords:
Inclusion body myositis
Idiopathic inflammatory myopathy
Muscle weakness
Creatinine kinase
Rimmed vacuoles
Resumen

La miositis por cuerpos de inclusión es una miopatía inflamatoria idiopática caracterizada por debilidad muscular, disfagia y biopsia muscular con inflamación y vacuolas ribeteadas. Presentamos el caso de una paciente a la que se le diagnosticó polimiositis pero que por falta de respuesta al tratamiento se le realizó una nueva biopsia que reveló miositis por cuerpos de inclusión.

Palabras clave:
Miositis por cuerpos de inclusión
Miopatía inflamatoria idiopática
Debilidad muscular
Disfagia
Vacuolas ribeteadas

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