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Adult Still Disease
Eva M. Escobedo, MD; B. J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Adult-onset Still disease (AOSD), Wissler-Fanconi syndrome
          • Definitions

            • Systemic inflammatory disease, featuring triad
              • Quotidian fevers (spiking, daily)
              • Evanescent rash
              • Chronic oligoarthritis or polyarthritis
            • Considered adult continuum of systemic juvenile idiopathic arthritis (SJIA)

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nuclear Medicine Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Microscopic Features

                            • Diagnostic Criteria (After Yamaguchi)

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Mitrovic S et al: Clinical phenotypes of adult-onset still's disease: new insights from pathophysiology and literature findings. J Clin Med. 10(12):2633, 2021
                                        2. Zhou X et al: FDG PET/CT used in identifying adult-onset Still's disease in connective tissue diseases. Clin Rheumatol. 39(9):2735-42, 2020
                                        3. Lebrun D et al: Validation of the Fautrel classification criteria for adult-onset Still's disease. Semin Arthritis Rheum. 47(4):578-85, 2018
                                        4. Dong MJ et al: 18F-FDG PET/CT in patients with adult-onset Still's disease. Clin Rheumatol. 34(12):2047-56, 2015
                                        5. Kadavath S et al: Adult-onset Still's disease-pathogenesis, clinical manifestations, and new treatment options. Ann Med. 47(1):6-14, 2015
                                        6. Manzini CU et al: Elevated troponin serum levels in adult onset Still's disease. Case Rep Rheumatol. 2015:732095, 2015
                                        7. Hayem F: Is Still's disease an autoinflammatory syndrome? Joint Bone Spine. 76(1):7-9, 2009
                                        8. Jadhav P et al: Myocarditis in adult onset Stills disease. Int J Rheum Dis. 12(3):272-4, 2009
                                        9. Lee SW et al: The mid-range of the adjusted level of ferritin can predict the chronic course in patients with adult onset Still's disease. J Rheumatol. 36(1):156-62, 2009
                                        10. Efthimiou P et al: Autoinflammatory syndromes and infections: pathogenetic and clinical implications. Clin Exp Rheumatol. 26(1 Suppl 48):S53-61, 2008
                                        11. Fautrel B: Adult-onset Still disease. Best Pract Res Clin Rheumatol. 22(5):773-92, 2008
                                        12. Kontzias A et al: Adult-onset Still's disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs. 68(3):319-37, 2008
                                        13. Singh S et al: Adult onset Still's disease: a study of 14 cases. Clin Rheumatol. 27(1):35-9, 2008
                                        14. Yamaguchi M et al: Preliminary criteria for classification of adult Still's disease. J Rheumatol. 19(3):424-30, 1992
                                        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

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Adult-onset Still disease (AOSD), Wissler-Fanconi syndrome
                                                • Definitions

                                                  • Systemic inflammatory disease, featuring triad
                                                    • Quotidian fevers (spiking, daily)
                                                    • Evanescent rash
                                                    • Chronic oligoarthritis or polyarthritis
                                                  • Considered adult continuum of systemic juvenile idiopathic arthritis (SJIA)

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Ultrasonographic Findings

                                                          • Nuclear Medicine Findings

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Microscopic Features

                                                                  • Diagnostic Criteria (After Yamaguchi)

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Mitrovic S et al: Clinical phenotypes of adult-onset still's disease: new insights from pathophysiology and literature findings. J Clin Med. 10(12):2633, 2021
                                                                              2. Zhou X et al: FDG PET/CT used in identifying adult-onset Still's disease in connective tissue diseases. Clin Rheumatol. 39(9):2735-42, 2020
                                                                              3. Lebrun D et al: Validation of the Fautrel classification criteria for adult-onset Still's disease. Semin Arthritis Rheum. 47(4):578-85, 2018
                                                                              4. Dong MJ et al: 18F-FDG PET/CT in patients with adult-onset Still's disease. Clin Rheumatol. 34(12):2047-56, 2015
                                                                              5. Kadavath S et al: Adult-onset Still's disease-pathogenesis, clinical manifestations, and new treatment options. Ann Med. 47(1):6-14, 2015
                                                                              6. Manzini CU et al: Elevated troponin serum levels in adult onset Still's disease. Case Rep Rheumatol. 2015:732095, 2015
                                                                              7. Hayem F: Is Still's disease an autoinflammatory syndrome? Joint Bone Spine. 76(1):7-9, 2009
                                                                              8. Jadhav P et al: Myocarditis in adult onset Stills disease. Int J Rheum Dis. 12(3):272-4, 2009
                                                                              9. Lee SW et al: The mid-range of the adjusted level of ferritin can predict the chronic course in patients with adult onset Still's disease. J Rheumatol. 36(1):156-62, 2009
                                                                              10. Efthimiou P et al: Autoinflammatory syndromes and infections: pathogenetic and clinical implications. Clin Exp Rheumatol. 26(1 Suppl 48):S53-61, 2008
                                                                              11. Fautrel B: Adult-onset Still disease. Best Pract Res Clin Rheumatol. 22(5):773-92, 2008
                                                                              12. Kontzias A et al: Adult-onset Still's disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs. 68(3):319-37, 2008
                                                                              13. Singh S et al: Adult onset Still's disease: a study of 14 cases. Clin Rheumatol. 27(1):35-9, 2008
                                                                              14. Yamaguchi M et al: Preliminary criteria for classification of adult Still's disease. J Rheumatol. 19(3):424-30, 1992