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Published on: September 12, 2016
Effective immune restoration after immunosuppressant discontinuation in a lupus patient presenting progressive
Guillaume Lefèvre1, Viviane Queyrel, Claude-Alain Maurage
1Department of Internal Medicine, National Reference Centre for Rare Systemic and Autoimmune Diseases (Systemic Sclerosis), Regional University Hospital and Henri Warembourg School of Medicine, Lille 2 University, Lille, France. guill_lefevre@hotmail.com
Abstract:
Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection of the central nervous system with JC virus. Few cases have been described in lupus patients. We describe biopsy-proven PML in a lupus patient receiving mycophenolate mofetil and corticosteroids. Although the patient received no antiviral treatment, the polymerase chain reaction test for JC virus became negative in cerebrospinal fluid after immunosuppressant discontinuation and the patient survived. We discuss the restoration of immune efficiency after immunosuppressant discontinuation in this case and compare the clinical, radiological and histological features with the inflammatory PML form described in human immunodeficiency virus-infected patients.
Insights
Progressive multifocal leukoencephalopathy (PML), a rare JC virus infection, occurred in a lupus patient on immunosuppressants. Discontinuing these drugs led to JC virus clearance and patient survival, highlighting immune restoration
Area of Science:
- Neuroimmunology
- Infectious Diseases
- Rheumatology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic CNS infection caused by JC virus.
- PML is infrequently reported in patients with systemic lupus erythematosus (SLE).
- Immunosuppressive therapies, including corticosteroids and mycophenolate mofetil, are common in SLE management and can increase PML risk.
Observation:
- A case of biopsy-proven PML is presented in a lupus patient undergoing treatment with mycophenolate mofetil and corticosteroids.
- The patient did not receive specific antiviral therapy for PML.
- Cerebrospinal fluid JC virus polymerase chain reaction (PCR) testing became negative after cessation of immunosuppressive agents.
Findings:
- Discontinuation of immunosuppressants resulted in the clearance of JC virus from the cerebrospinal fluid.
- The patient survived the PML episode, suggesting a favorable prognosis with immune reconstitution.
- Clinical, radiological, and histological features were compared with inflammatory PML in HIV-infected patients.
Implications:
- This case underscores the potential for immune recovery to control PML in immunocompromised patients, even without direct antiviral treatment.
- It highlights the importance of considering PML in lupus patients on immunosuppressive therapy presenting with neurological symptoms.
- Understanding the dynamics of immune restoration is crucial for managing opportunistic infections in immunosuppressed populations.
