Resumo Antecedentes A síndrome inflamatória de reconstituição imune (SIRI) da leucoencefalopatia multifocal progressiva (LEMP) em pessoas vivendo com HIV/Aids (PVHA) foi raramente descrita em países de baixa e média renda. Objetivo Descrever a prevalência da SIRI-LEMP- em PVHA com LEMP e suas principais características em um hospital no Brasil. Métodos Foi realizado um estudo de coorte retrospectivo. Incluímos PVHA com SIRI-LEMP admitidos no Instituto de Infectologia Emílio Ribas, São Paulo, Brasil, entre 2011 e 2021. Recuperamos informações sobre manifestações neurológicas, neuroimagem, tratamento e desfecho. Resultados Identificamos 11 (11,8%) casos de SIRI-LEMP entre 93 pacientes com LEMP definitiva. Oito (73%) casos eram homens e a mediana de idade (amplitude interquartile - AIQ) foi de 41 (27–50) anos. Sete (63,6%) pacientes desenvolveram SIRI-LEMP “desmascarada” e 4 (36,4%) casos apresentaram SIRI-LEMP “paradoxal”. A mediana de tempo (AIQ) desde o início da terapia antirretroviral combinada (cART) até o diagnóstico de SIRI foi de 49 (30–70) dias. Dez (90,9%) pacientes receberam corticoide. Houve 4 (36%) óbitos intra-hospitalares e 3 foram associados à pneumonia hospitalar. Dos 7 (64%) pacientes que sobreviveram, 5 (71,5%) ficaram com sequelas na alta. Um ano após o diagnóstico de SIRI-LEMP, 6 (54,5%) pacientes estavam vivos. Conclusão A prevalência de SIRI-LEMP foi de 11,8%. A maioria dos pacientes apresentava SIRI-LEMP “desmascarada”. A mortalidade e morbidade hospitalar foram altas. A sobrevida em 1 ano foi semelhante à descrita em alguns países de alta renda. (SIRI (LEMP HIVAids HIV Aids (PVHA renda SIRILEMP Brasil retrospectivo Ribas Paulo 201 2021 neurológicas neuroimagem desfecho 11,8% 118 8 (11,8% 9 definitiva 73% 73 (73% amplitude AIQ 27–50 2750 27 50 (27–50 anos 63,6% 636 63 (63,6% desmascarada “desmascarada 36,4% 364 36 (36,4% paradoxal. paradoxal . “paradoxal” (AIQ cART (cART 30–70 3070 30 70 (30–70 dias 90,9% 909 90 (90,9% corticoide 36% (36% intrahospitalares intra hospitalares 64% 64 (64% sobreviveram 71,5% 715 71 (71,5% SIRILEMP, LEMP, 54,5% 545 54 (54,5% vivos desmascarada. altas 20 202 11,8 (11,8 (73 27–5 275 2 (27–5 63,6 (63,6 36,4 (36,4 “paradoxal 30–7 307 (30–7 90,9 (90,9 (36 (64 71,5 (71,5 54,5 (54,5 11, (11, (7 27– (27– 63, (63, 36, (36, 30– (30– 90, (90, (3 (6 71, (71, 54, (54, (11 ( (27 (63 (30 (90 (71 (54 (1 (2 (9 (5
Abstract Background Progressive multifocal leukoencephalopathy (PML) - immune reconstitution inflammatory syndrome (IRIS) in people living with HIV/AIDS (PLWHA) has been rarely described in low- and middle-income countries. Objective To describe the prevalence of PML-IRIS among PLWHA with PML and its main features in a tertiary hospital in Brazil. Methods We performed a retrospective cohort study. We included PLWHA with PML-IRIS patients admitted at Instituto de Infectologia Emílio Ribas, São Paulo, Brazil, between 2011 and 2021. We retrieved information on neurological manifestations, neuroimaging findings, treatments, and outcomes. Results We identified 11 (11.8%) PML-IRIS cases among 93 patients with definite PML. Eight (73%) cases were men and had a median (IQR) age of 41 (27–50) years. Seven (63.6%) patients developed unmasking PML-IRIS and 4 (36.4%) had paradoxical PML-IRIS. The median (IQR) time from initiation of combined antiretroviral therapy (cART) to IRIS diagnosis was 49 (30–70) days. Ten (90.9%) patients received corticosteroids. There were 4 (36%) in-hospital deaths and 3 were associated with hospital-acquired pneumonia. Among the 7 (64%) patients who survived, 5 (71.5%) had sequelae at discharge. One year after the PML-IRIS diagnosis, 6 (54.5%) patients were alive. Conclusion The prevalence of PML-IRIS was 11.8%. Most patients had unmasking PML-IRIS. In-hospital mortality and morbidity were high. One-year survival was similar to that described in some high-income countries. (PML (IRIS HIVAIDS HIV AIDS (PLWHA low middleincome middle income countries PMLIRIS Brazil study Ribas Paulo 201 2021 manifestations findings treatments outcomes 1 11.8% 118 8 (11.8% 9 73% 73 (73% IQR (IQR 27–50 2750 27 50 (27–50 years 63.6% 636 63 (63.6% 36.4% 364 36 (36.4% PMLIRIS. IRIS. cART (cART 30–70 3070 30 70 (30–70 days 90.9% 909 90 (90.9% corticosteroids 36% (36% inhospital hospitalacquired acquired pneumonia 64% 64 (64% survived 71.5% 715 71 (71.5% discharge 54.5% 545 54 (54.5% alive Inhospital In high Oneyear highincome 20 202 11.8 (11.8 (73 27–5 275 2 (27–5 63.6 (63.6 36.4 (36.4 30–7 307 (30–7 90.9 (90.9 (36 (64 71.5 (71.5 54.5 (54.5 11. (11. (7 27– (27– 63. (63. 36. (36. 30– (30– 90. (90. (3 (6 71. (71. 54. (54. (11 ( (27 (63 (30 (90 (71 (54 (1 (2 (9 (5