RESEARCH - PML: a national estimate of frequency in SLE and other rheumatic diseases

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Arthritis Rheum. 2009 Dec;60(12):3761-5.

Progressive multifocal leukoencephalopathy: A national estimate of

frequency in systemic lupus erythematosus and other rheumatic

diseases.

Molloy ES, Calabrese LH.

Cleveland Clinic, Cleveland, Ohio.

OBJECTIVE: Progressive multifocal leukoencephalopathy (PML) is a rare,

typically fatal, central nervous system demyelinating disease that

results from reactivation of the JC virus, which generally occurs in

immunosuppressed hosts. The aim of this study was to generate a

national estimate of the frequency of PML among patients with

rheumatic diseases.

METHODS: Data were obtained from the Nationwide Inpatient Sample

database. This is a 20% sample of all hospital discharges, weighted to

represent the entire US inpatient population. Data were analyzed for

the years 1998-2005 inclusive, representing 297,797,180 hospital

discharges. Cases of PML, systemic lupus erythematosus (SLE), and

other rheumatic diseases were identified by diagnostic codes from the

International Classification of Diseases, Ninth Revision, Clinical

Modification.

RESULTS: A total of 9,675 cases of PML were identified. The majority

were associated with human immunodeficiency virus (HIV; 7,934 patients

[82.00%]), hematologic cancers (813 patients [8.40%]), and solid

cancers (274 patients [2.83%]). Among the rheumatic diseases, 43 cases

of PML (0.44%) were associated with SLE, 24 (0.25%) with rheumatoid

arthritis (RA), and 25 (0.26%) with other connective tissue diseases

(CTDs). When patients with other potential risk factors for PML (HIV,

malignancy, bone marrow or other organ transplantation) were excluded,

the rates of PML per 100,000 discharges coded for SLE, RA, and other

CTDs were 4, 0.4 and 2, respectively, compared with a rate of PML in

the background population of 0.2/100,000 discharges.

CONCLUSION: This study was confined to hospitalized patients with

rheumatic diseases, and it was also limited by the lack of information

regarding immunosuppressive therapy. Nevertheless, the findings

suggest that, although rare overall, PML occurs more commonly in SLE

than in other rheumatic diseases.

PMID: 19950261

http://www.ncbi.nlm.nih.gov/pubmed/19950261

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