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Scleroderma
Julia Alegría, MD; Jonathan Hero Chung, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Systemic sclerosis
              • Definitions

                • Generalized connective tissue disorder affecting multiple organs, including skin, lungs, heart, and kidneys
                • Limited cutaneous systemic sclerosis (60%)
                  • Skin involvement: Hands, forearms, feet, face
                  • Longstanding Raynaud phenomenon
                  • CREST syndrome: Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias
                • Diffuse cutaneous systemic sclerosis (40%)
                  • Acute onset: Raynaud phenomenon, acral and truncal skin involvement
                  • High frequency of interstitial lung disease
                • Scleroderma sine scleroderma (rare)
                  • Interstitial lung disease without skin manifestations

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • Other Modality Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          Selected References

                                          1. Chung JH et al: Imaging features of systemic sclerosis-associated interstitial lung disease. J Vis Exp. 2020
                                          2. Perelas A et al: Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 8(3):304-20, 2020
                                          3. Sanchez F et al: Pathological entities that may affect the lungs and the myocardium. Evaluation with chest CT and cardiac MR. Clin Imaging. 70:124-35, 2020
                                          4. Elicker BM et al: Imaging of the thoracic manifestations of connective tissue disease. Clin Chest Med. 40(3):655-66, 2019
                                          5. Kusmirek JE et al: Thoracic manifestations of connective tissue diseases. Semin Ultrasound CT MR. 40(3):239-54, 2019
                                          6. Strollo D et al: Imaging lung disease in systemic sclerosis. Curr Rheumatol Rep. 12(2):156-61, 2010
                                          7. Lynch DA: Lung disease related to collagen vascular disease. J Thorac Imaging. 24(4):299-309, 2009
                                          8. de Azevedo AB et al: Prevalence of pulmonary hypertension in systemic sclerosis. Clin Exp Rheumatol. 23(4):447-54, 2005
                                          9. Galie N et al: Pulmonary arterial hypertension associated to connective tissue diseases. Lupus. 14(9):713-7, 2005
                                          10. Highland KB et al: New Developments in Scleroderma Interstitial Lung Disease. Curr Opin Rheumatol. 17(6):737-45, 2005
                                          11. Desai SR et al: CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology. 232(2):560-7, 2004
                                          12. Kim EA et al: Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. Radiographics. 2002 Oct;22 Spec No:S151-65. Review. Erratum in: Radiographics. 23(5):1340, 2003
                                          13. Kim EA et al: Interstitial pneumonia in progressive systemic sclerosis: serial high-resolution CT findings with functional correlation. J Comput Assist Tomogr. 25(5):757-63, 2001
                                          14. Diot E et al: Relationship between abnormalities on high-resolution CT and pulmonary function in systemic sclerosis. Chest. 114(6):1623-9, 1998
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Synonyms

                                                        • Systemic sclerosis
                                                      • Definitions

                                                        • Generalized connective tissue disorder affecting multiple organs, including skin, lungs, heart, and kidneys
                                                        • Limited cutaneous systemic sclerosis (60%)
                                                          • Skin involvement: Hands, forearms, feet, face
                                                          • Longstanding Raynaud phenomenon
                                                          • CREST syndrome: Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias
                                                        • Diffuse cutaneous systemic sclerosis (40%)
                                                          • Acute onset: Raynaud phenomenon, acral and truncal skin involvement
                                                          • High frequency of interstitial lung disease
                                                        • Scleroderma sine scleroderma (rare)
                                                          • Interstitial lung disease without skin manifestations

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • Other Modality Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Consider

                                                                                  Selected References

                                                                                  1. Chung JH et al: Imaging features of systemic sclerosis-associated interstitial lung disease. J Vis Exp. 2020
                                                                                  2. Perelas A et al: Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 8(3):304-20, 2020
                                                                                  3. Sanchez F et al: Pathological entities that may affect the lungs and the myocardium. Evaluation with chest CT and cardiac MR. Clin Imaging. 70:124-35, 2020
                                                                                  4. Elicker BM et al: Imaging of the thoracic manifestations of connective tissue disease. Clin Chest Med. 40(3):655-66, 2019
                                                                                  5. Kusmirek JE et al: Thoracic manifestations of connective tissue diseases. Semin Ultrasound CT MR. 40(3):239-54, 2019
                                                                                  6. Strollo D et al: Imaging lung disease in systemic sclerosis. Curr Rheumatol Rep. 12(2):156-61, 2010
                                                                                  7. Lynch DA: Lung disease related to collagen vascular disease. J Thorac Imaging. 24(4):299-309, 2009
                                                                                  8. de Azevedo AB et al: Prevalence of pulmonary hypertension in systemic sclerosis. Clin Exp Rheumatol. 23(4):447-54, 2005
                                                                                  9. Galie N et al: Pulmonary arterial hypertension associated to connective tissue diseases. Lupus. 14(9):713-7, 2005
                                                                                  10. Highland KB et al: New Developments in Scleroderma Interstitial Lung Disease. Curr Opin Rheumatol. 17(6):737-45, 2005
                                                                                  11. Desai SR et al: CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology. 232(2):560-7, 2004
                                                                                  12. Kim EA et al: Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. Radiographics. 2002 Oct;22 Spec No:S151-65. Review. Erratum in: Radiographics. 23(5):1340, 2003
                                                                                  13. Kim EA et al: Interstitial pneumonia in progressive systemic sclerosis: serial high-resolution CT findings with functional correlation. J Comput Assist Tomogr. 25(5):757-63, 2001
                                                                                  14. Diot E et al: Relationship between abnormalities on high-resolution CT and pulmonary function in systemic sclerosis. Chest. 114(6):1623-9, 1998