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Immune Reconstitution Inflammatory Syndrome (IRIS)
Miral D. Jhaveri, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Immune reconstitution inflammatory syndrome (IRIS)
              • Synonyms

                • Neuro-IRIS
              • Definitions

                • Dysregulated immune response with worsening/unmasking of preexisting opportunist infection
                  • After starting antiretroviral therapy (ART)
                  • Patients with multiple sclerosis (MS), immunomodulatory therapy

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. Bartsch T et al: The spectrum of progressive multifocal leukoencephalopathy: a practical approach. Eur J Neurol. 26(4):566-e41, 2019
                                        2. Bowen L et al: CNS immune reconstitution inflammatory syndrome. Handb Clin Neurol. 152:167-76, 2018
                                        3. Dellière S et al: Understanding pathogenesis and care challenges of immune reconstitution inflammatory syndrome in fungal infections. J Fungi (Basel). 4(4), 2018
                                        4. Sueki H et al: Immune reconstitution inflammatory syndrome in non-HIV immunosuppressed patients. J Dermatol. 45(1):3-9, 2018
                                        5. Calic Z et al: Treatment of progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome with intravenous immunoglobulin in a patient with multiple sclerosis treated with fingolimod after discontinuation of natalizumab. J Clin Neurosci. 22(3):598-600, 2015
                                        6. Post MJ et al: CNS-immune reconstitution inflammatory syndrome in the setting of HIV infection, part 1: overview and discussion of progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome and cryptococcal-immune reconstitution inflammatory syndrome. AJNR Am J Neuroradiol. 34(7):1297-307, 2013
                                        7. Post MJ et al: CNS-immune reconstitution inflammatory syndrome in the setting of HIV infection, part 2: discussion of neuro-immune reconstitution inflammatory syndrome with and without other pathogens. AJNR Am J Neuroradiol. 34(7):1308-18, 2013
                                        8. Kranick SM et al: Neurologic complications of HIV-1 infection and its treatment in the era of antiretroviral therapy. Continuum (Minneap Minn). 18(6 Infectious Disease):1319-37, 2012
                                        9. Major EO: Progressive multifocal leukoencephalopathy in patients on immunomodulatory therapies. Annu Rev Med. 61:35-47, 2010
                                        10. Elston JW et al: Immune reconstitution inflammatory syndrome. Int J STD AIDS. 20(4):221-4, 2009
                                        11. McCombe JA et al: Neurologic immune reconstitution inflammatory syndrome in HIV/AIDS: outcome and epidemiology. Neurology. 72(9):835-41, 2009
                                        12. Pepper DJ et al: Neurologic manifestations of paradoxical tuberculosis-associated immune reconstitution inflammatory syndrome: a case series. Clin Infect Dis. 48(11):e96-107, 2009
                                        13. Smith AB et al: From the archives of the AFIP: central nervous system infections associated with human immunodeficiency virus infection: radiologic-pathologic correlation. Radiographics. 2008 Nov-Dec;28(7):2033-58. Review. Erratum in: Radiographics. 29(2):638, 2009
                                        14. Sun HY et al: Immune reconstitution inflammatory syndrome in non-HIV immunocompromised patients. Curr Opin Infect Dis. 22(4):394-402, 2009
                                        15. Tan K et al: PML-IRIS in patients with HIV infection: clinical manifestations and treatment with steroids. Neurology. 72(17):1458-64, 2009
                                        16. Tremont-Lukats IW et al: The immune inflammatory reconstitution syndrome and central nervous system toxoplasmosis. Ann Intern Med. 150(9):656-7, 2009
                                        17. Meintjes G et al: Prevention and treatment of the immune reconstitution inflammatory syndrome. Curr Opin HIV AIDS. 3(4):468-76, 2008
                                        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

                                                      • Immune reconstitution inflammatory syndrome (IRIS)
                                                    • Synonyms

                                                      • Neuro-IRIS
                                                    • Definitions

                                                      • Dysregulated immune response with worsening/unmasking of preexisting opportunist infection
                                                        • After starting antiretroviral therapy (ART)
                                                        • Patients with multiple sclerosis (MS), immunomodulatory therapy

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. Bartsch T et al: The spectrum of progressive multifocal leukoencephalopathy: a practical approach. Eur J Neurol. 26(4):566-e41, 2019
                                                                              2. Bowen L et al: CNS immune reconstitution inflammatory syndrome. Handb Clin Neurol. 152:167-76, 2018
                                                                              3. Dellière S et al: Understanding pathogenesis and care challenges of immune reconstitution inflammatory syndrome in fungal infections. J Fungi (Basel). 4(4), 2018
                                                                              4. Sueki H et al: Immune reconstitution inflammatory syndrome in non-HIV immunosuppressed patients. J Dermatol. 45(1):3-9, 2018
                                                                              5. Calic Z et al: Treatment of progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome with intravenous immunoglobulin in a patient with multiple sclerosis treated with fingolimod after discontinuation of natalizumab. J Clin Neurosci. 22(3):598-600, 2015
                                                                              6. Post MJ et al: CNS-immune reconstitution inflammatory syndrome in the setting of HIV infection, part 1: overview and discussion of progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome and cryptococcal-immune reconstitution inflammatory syndrome. AJNR Am J Neuroradiol. 34(7):1297-307, 2013
                                                                              7. Post MJ et al: CNS-immune reconstitution inflammatory syndrome in the setting of HIV infection, part 2: discussion of neuro-immune reconstitution inflammatory syndrome with and without other pathogens. AJNR Am J Neuroradiol. 34(7):1308-18, 2013
                                                                              8. Kranick SM et al: Neurologic complications of HIV-1 infection and its treatment in the era of antiretroviral therapy. Continuum (Minneap Minn). 18(6 Infectious Disease):1319-37, 2012
                                                                              9. Major EO: Progressive multifocal leukoencephalopathy in patients on immunomodulatory therapies. Annu Rev Med. 61:35-47, 2010
                                                                              10. Elston JW et al: Immune reconstitution inflammatory syndrome. Int J STD AIDS. 20(4):221-4, 2009
                                                                              11. McCombe JA et al: Neurologic immune reconstitution inflammatory syndrome in HIV/AIDS: outcome and epidemiology. Neurology. 72(9):835-41, 2009
                                                                              12. Pepper DJ et al: Neurologic manifestations of paradoxical tuberculosis-associated immune reconstitution inflammatory syndrome: a case series. Clin Infect Dis. 48(11):e96-107, 2009
                                                                              13. Smith AB et al: From the archives of the AFIP: central nervous system infections associated with human immunodeficiency virus infection: radiologic-pathologic correlation. Radiographics. 2008 Nov-Dec;28(7):2033-58. Review. Erratum in: Radiographics. 29(2):638, 2009
                                                                              14. Sun HY et al: Immune reconstitution inflammatory syndrome in non-HIV immunocompromised patients. Curr Opin Infect Dis. 22(4):394-402, 2009
                                                                              15. Tan K et al: PML-IRIS in patients with HIV infection: clinical manifestations and treatment with steroids. Neurology. 72(17):1458-64, 2009
                                                                              16. Tremont-Lukats IW et al: The immune inflammatory reconstitution syndrome and central nervous system toxoplasmosis. Ann Intern Med. 150(9):656-7, 2009
                                                                              17. Meintjes G et al: Prevention and treatment of the immune reconstitution inflammatory syndrome. Curr Opin HIV AIDS. 3(4):468-76, 2008