Covert cryptococcal meningitis in a patient with systemic lupus erythematous

Cryptococcal meningitis is a rare but well-recognized illness with a high mortality rate in immunosuppressed patients with systemic lupus erythematosus (SLE). The diagnosis of cryptococcal meningitis in these patients can be challenging, especially in the emergency department (ED), as the clinical presentation may be non-specific, which can lead to delayed treatment.

To recognize risk factors associated with the development of cryptococcal meningitis infection in patients with SLE and to provide an update on the clinical presentation, prognosis, and therapeutic options.

A 21-year-old man with SLE presented with a 4-day history of headache, fever, nausea, and vomiting after being discharged from the ED 1 day before this visit, after lumbar puncture showed normal values. One week before, he had completed 7-day pulse therapy with intravenous cyclophosphamide and intravenous methylprednisone for lupus nephritis. The patient was febrile, but the remainder of the examination was normal. Laboratory data showed lymphopenia. Given his immunocompromised state, a cryptococcal antigen was added to cerebrospinal fluid (CSF) sent from the prior ED visit and was positive at a titer of 1:8. The patient was treated with amphotericin B and 5-flucytosine for 6 weeks. Ten months later the patient remained free of infection.

Normal neurological and CSF examination do not exclude cryptococcal meningitis in immunocompromised patients with SLE. India ink or, preferably, latex agglutination test and CSF fungal culture are recommended. A high level of suspicion is the key in the diagnosis of cryptococcal meningitis and will help avoid delays in treatment.