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Progressive Systemic Sclerosis
Brett W. Carter, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Progressive systemic sclerosis (PSS)
              • Synonyms

                • Scleroderma
                • Systemic sclerosis
              • Definitions

                • Generalized autoimmune connective tissue disorder affecting multiple organ systems including skin, lungs, heart, and kidneys
                • Limited cutaneous systemic sclerosis (60%)
                  • Skin involvement of hands, forearms, feet, and face
                  • Longstanding Raynaud phenomenon
                  • CREST syndrome: Calcinosis, Raynaud, esophageal dysmotility, sclerodactyly, and telangiectasias
                • Diffuse cutaneous systemic sclerosis (40%)
                  • Acute onset: Raynaud, 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. Capobianco J et al: Thoracic manifestations of collagen vascular diseases. Radiographics. 32(1):33-50, 2012
                                          2. Goldin JG et al: High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest. 134(2):358-67, 2008
                                          3. Madani G et al: The role of radiology in the management of systemic sclerosis. Clin Radiol. 63(9):959-67, 2008
                                          4. Wells AU: High-resolution computed tomography and scleroderma lung disease. Rheumatology (Oxford). 47 Suppl 5:v59-61, 2008
                                          5. 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
                                          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

                                                      • Abbreviations

                                                        • Progressive systemic sclerosis (PSS)
                                                      • Synonyms

                                                        • Scleroderma
                                                        • Systemic sclerosis
                                                      • Definitions

                                                        • Generalized autoimmune connective tissue disorder affecting multiple organ systems including skin, lungs, heart, and kidneys
                                                        • Limited cutaneous systemic sclerosis (60%)
                                                          • Skin involvement of hands, forearms, feet, and face
                                                          • Longstanding Raynaud phenomenon
                                                          • CREST syndrome: Calcinosis, Raynaud, esophageal dysmotility, sclerodactyly, and telangiectasias
                                                        • Diffuse cutaneous systemic sclerosis (40%)
                                                          • Acute onset: Raynaud, 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. Capobianco J et al: Thoracic manifestations of collagen vascular diseases. Radiographics. 32(1):33-50, 2012
                                                                                  2. Goldin JG et al: High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest. 134(2):358-67, 2008
                                                                                  3. Madani G et al: The role of radiology in the management of systemic sclerosis. Clin Radiol. 63(9):959-67, 2008
                                                                                  4. Wells AU: High-resolution computed tomography and scleroderma lung disease. Rheumatology (Oxford). 47 Suppl 5:v59-61, 2008
                                                                                  5. 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