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Connective Tissue Disorders, Immunological Diseases, and Vasculitis
Immunological and Connective Tissue Disorders
Erdheim-Chester Disease
Erdheim-Chester Disease
Carlos S. Restrepo, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Erdheim-Chester disease (ECD)

            • Definitions

              • Non-Langerhans cell histiocytosis of unknown origin

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • Gross Pathologic & Surgical Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Goyal G et al: Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era. Blood. 135(22):1929-45, 2020
                                    2. Mirmomen SM et al: Thoracic involvement in Erdheim-Chester disease: computed tomography imaging findings and their association with the BRAFV600E mutation. Eur Radiol. 28(11):4635-42, 2018
                                    3. Estrada-Veras JI et al: The clinical spectrum of Erdheim-Chester disease: an observational cohort study. Blood Adv. 1(6):357-66, 2017
                                    4. Vargas D et al: Cardiothoracic manifestations of primary histiocytoses. Br J Radiol. 89(1068):20160347, 2016
                                    5. Haroche J et al: Images in cardiovascular medicine. Cardiac involvement in Erdheim-Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients. Circulation. 119(25):e597-8, 2009
                                    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

                                                • Erdheim-Chester disease (ECD)

                                              • Definitions

                                                • Non-Langerhans cell histiocytosis of unknown origin

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • Gross Pathologic & Surgical Features

                                                          • Microscopic Features

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Goyal G et al: Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era. Blood. 135(22):1929-45, 2020
                                                                      2. Mirmomen SM et al: Thoracic involvement in Erdheim-Chester disease: computed tomography imaging findings and their association with the BRAFV600E mutation. Eur Radiol. 28(11):4635-42, 2018
                                                                      3. Estrada-Veras JI et al: The clinical spectrum of Erdheim-Chester disease: an observational cohort study. Blood Adv. 1(6):357-66, 2017
                                                                      4. Vargas D et al: Cardiothoracic manifestations of primary histiocytoses. Br J Radiol. 89(1068):20160347, 2016
                                                                      5. Haroche J et al: Images in cardiovascular medicine. Cardiac involvement in Erdheim-Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients. Circulation. 119(25):e597-8, 2009