Progressive multifocal leucoencephalopathy associated with lupus and methotrexate overdose
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Published on: June 8, 2022
1Department of Neurology, University of Rochester, Rochester, New York, USA. david_shprecher@urmc.rochester.edu
Abstract:
Progressive multifocal leucoencephalopathy (PML) is a CNS infection of oligodendrocytes by JC virus, which rarely occurs in lupus, and can be mistaken for antiphospholipid antibody syndrome or neuropsychiatric systemic lupus erythematosus (NSLE). This case of PML in a patient with systemic lupus erythematosus on supra-therapeutic doses of methotrexate emphasises that CNS infection is an important diagnostic consideration before empiric treatment with immunosuppresants for NSLE.
Insights
Progressive multifocal leukoencephalopathy (PML), a rare JC virus CNS infection, can mimic lupus complications. Early diagnosis is crucial before immunosuppressive therapy for neuropsychiatric lupus.
Area of Science:
- Neurology
- Infectious Diseases
- Immunology
Background:
- Systemic lupus erythematosus (SLE) can present with diverse neurological manifestations.
- Neuropsychiatric lupus (NSLE) diagnosis can be challenging, often requiring exclusion of other conditions.
- JC virus (JCV) causes Progressive Multifocal Leukoencephalopathy (PML), a rare but serious CNS demyelinating disease.
Observation:
- A case of PML in a patient with SLE is presented.
- The patient was receiving supra-therapeutic doses of methotrexate.
- PML was initially considered in the differential diagnosis of the patient's neurological symptoms.
Findings:
- PML, a CNS infection by JC virus, rarely occurs in lupus patients.
- PML can be misdiagnosed as antiphospholipid antibody syndrome or NSLE.
- The presented case highlights PML as a critical consideration in SLE patients with CNS symptoms.
Implications:
- Central nervous system (CNS) infections must be considered before initiating immunosuppressants for NSLE.
- Supra-therapeutic methotrexate doses may increase the risk of opportunistic infections like PML in SLE patients.
- This case underscores the importance of vigilant diagnosis in SLE to differentiate infectious from autoimmune neurological conditions.
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