BACKGROUND: Cryptococcal meningitis (CM) is a rare condition in non-HIV patients. The clinical manifestations in patients with systemic lupus erythematosus (SLE) are often confused with immunological activity, resulting in delayed diagnosis. AIMS: We describe the natural history and outcome of eight HIV-negative patients with SLE and confirmed CM. RESULTS: Mean age at the time of infection was 30·2 years (20-42). Mean dose of prednisone was 38·3 ± 13 mg/day and of azathioprine was 95 ± 37 mg/day. The most common clinical manifestation was headache with nausea or vomit (75%), followed by altered sensorium (50%), fever (50%), cranial nerve deficits (37%), or seizures (25%). Mean time between symptoms onset and diagnosis was 19 days (6-56). All patients had low lymphocyte cell counts (504 ± 229 cells/μl) and low CD4+ cell counts (113·2 ± 59·2 cells/μl). Active SLE assessed by a systemic lupus erythematosus disease activity index (SLEDAI) score ≧4 was found in 83% patients at the time of the diagnosis and 87% had renal involvement. The positivity of cryptococcal antigen, India ink stain, and culture in the cerebrospinal fluid (CSF) was 90, 70, and 50%, respectively. Magnetic resonance was abnormal in 90% of the patients. Higher titers of cryptococcal antigen were suggestive of worse outcome and increased hospital stay. After a mean follow-up of 4·9 years, one patient had a relapse of the CM, associated with persistent low CD4+ cell counts. CONCLUSIONS: Cryptococcal meningitis in patients with SLE was associated with severe delay in diagnosis and profound lymphopenia. Follow-up should include CD4+ cell counts, and maintenance treatment with fluconazole should be continued until lymphopenia resolution.
BACKGROUND:Cryptococcal meningitis (CM) is a rare condition in non-HIV patients. The clinical manifestations in patients with systemic lupus erythematosus (SLE) are often confused with immunological activity, resulting in delayed diagnosis. AIMS: We describe the natural history and outcome of eight HIV-negative patients with SLE and confirmed CM. RESULTS: Mean age at the time of infection was 30·2 years (20-42). Mean dose of prednisone was 38·3 ± 13 mg/day and of azathioprine was 95 ± 37 mg/day. The most common clinical manifestation was headache with nausea or vomit (75%), followed by altered sensorium (50%), fever (50%), cranial nerve deficits (37%), or seizures (25%). Mean time between symptoms onset and diagnosis was 19 days (6-56). All patients had low lymphocyte cell counts (504 ± 229 cells/μl) and low CD4+ cell counts (113·2 ± 59·2 cells/μl). Active SLE assessed by a systemic lupus erythematosus disease activity index (SLEDAI) score ≧4 was found in 83% patients at the time of the diagnosis and 87% had renal involvement. The positivity of cryptococcal antigen, India ink stain, and culture in the cerebrospinal fluid (CSF) was 90, 70, and 50%, respectively. Magnetic resonance was abnormal in 90% of the patients. Higher titers of cryptococcal antigen were suggestive of worse outcome and increased hospital stay. After a mean follow-up of 4·9 years, one patient had a relapse of the CM, associated with persistent low CD4+ cell counts. CONCLUSIONS:Cryptococcal meningitis in patients with SLE was associated with severe delay in diagnosis and profound lymphopenia. Follow-up should include CD4+ cell counts, and maintenance treatment with fluconazole should be continued until lymphopenia resolution.
Entities:
Keywords:
Central nervous system infections,; Cryptococcal meningitis,; Cryptococcosis in HIV-negative patients,; Fungal infections; Systemic connective tissue diseases,; Systemic lupus erythematosus,
Authors: Ching Soong Khoo; Marsilla Mariaty Marzukie; Soo Shu Yap; Wan Nur Nafisah Wan Yahya; Hui Jan Tan Journal: J Neurosci Rural Pract Date: 2020-03-03