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Connective Tissue Disorders, Immunological Diseases, and Vasculitis
Immunological and Connective Tissue Disorders
Dermatomyositis/Polymyositis
Dermatomyositis/Polymyositis
Carlos S. Restrepo, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Dermatomyositis/polymyositis (D/P)

            • Synonyms

              • Polymyositis/dermatomyositis interstitial lung disease (ILD)
              • Idiopathic inflammatory myopathy ILD

            • Definitions

              • Autoimmune myopathy
                • Manifests with inflammation and weakness
                • Dermatomyositis exhibits variety of cutaneous manifestations
                • Polymyositis involves proximal muscles over weeks or months
              • May be complicated by connective tissue disease-associated interstitial lung disease (CTD-ILD), aspiration bronchiolitis, &/or hypoventilation from affected respiratory muscles
                • CTD-ILD: Diffuse interstitial pneumonia secondary to D/P
                • Aspiration bronchiolitis: Typically diffuse

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • Microscopic Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Wang H et al: Pulmonary hypertension in polymyositis. Clin Rheumatol. 34(12):2105-12, 2015
                                    2. Tanizawa K et al: The prognostic value of HRCT in myositis-associated interstitial lung disease. Respir Med. 107(5):745-52, 2013
                                    3. Hervier B et al: Clinical manifestations of anti-synthetase syndrome positive for anti-alanyl-tRNA synthetase (anti-PL12) antibodies: a retrospective study of 17 cases. Rheumatology (Oxford). 49(5):972-6, 2010
                                    4. Chen IJ et al: Interstitial lung disease in polymyositis and dermatomyositis. Clin Rheumatol. 28(6):639-46, 2009
                                    5. Kang EH et al: Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology (Oxford). 44(10):1282-6, 2005
                                    6. Bonnefoy O et al: Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis. Eur J Radiol. 49(3):235-44, 2004
                                    7. Douglas WW et al: Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 164(7):1182-1185, 2001.
                                    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

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Dermatomyositis/polymyositis (D/P)

                                              • Synonyms

                                                • Polymyositis/dermatomyositis interstitial lung disease (ILD)
                                                • Idiopathic inflammatory myopathy ILD

                                              • Definitions

                                                • Autoimmune myopathy
                                                  • Manifests with inflammation and weakness
                                                  • Dermatomyositis exhibits variety of cutaneous manifestations
                                                  • Polymyositis involves proximal muscles over weeks or months
                                                • May be complicated by connective tissue disease-associated interstitial lung disease (CTD-ILD), aspiration bronchiolitis, &/or hypoventilation from affected respiratory muscles
                                                  • CTD-ILD: Diffuse interstitial pneumonia secondary to D/P
                                                  • Aspiration bronchiolitis: Typically diffuse

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • Microscopic Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                • Treatment

                                                                  DIAGNOSTIC CHECKLIST

                                                                  • Consider

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Wang H et al: Pulmonary hypertension in polymyositis. Clin Rheumatol. 34(12):2105-12, 2015
                                                                      2. Tanizawa K et al: The prognostic value of HRCT in myositis-associated interstitial lung disease. Respir Med. 107(5):745-52, 2013
                                                                      3. Hervier B et al: Clinical manifestations of anti-synthetase syndrome positive for anti-alanyl-tRNA synthetase (anti-PL12) antibodies: a retrospective study of 17 cases. Rheumatology (Oxford). 49(5):972-6, 2010
                                                                      4. Chen IJ et al: Interstitial lung disease in polymyositis and dermatomyositis. Clin Rheumatol. 28(6):639-46, 2009
                                                                      5. Kang EH et al: Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology (Oxford). 44(10):1282-6, 2005
                                                                      6. Bonnefoy O et al: Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis. Eur J Radiol. 49(3):235-44, 2004
                                                                      7. Douglas WW et al: Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 164(7):1182-1185, 2001.