Fatal cryptococcal meningitis in a juvenile lupus erythematosus patient

Cryptococcosis is a fungal infection caused by Cryptococcus neoformans, generally associated with immunodeficiency and immunosuppressive agents, and it is rarely reported in systemic lupus erythematosus (SLE), particularly in juvenile SLE (JSLE). From January 1983 to June 2011, 5,604 patients were followed at our University Hospital and 283 (5%) of them met the American College of Rheumatology (ACR) classification criteria for SLE. Only one (0.35%) of our JSLE patients had cryptococcal meningitis and is described in this report. A 10-year old girl was diagnosed with JSLE. By the age of 15 years, she presented persistent headaches, nausea and vomiting for a 5 day period without fever, after a cave-exploring trip. At that moment, she was under 10 mg/day of prednisone, azathioprine and hydroxychloroquine. A lumbar puncture was performed and India ink preparation was positive for cryptococcosis, cerebrospinal fluid culture yielded Cryptococcus neoformans and serum cryptococcal antigen titer was 1:128. Azathioprine was suspended, and liposomal amphotericin B was introduced. Despite of treatment, after four days she developed amaurosis and fell into a coma. A computer tomography of the brain showed diffuse ischemic areas and nodules suggesting fungal infection. Four days later, she developed severe sepsis and vancomycin and meropenem were prescribed, nevertheless she died due to septic shock. In conclusion, cryptococcal meningitis is a rare and severe opportunistic infection in juvenile lupus population. This study reinforces the importance of an early diagnosis and prompt introduction of antifungal agents, especially in patients with history of contact with bird droppings.