link
Bookmarks
Progressive Systemic Sclerosis
B.J. Manaster, MD, PhD, FACR
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
6
19
0

KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Synonyms

          • Scleroderma, diffuse scleroderma
            • CREST syndrome (calcifications, Raynaud phenomenon, esophageal hypomobility, sclerodactyly, telangiectasia) = limited scleroderma
        • Definitions

          • Progressive systemic sclerosis (PSS): Multisystem disorder characterized by skin thickening and vasculitis
            • Triad of widespread microangiopathy, fibrosis, and autoimmunity

        IMAGING

        • General Features

          • Imaging Recommendations

            • Radiographic Findings

              • Ultrasonographic Findings

                • CT Findings

                  • MR Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Freire V et al: Hand and wrist involvement in systemic sclerosis: US features. Radiology. 269(3):824-30, 2013
                                    2. Hachulla E et al: Diagnosis and classification of systemic sclerosis. Clin Rev Allergy Immunol. 40(2):78-83, 2011
                                    3. Schanz S et al: Localized scleroderma: MR findings and clinical features. Radiology. 260(3):817-24, 2011
                                    4. Agarwal SK et al: The genetics of scleroderma (systemic sclerosis). Curr Opin Rheumatol. 22(2):133-8, 2010
                                    5. Hachulla E et al: Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class II dyspnoea: mild symptoms but severe outcome. Rheumatology (Oxford). 49(5):940-4, 2010
                                    6. Manetti M et al: Mechanisms in the loss of capillaries in systemic sclerosis: angiogenesis versus vasculogenesis. J Cell Mol Med. 14(6A):1241-54, 2010
                                    7. Nihtyanova SI et al: Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 103(2):109-15, 2010
                                    8. Low AH et al: Magnetic resonance imaging of the hand in systemic sclerosis. J Rheumatol. 36(5):961-4, 2009
                                    9. Ranque B et al: A descriptive and prognostic study of systemic sclerosis-associated myopathies. Ann Rheum Dis. 68(9):1474-7, 2009
                                    10. Schulze M et al: MRI findings in inflammatory muscle diseases and their noninflammatory mimics. AJR Am J Roentgenol. 192(6):1708-16, 2009
                                    11. Braun-Moscovici Y et al: Vitamin D, parathyroid hormone, and acroosteolysis in systemic sclerosis. J Rheumatol. 35(11):2201-5, 2008
                                    12. Madani G et al: The role of radiology in the management of systemic sclerosis. Clin Radiol. 63(9):959-67, 2008
                                    13. Nassenstein K et al: Detection of myocardial fibrosis in systemic sclerosis by contrast-enhanced magnetic resonance imaging. Rofo. 180(12):1054-60, 2008
                                    Related Anatomy
                                    Loading...
                                    Related Differential Diagnoses
                                    Loading...
                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Synonyms

                                            • Scleroderma, diffuse scleroderma
                                              • CREST syndrome (calcifications, Raynaud phenomenon, esophageal hypomobility, sclerodactyly, telangiectasia) = limited scleroderma
                                          • Definitions

                                            • Progressive systemic sclerosis (PSS): Multisystem disorder characterized by skin thickening and vasculitis
                                              • Triad of widespread microangiopathy, fibrosis, and autoimmunity

                                          IMAGING

                                          • General Features

                                            • Imaging Recommendations

                                              • Radiographic Findings

                                                • Ultrasonographic Findings

                                                  • CT Findings

                                                    • MR Findings

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Staging, Grading, & Classification

                                                            • Microscopic Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Freire V et al: Hand and wrist involvement in systemic sclerosis: US features. Radiology. 269(3):824-30, 2013
                                                                      2. Hachulla E et al: Diagnosis and classification of systemic sclerosis. Clin Rev Allergy Immunol. 40(2):78-83, 2011
                                                                      3. Schanz S et al: Localized scleroderma: MR findings and clinical features. Radiology. 260(3):817-24, 2011
                                                                      4. Agarwal SK et al: The genetics of scleroderma (systemic sclerosis). Curr Opin Rheumatol. 22(2):133-8, 2010
                                                                      5. Hachulla E et al: Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class II dyspnoea: mild symptoms but severe outcome. Rheumatology (Oxford). 49(5):940-4, 2010
                                                                      6. Manetti M et al: Mechanisms in the loss of capillaries in systemic sclerosis: angiogenesis versus vasculogenesis. J Cell Mol Med. 14(6A):1241-54, 2010
                                                                      7. Nihtyanova SI et al: Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 103(2):109-15, 2010
                                                                      8. Low AH et al: Magnetic resonance imaging of the hand in systemic sclerosis. J Rheumatol. 36(5):961-4, 2009
                                                                      9. Ranque B et al: A descriptive and prognostic study of systemic sclerosis-associated myopathies. Ann Rheum Dis. 68(9):1474-7, 2009
                                                                      10. Schulze M et al: MRI findings in inflammatory muscle diseases and their noninflammatory mimics. AJR Am J Roentgenol. 192(6):1708-16, 2009
                                                                      11. Braun-Moscovici Y et al: Vitamin D, parathyroid hormone, and acroosteolysis in systemic sclerosis. J Rheumatol. 35(11):2201-5, 2008
                                                                      12. Madani G et al: The role of radiology in the management of systemic sclerosis. Clin Radiol. 63(9):959-67, 2008
                                                                      13. Nassenstein K et al: Detection of myocardial fibrosis in systemic sclerosis by contrast-enhanced magnetic resonance imaging. Rofo. 180(12):1054-60, 2008