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Inflammatory Myopathy
Kirkland W. Davis, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Idiopathic inflammatory myopathy (IIM), myositis
          • Definitions

            • Group of rare, heterogeneous, acquired autoimmune conditions causing inflammation in muscle → muscle weakness & pain/tenderness
            • Current recognized phenotypes (based on clinical, lab, histologic data) include dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM), immune-mediated necrotizing myopathy (IMNM, a.k.a. necrotizing autoimmune myositis)
            • Antisynthetase syndrome recently added to list: subset of overlap syndrome/overlap myositis
            • Focal myositis: focal form, not recognized phenotype; imaging finding that may be harbinger of development of true IIM

          IMAGING

          • General Features

            • Imaging Recommendations

              • Radiographic Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • CT Findings

                      • Nuclear Medicine Findings

                        • Advanced Imaging Techniques

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          • Diagnosis

                                            Selected References

                                            1. Coskun Benlidayi I et al: The pathophysiological effects of exercise in the management of idiopathic inflammatory myopathies: a scoping review. Int J Rheum Dis. 24(7):896-903, 2021
                                            2. Palmer W et al: Muscle imaging. Musculoskeletal Diseases 2021-2024: Diagnostic Imaging. Springer, 2021
                                            3. Sudoł-Szopińska I et al: Rheumatoid hand and other hand-deforming rheumatic conditions. Semin Musculoskelet Radiol. 25(2):232-45, 2021
                                            4. Albayda J et al: Diagnostic value of muscle ultrasound for myopathies and myositis. Curr Rheumatol Rep. 22(11):82, 2020
                                            5. Dalakas MC: Inflammatory myopathies: update on diagnosis, pathogenesis and therapies, and COVID-19-related implications. Acta Myol. 39(4):289-301, 2020
                                            6. Kubínová K et al: How imaging can assist with diagnosis and monitoring of disease in myositis. Curr Rheumatol Rep. 22(10):62, 2020
                                            7. Guimarães JB et al: Diagnostic imaging of inflammatory myopathies: new concepts and a radiological approach. Curr Rheumatol Rep. 21(3):8, 2019
                                            8. Selva-O'Callaghan A et al: PET scan: nuclear medicine imaging in myositis. Curr Rheumatol Rep. 21(11):64, 2019
                                            9. Tomas X et al: Whole-body MRI and pathological findings in adult patients with myopathies. Skeletal Radiol. 48(5):653-76, 2019
                                            10. Endo Y et al: Myositis and fasciitis: role of imaging. Semin Musculoskelet Radiol. 22(3):286-98, 2018
                                            11. Jacques T et al: Musculoskeletal manifestations of non-RA connective tissue diseases: scleroderma, systemic lupus erythematosus, still's disease, dermatomyositis/polymyositis, Sjögren's syndrome, and mixed connective tissue disease. Semin Musculoskelet Radiol. 22(2):166-79, 2018
                                            12. Kubínová K et al: The role of imaging in evaluating patients with idiopathic inflammatory myopathies. Clin Exp Rheumatol. 36 Suppl 114(5):74-81, 2018
                                            13. Oldroyd A et al: Recent developments in classification criteria and diagnosis guidelines for idiopathic inflammatory myopathies. Curr Opin Rheumatol. 30(6):606-13, 2018
                                            14. Delavan JA et al: Gemcitabine-induced radiation recall myositis. Skeletal Radiol. 44(3):451-5, 2015
                                            15. Jarraya M et al: Atypical proliferative myositis: original MR description with pathologic correlation: case report. Skeletal Radiol. 43(8):1155-9, 2014
                                            16. Yoshida K et al: Fasciitis as a common lesion of dermatomyositis, demonstrated early after disease onset by en bloc biopsy combined with magnetic resonance imaging. Arthritis Rheum. 62(12):3751-9, 2010
                                            17. Gardner-Medwin JM et al: MRI in juvenile idiopathic arthritis and juvenile dermatomyositis. Ann N Y Acad Sci. 1154:52-83, 2009
                                            18. Schulze M et al: MRI findings in inflammatory muscle diseases and their noninflammatory mimics. AJR Am J Roentgenol. 192(6):1708-16, 2009
                                            19. Tomasová Studynková J et al: The role of MRI in the assessment of polymyositis and dermatomyositis. Rheumatology (Oxford). 46(7):1174-9, 2007
                                            20. Chan WP et al: MR imaging of primary skeletal muscle diseases in children. AJR Am J Roentgenol. 179(4):989-97, 2002
                                            21. O'Connell MJ et al: Whole-body MR imaging in the diagnosis of polymyositis. AJR Am J Roentgenol. 179(4):967-71, 2002
                                            22. Adams EM et al: The idiopathic inflammatory myopathies: spectrum of MR imaging findings. Radiographics. 15(3):563-74, 1995
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Synonyms

                                                      • Idiopathic inflammatory myopathy (IIM), myositis
                                                    • Definitions

                                                      • Group of rare, heterogeneous, acquired autoimmune conditions causing inflammation in muscle → muscle weakness & pain/tenderness
                                                      • Current recognized phenotypes (based on clinical, lab, histologic data) include dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM), immune-mediated necrotizing myopathy (IMNM, a.k.a. necrotizing autoimmune myositis)
                                                      • Antisynthetase syndrome recently added to list: subset of overlap syndrome/overlap myositis
                                                      • Focal myositis: focal form, not recognized phenotype; imaging finding that may be harbinger of development of true IIM

                                                    IMAGING

                                                    • General Features

                                                      • Imaging Recommendations

                                                        • Radiographic Findings

                                                          • MR Findings

                                                            • Ultrasonographic Findings

                                                              • CT Findings

                                                                • Nuclear Medicine Findings

                                                                  • Advanced Imaging Techniques

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    • Diagnosis

                                                                                      Selected References

                                                                                      1. Coskun Benlidayi I et al: The pathophysiological effects of exercise in the management of idiopathic inflammatory myopathies: a scoping review. Int J Rheum Dis. 24(7):896-903, 2021
                                                                                      2. Palmer W et al: Muscle imaging. Musculoskeletal Diseases 2021-2024: Diagnostic Imaging. Springer, 2021
                                                                                      3. Sudoł-Szopińska I et al: Rheumatoid hand and other hand-deforming rheumatic conditions. Semin Musculoskelet Radiol. 25(2):232-45, 2021
                                                                                      4. Albayda J et al: Diagnostic value of muscle ultrasound for myopathies and myositis. Curr Rheumatol Rep. 22(11):82, 2020
                                                                                      5. Dalakas MC: Inflammatory myopathies: update on diagnosis, pathogenesis and therapies, and COVID-19-related implications. Acta Myol. 39(4):289-301, 2020
                                                                                      6. Kubínová K et al: How imaging can assist with diagnosis and monitoring of disease in myositis. Curr Rheumatol Rep. 22(10):62, 2020
                                                                                      7. Guimarães JB et al: Diagnostic imaging of inflammatory myopathies: new concepts and a radiological approach. Curr Rheumatol Rep. 21(3):8, 2019
                                                                                      8. Selva-O'Callaghan A et al: PET scan: nuclear medicine imaging in myositis. Curr Rheumatol Rep. 21(11):64, 2019
                                                                                      9. Tomas X et al: Whole-body MRI and pathological findings in adult patients with myopathies. Skeletal Radiol. 48(5):653-76, 2019
                                                                                      10. Endo Y et al: Myositis and fasciitis: role of imaging. Semin Musculoskelet Radiol. 22(3):286-98, 2018
                                                                                      11. Jacques T et al: Musculoskeletal manifestations of non-RA connective tissue diseases: scleroderma, systemic lupus erythematosus, still's disease, dermatomyositis/polymyositis, Sjögren's syndrome, and mixed connective tissue disease. Semin Musculoskelet Radiol. 22(2):166-79, 2018
                                                                                      12. Kubínová K et al: The role of imaging in evaluating patients with idiopathic inflammatory myopathies. Clin Exp Rheumatol. 36 Suppl 114(5):74-81, 2018
                                                                                      13. Oldroyd A et al: Recent developments in classification criteria and diagnosis guidelines for idiopathic inflammatory myopathies. Curr Opin Rheumatol. 30(6):606-13, 2018
                                                                                      14. Delavan JA et al: Gemcitabine-induced radiation recall myositis. Skeletal Radiol. 44(3):451-5, 2015
                                                                                      15. Jarraya M et al: Atypical proliferative myositis: original MR description with pathologic correlation: case report. Skeletal Radiol. 43(8):1155-9, 2014
                                                                                      16. Yoshida K et al: Fasciitis as a common lesion of dermatomyositis, demonstrated early after disease onset by en bloc biopsy combined with magnetic resonance imaging. Arthritis Rheum. 62(12):3751-9, 2010
                                                                                      17. Gardner-Medwin JM et al: MRI in juvenile idiopathic arthritis and juvenile dermatomyositis. Ann N Y Acad Sci. 1154:52-83, 2009
                                                                                      18. Schulze M et al: MRI findings in inflammatory muscle diseases and their noninflammatory mimics. AJR Am J Roentgenol. 192(6):1708-16, 2009
                                                                                      19. Tomasová Studynková J et al: The role of MRI in the assessment of polymyositis and dermatomyositis. Rheumatology (Oxford). 46(7):1174-9, 2007
                                                                                      20. Chan WP et al: MR imaging of primary skeletal muscle diseases in children. AJR Am J Roentgenol. 179(4):989-97, 2002
                                                                                      21. O'Connell MJ et al: Whole-body MR imaging in the diagnosis of polymyositis. AJR Am J Roentgenol. 179(4):967-71, 2002
                                                                                      22. Adams EM et al: The idiopathic inflammatory myopathies: spectrum of MR imaging findings. Radiographics. 15(3):563-74, 1995