Journal Article

Progressive multifocal leukoencephalopathy associated with systemic lupus erythematosus: longitudinal observation of lymphocytes, JC virus in cerebrospinal fluid, and brain magnetic resonance imaging

Nakamichi, Kazuo, Maruyama, Hirofumi, Sugimoto, Tomohiro, Yasutomi, Hiroko, Toko, Megumi, Ueno, Hiroki, Yamada, Hidetada, Nakamori, Masahiro, Aoki, Shiro

2024

Overview

Progressive multifocal leukoencephalopathy (PML) rarely occurs in patients with systemic lupus erythematosus (SLE). This report presents the case of a patient who developed PML due to SLE-associated multiple factors. A 60-year-old woman diagnosed with SLE undergoing multiple immunosuppressive therapies, including azathioprine, presented with cerebral cortical symptoms, lymphocytopenia, and vitamin B12 deficiency and was subsequently diagnosed with SLE-associated PML. We evaluated the cause and disease activity of PML, focusing on the longitudinal assessment of lymphocytopenia, JC virus (JCV) DNA copy number in the cerebrospinal fluid, and magnetic resonance imaging (MRI) findings. Discontinuing azathioprine and initiating alternative immunosuppressive treatments with intramuscular vitamin B12 injections affected lymphocytopenia and disease management. However, despite recovery from lymphopenia and JCV DNA copy number being low, the large hyperintense and punctate lesions observed on the fluid-attenuated inversion recovery (FLAIR) images exhibited varying behaviors, indicating that the balance between contributing factors for PML may have fluctuated after the initial treatment. Clinicians should be meticulous when assessing the underlying pathology of the multifactorial causes of PML due to SLE. The difference in the transition pattern of these lesions on FLAIR images may be one of the characteristics of MRI findings in PML associated with SLE, reflecting fluctuations in disease activity and the progression stage of PML.

• Publisher: Springer International Publishing,Nature Publishing Group
• Subject: Antibodies / Apraxia / Azathioprine / Azathioprine - therapeutic use / Biomedical and Life Sciences / Biomedicine / Blood / Brain - diagnostic imaging / Brain - pathology / Brain - virology / Case Report / Case reports / Cerebrospinal fluid / Consent / Copy number / Disease / DNA viruses / DNA, Viral - cerebrospinal fluid / DNA, Viral - genetics / Female / Hospitals / Humans / Immunology / Immunosuppressive agents / Immunosuppressive Agents - therapeutic use / Infectious Diseases / JC Virus - genetics / JC Virus - pathogenicity / Lesions / Leukoencephalopathy / Leukoencephalopathy, Progressive Multifocal - cerebrospinal fluid / Leukoencephalopathy, Progressive Multifocal - diagnostic imaging / Leukoencephalopathy, Progressive Multifocal - drug therapy / Leukoencephalopathy, Progressive Multifocal - pathology / Leukoencephalopathy, Progressive Multifocal - virology / Lupus / Lupus Erythematosus, Systemic - cerebrospinal fluid / Lupus Erythematosus, Systemic - complications / Lupus Erythematosus, Systemic - diagnostic imaging / Lupus Erythematosus, Systemic - drug therapy / Lupus Erythematosus, Systemic - virology / Lymphocytes / Lymphocytes - immunology / Lymphocytes - pathology / Lymphocytes - virology / Lymphopenia / Lymphopenia - cerebrospinal fluid / Lymphopenia - complications / Lymphopenia - diagnostic imaging / Lymphopenia - virology / Magnetic Resonance Imaging / Middle Aged / Neuroimaging / Neurology / Neurosciences / Progressive multifocal leukoencephalopathy / Systemic lupus erythematosus / Virology / Viruses / Vitamin B12 / Vitamin deficiency