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What are the Characteristics of Central Nervous System Infections in SLE?

Meningitis is the most frequent clinical syndrome in SLE patients with CNS infections. C. neoformans, M. tuberculosis and L. monocytogenes are the most common causative pathogens.

In patients with systemic lupus erythematosus (SLE) with suspected central nervous system (CNS) tuberculosis (TB), empiric anti-TB treatment is justified while waiting on the results of diagnostic tests. This is according to a meta-analysis published in Lupus Science & Medicine1 that aimed to describe the clinical and microbiological characteristics, and outcomes of CNS infections in patients with SLE.

CNS infections are associated with significant morbidity and mortality in immunocompromised patients with SLE. While early diagnosis and appropriate treatment are critical, there is limited information about the clinical characteristics and outcome of CNS infections in patients with SLE.

The meta-analysis included 6 studies involving 17,751 patients with SLE and 209 SLE cases (89.5% female, mean age 32.2 years) with CNS infection.

The frequency rate was 0.012 for CNS infections in patients with SLE. The most common clinical syndrome was meningitis (93.5%), while the most common causative pathogens were Cryptococcus neoformans (35.9%), Mycobacterium tuberculosis (27.1%) and Listeria monocytogenes (17.4%). Most patients (92.4%) were taking oral systemic corticosteroids, with a prednisone equivalent mean daily dose of 30.9 mg/day. The mean SLE Disease Activity Index (SLEDAI) score was 7.9.

“Clinicians should maintain a high index of suspicion for cryptococcal and TB meningitis in patients with SLE with suspected CNS infection, particularly in those with higher SLEDAI and on higher doses of systemic corticosteroids,” wrote Fereshte Sheybani, MD, of Mashhad University of Medical Sciences in Iran, and colleagues.

A time interval of 52 months was seen between the diagnosis of SLE and CNS infection and the interval from neurological symptom onset to the diagnosis of CNS infection was 14.2 days. Headache and fever were the most common symptoms of CNS infection. The mortality rate was 29%, the persistent neurological sequelae rate was 13.3% and the relapse rate was 4.7%.

The high frequency of C. neoformans as a causative pathogen of CNS infections suggests that “antifungals might be potentially useful empirically in patients with SLE who are suspected to have fungal CNS infections,” the investigators wrote. “However, with respect to side effects such as toxicity and high cost of antifungals, decision regarding early antifungal therapy should be guided by early and less time-consuming fungal diagnostic tests.”

Reference:

Molooghi K, Sheybani F, Naderi H, et al. Central nervous system infections in patients with systemic lupus erythematosus: a systematic review and meta-analysis. Lupus Science Medicine 2022;9:e000560. doi: 10.1136/lupus-2021-000560