The subject was a 22-year-old woman who developed high
fever and
arthralgias and eruptions in the extremities around June 2005. She sought medical advice at a nearby dermatology clinic, where hepatic dysfunction was noted on blood testing. The patient was thus hospitalized the next day. Although CRP levels were significantly high, no sign of
infection was observed and bone marrow cell differentiation was normal.
Adult onset Still's disease was diagnosed based on the observation of persistent high
fever >39 degrees C, eruptions, increased leukocytes, pharyngeal
pain,
splenomegaly, hepatic dysfunction, negative
autoantibody results from blood testing, and high serum
ferritin levels. Administration of
prednisolone 30 mg/day was initiated, but proved ineffective.
Steroid pulse
therapy was conducted, and the subject was transferred to our medical facility for continued treatment. Attempts were made to control the disease using combined
steroid and
cyclosporine administration; but exacerbation of high serum
ferritin levels and hepatic dysfunctions were observed, so a second course of
steroid pulse
therapy was conducted. Symptoms improved temporarily, but
steroid levels were difficult to reduce.
Cyclosporine was therefore replaced by
methotrexate, and administration of
infliximab was initiated. In the course of treatment, administration of a
sulfamethoxazole/trimethoprim combination was initiated, but was discontinued due to suspicion of
drug-induced hepatic injury. A second administration of
infliximab was conducted in late August, and rapid improvements in clinical symptoms and abnormal test values was observed. However, high
fever and
headache developed suddenly in early September. Based on the results of spinal fluid testing, blood and spinal fluid cultures and MRI of the head,
Listeria meningoencephalitis was diagnosed.
Diplopia and impaired consciousness occurred during the disease course, and formation of a
brain abscess was observed on imaging. However, symptoms were controlled by long-term combination administration of
ampicillin and
gentamicin. Administration of
infliximab was discontinued for treatment of
adult onset Still's disease, and
steroid levels were reduced following double-membrane filtration
plasma exchange. On follow-up, no relapse of symptoms or abnormalities in blood test values were observed, so the subject was discharged from our medical facility in December 2005. In treatment for
rheumatic diseases, a dramatic improvement in treatment results for pathologies displaying tolerance against conventional treatments has been acquired with the development of
biological drugs. However,
opportunistic infections represent a serious problem, and appropriate preventative measures are required. The present report describes a case in which the subject was affected by
Listeria meningoencephalitis during administration of
infliximab for
steroid-dependent adult Still's disease. Since
listeriosis is one of the complications, along with
tuberculosis, that warrants precautionary measures, this case is reported and discussed.