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Systemic Sclerosis
Kirkland W. Davis, MD, FACR; B. J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        • Diagnostic Checklist

          TERMINOLOGY

          • Synonyms

            • Scleroderma, progressive systemic sclerosis (SSc)
          • Definitions

            • SSc: uncommon autoimmune disorder leading to fibrosis & collagen deposition as well as vasculopathy
              • MSK symptoms common; commonly source of disability

          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

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. Basyal B et al: Avascular necrosis in systemic sclerosis patients: a case-based review of demographics, presentation, and management. Clin Rheumatol. 40(1):399-405, 2021
                                        2. Mar D et al: A narrative review of imaging in calcinosis associated with systemic sclerosis. Clin Rheumatol. 40(10):3867-74, 2021
                                        3. Sudoł-Szopińska I et al: Rheumatoid hand and other hand-deforming rheumatic conditions. Semin Musculoskelet Radiol. 25(2):232-45, 2021
                                        4. Sandler RD et al: Musculoskeletal hand involvement in systemic sclerosis. Semin Arthritis Rheum. 50(2):329-34, 2020
                                        5. Freire V et al: Radiological identification and analysis of soft tissue musculoskeletal calcifications. Insights Imaging. 9(4):477-92, 2018
                                        6. 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
                                        7. Williams AA et al: The scleroderma hand: manifestations of disease and approach to management. J Hand Surg Am. 43(6):550-7, 2018
                                        8. Botou A et al: Acro-osteolysis. Clin Rheumatol. 36(1):9-14, 2017
                                        9. Freire V et al: Hand and wrist involvement in systemic sclerosis: US features. Radiology. 269(3):824-30, 2013
                                        10. Hachulla E et al: Diagnosis and classification of systemic sclerosis. Clin Rev Allergy Immunol. 40(2):78-83, 2011
                                        11. Schanz S et al: Localized scleroderma: MR findings and clinical features. Radiology. 260(3):817-24, 2011
                                        12. Agarwal SK et al: The genetics of scleroderma (systemic sclerosis). Curr Opin Rheumatol. 22(2):133-8, 2010
                                        13. 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
                                        14. 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
                                        15. 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
                                        16. Low AH et al: Magnetic resonance imaging of the hand in systemic sclerosis. J Rheumatol. 36(5):961-4, 2009
                                        17. Ranque B et al: A descriptive and prognostic study of systemic sclerosis-associated myopathies. Ann Rheum Dis. 68(9):1474-7, 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. Braun-Moscovici Y et al: Vitamin D, parathyroid hormone, and acroosteolysis in systemic sclerosis. J Rheumatol. 35(11):2201-5, 2008
                                        20. Madani G et al: The role of radiology in the management of systemic sclerosis. Clin Radiol. 63(9):959-67, 2008
                                        21. 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
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Scleroderma, progressive systemic sclerosis (SSc)
                                                • Definitions

                                                  • SSc: uncommon autoimmune disorder leading to fibrosis & collagen deposition as well as vasculopathy
                                                    • MSK symptoms common; commonly source of disability

                                                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

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. Basyal B et al: Avascular necrosis in systemic sclerosis patients: a case-based review of demographics, presentation, and management. Clin Rheumatol. 40(1):399-405, 2021
                                                                              2. Mar D et al: A narrative review of imaging in calcinosis associated with systemic sclerosis. Clin Rheumatol. 40(10):3867-74, 2021
                                                                              3. Sudoł-Szopińska I et al: Rheumatoid hand and other hand-deforming rheumatic conditions. Semin Musculoskelet Radiol. 25(2):232-45, 2021
                                                                              4. Sandler RD et al: Musculoskeletal hand involvement in systemic sclerosis. Semin Arthritis Rheum. 50(2):329-34, 2020
                                                                              5. Freire V et al: Radiological identification and analysis of soft tissue musculoskeletal calcifications. Insights Imaging. 9(4):477-92, 2018
                                                                              6. 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
                                                                              7. Williams AA et al: The scleroderma hand: manifestations of disease and approach to management. J Hand Surg Am. 43(6):550-7, 2018
                                                                              8. Botou A et al: Acro-osteolysis. Clin Rheumatol. 36(1):9-14, 2017
                                                                              9. Freire V et al: Hand and wrist involvement in systemic sclerosis: US features. Radiology. 269(3):824-30, 2013
                                                                              10. Hachulla E et al: Diagnosis and classification of systemic sclerosis. Clin Rev Allergy Immunol. 40(2):78-83, 2011
                                                                              11. Schanz S et al: Localized scleroderma: MR findings and clinical features. Radiology. 260(3):817-24, 2011
                                                                              12. Agarwal SK et al: The genetics of scleroderma (systemic sclerosis). Curr Opin Rheumatol. 22(2):133-8, 2010
                                                                              13. 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
                                                                              14. 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
                                                                              15. 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
                                                                              16. Low AH et al: Magnetic resonance imaging of the hand in systemic sclerosis. J Rheumatol. 36(5):961-4, 2009
                                                                              17. Ranque B et al: A descriptive and prognostic study of systemic sclerosis-associated myopathies. Ann Rheum Dis. 68(9):1474-7, 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. Braun-Moscovici Y et al: Vitamin D, parathyroid hormone, and acroosteolysis in systemic sclerosis. J Rheumatol. 35(11):2201-5, 2008
                                                                              20. Madani G et al: The role of radiology in the management of systemic sclerosis. Clin Radiol. 63(9):959-67, 2008
                                                                              21. Nassenstein K et al: Detection of myocardial fibrosis in systemic sclerosis by contrast-enhanced magnetic resonance imaging. Rofo. 180(12):1054-60, 2008