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.