Background/Purpose:  In the era of powerful immunosuppression, opportunistic infections are an increasing concern in Systemic Lupus Erythematosus (SLE). A potentially fatal opportunistic demyelinating disease of the central nervous system, progressive multifocal leukoencephalopathy (PML), results from infection of oligodendrocytes by John Cunningham Virus (JC virus). Reactivation of the latent JC virus was recently described in SLE patients; the incidence of PML was estimated at 4/100,000 SLE discharges. This study was initiated to evaluate the prevalence of PML in adult and pediatric SLE patients at two large academic centers, with a focus on validating PML and SLE diagnoses with clinical information obtained from corresponding medical records, in order to better define the risk of PML in SLE.

Methods:  This is a retrospective cohort study evaluating the prevalence of PML in two large academic centers. Patients with SLE were identified using the validated method of either an International Classification of Diseases, Ninth Revision (ICD-9) code of 710.0 or 695.4. All patients with SLE admitted to Columbia University Medical Center (CUMC) between 1986 and 2013 using electronic medical record data (EMR) from the Clinical Data Warehouse, or at Northwell Health (NWH) between 2013 and 2016, were included. Patients with Rheumatoid Arthritis (RA), identified by ICD-9 code 714, were evaluated as the disease control group. Among the case and control groups, cases of PML were identified using ICD-9 code of 046.3. Medication exposure was evaluated in the SLE patients.

Results:  A total of 5409 individual SLE patients admitted to CUMC from 1983 to 2013 and 1788 SLE patients admitted to NWH from 2013 to 2016 were identified. Of these 6847 SLE patients, 3 patients also had an ICD-9 diagnosis code of 046.3 for PML. Upon review of the EMR, the diagnosis of PML was substantiated for one patient; the second patient was evaluated for PML but had CSF negative for JC virus and was treated for CNS lupus. EMR admission data could not be retrieved for the third patient and the diagnosis could not be confirmed. None of the 10,776 patients admitted for RA at CUMC had PML. Out of the 5409 SLE patients at CUMC, 212 were also renal transplant recipients and 83 had concomitant HIV/AIDS. Based on inpatient pharmacy records of the 5409 hospitalized SLE patients at CUMC, 59.2% were treated with steroids and 16.09% with immunosuppressants (7.76% mycophenolate, 3.42% cyclophosphamide, 2.88% azathioprine and 2.03% rituximab). Of note, none of the patients with PML had juvenile SLE (jSLE). At CUMC, the prevalence of PML in hospitalized patients is between 1.8-3.7 per 10,000 discharges and 0 at NWH. Based on these data, the combined prevalence of PML in hospitalized SLE patients at the two hospitals is between 1 and 2 per 7,197 admitted patients=1.4-2.8/10,000.

Conclusion:  The prevalence of PML in adult SLE patients is less than 3/10,000 patients; PML has not yet been documented in jSLE. These data do not substantiate the need for JC virus screening in SLE patients prior to initiation of immunosuppression. Additionally, our experience emphasizes the need for thorough review of the data obtained from EMR.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prevalence-of-progressive-multifocal-leukoencephalopathy-in-adults-and-children-with-systemic-lupus-erythematosus/