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Characteristics of Progressive Multifocal Leukoencephalopathy Associated With Sarcoidosis Without Therapeutic Immune
Caleb R S McEntire1, Anita Fletcher2, Michel Toledano3
1Department of Neurology, Mass General Brigham, Boston, Massachusetts.
Importance:
Progressive multifocal leukoencephalopathy can occur in the context of systemic sarcoidosis (S-PML) in the absence of therapeutic immune suppression and can initially be mistaken for neurosarcoidosis or other complications of sarcoidosis. Earlier recognition of S-PML could lead to more effective treatment of the disease.
Objective:
To describe characteristics of patients with S-PML.
Design, Setting, And Participants:
For this case series, records from 8 academic medical centers in the United States were reviewed from 2004 to 2022. A systematic review of literature from 1955 to 2022 yielded data for additional patients. Included were patients with S-PML who were not receiving therapeutic immune suppression. The median follow-up time for patients who survived the acute range of illness was 19 months (range, 2-99). Data were analyzed in February 2023.
Exposures:
Sarcoidosis without active therapeutic immune suppression.
Main Outcomes And Measures:
Clinical, laboratory, and radiographic features of patients with S-PML.
Results:
Twenty-one patients with S-PML not receiving therapeutic immune suppression were included in this study, and data for 37 patients were collected from literature review. The median age of the 21 study patients was 56 years (range, 33-72), 4 patients (19%) were female, and 17 (81%) were male. The median age of the literature review patients was 49 years (range, 21-74); 12 of 34 patients (33%) with reported sex were female, and 22 (67%) were male. Nine of 21 study patients (43%) and 18 of 31 literature review patients (58%) had simultaneous presentation of systemic sarcoidosis and PML. Six of 14 study patients (43%) and 11 of 19 literature review patients (58%) had a CD4+ T-cell count greater than 200/μL. In 2 study patients, a systemic flare of sarcoidosis closely preceded S-PML development. Ten of 17 study patients (59%) and 21 of 35 literature review patients (60%) died during the acute phase of illness. No meaningful predictive differences were found between patients who survived S-PML and those who did not.
Conclusions And Relevance:
In this case series, patients with sarcoidosis developed PML in the absence of therapeutic immune suppression, and peripheral blood proxies of immune function were often only mildly abnormal. Systemic sarcoidosis flares may rarely herald the onset of S-PML. Clinicians should consider PML in any patient with sarcoidosis and new white matter lesions on brain magnetic resonance imaging.
Insights
Progressive multifocal leukoencephalopathy (PML) can occur in sarcoidosis patients without immunosuppression, often mimicking other sarcoidosis complications. Early recognition of sarcoidosis-associated PML is crucial for effective treatment.
Area of Science:
- Neurology
- Immunology
- Infectious Diseases
Background:
- Progressive multifocal leukoencephalopathy (PML) is typically associated with therapeutic immunosuppression.
- Systemic sarcoidosis can present with neurological manifestations, complicating diagnosis.
- Sarcoidosis-associated PML (S-PML) occurring without immunosuppression is rare and challenging to distinguish from other sarcoidosis-related neurological issues.
Approach:
- This study reviewed records from 8 US academic medical centers (2004-2022) and conducted a systematic literature review (1955-2022).
- Data from 21 patients with S-PML not receiving immunosuppression were analyzed, supplemented by 37 cases from the literature.
- Clinical, laboratory, and radiographic features were examined to characterize S-PML.
Key Points:
- S-PML occurred in sarcoidosis patients despite the absence of therapeutic immune suppression.
- Immune function markers, like CD4+ T-cell counts, were often only mildly abnormal.
- A systemic sarcoidosis flare occasionally preceded the onset of S-PML.
Conclusions:
- PML should be considered in sarcoidosis patients presenting with new white matter lesions on MRI, even without immunosuppression.
- S-PML diagnosis can be delayed due to its similarity to other sarcoidosis complications.
- Earlier identification of S-PML may improve patient outcomes, despite a high acute mortality rate.
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