Case report
A 31-year-old woman was diagnosed with AOSD in October 2013. The
diagnosis was based on the presence of pyrexia, skin rash, sore throat,
lymphadenopathy, leukocytosis, hyperferritinemia, and the lack of
rheumatoid factor and antinuclear antibodies. The clinical findings met
four major criteria (fever, arthralgia, typical rash, leukocytosis) and
three minor criteria (sore throat, lymphadenopathy, negative test for
antinuclear antibody and rheumatoid factor) for AOSD classification
proposed by Yamaguchi et al.1 After the administration
of 40 mg of prednisolone (PSL) per day, her symptoms improved and PSL
was tapered. Prednisolone was maintained at 5–15 mg/day, depending on
the severity of AOSD, but the patient’s symptoms sometimes relapsed
because of drug discontinuation. In October 2015, 50 mg/day cyclosporine
A (CyA) was started, and the dose was increased to 100 mg/day in
February 2017. In July 2017, tocilizumab was started as persistent
fever, lymphadenopathy, and hyperferritinemia (3944 ng/mL) persisted.
In October 2017, the patient was admitted to our hospital with liver
dysfunction, which was the only finding, without any symptoms. Worsening
of AOSD was not suspected as the C-reactive protein (CRP) concentration
was almost normal and there was only mild hyperferritinemia (Table 1).
Liver biopsy revealed the collapse of hepatocytes around the central
vein (Figure 1), infiltration of eosinophils, Kupffer cell hyperplasia,
and mild fibrosis around the Glisson’s capsule. We suspected liver
dysfunction due to AOSD, acute-onset AIH, or drug-induced liver injury
(DILI) caused by tocilizumab. We started methylprednisolone (mPSL) 125
mg/day for the first 3 days, followed by oral administration of PSL (50
mg/day) and CyA (100 mg/day). Prednisolone was gradually tapered and
continued at 5 mg/day. However, in September 2020, both PSL and CyA were
discontinued because of the patient’s strong desire to stop taking them.
Thereafter, she presented to our hospital in January 2021 with a history
of fatigue, loss of appetite, and jaundice for 3 days. Physical
examination revealed diffuse jaundice, but no lymphadenopathy or rash
that suggested the worsening of AOSD. The laboratory findings are shown
in Table 1. There were no specific results for other liver diseases, as
of October 2017. Imaging revealed hepatosplenomegaly without tumorous or
obstructive hepatobiliary diseases (Figure 2). Liver biopsy revealed
interface hepatitis, hepatocyte rosette formation, periportal
inflammation, plasma cell infiltrates, and emperipolesis; these are
indicative of AIH (Figure 3). As her revised international AIH group
score was 18, we diagnosed the patient with AIH complicated by AOSD. We
started mPSL semi-pulse therapy (500 mg/day) for the first 3 days,
followed by a maintenance dose of oral PSL 50 mg/day in combination with
ursodeoxycholic acid 600 mg/day. Her symptoms and liver dysfunction
improved gradually, but liver function was exacerbated on day 8. We
therefore administered 100 mg of CyA, which improved liver function
(Figure 4). Subsequently, PSL was tapered by 5–10 mg every 2 weeks. The
patient’s liver function is currently normal on a dosage of PSL 3 mg/day
and CyA 100 mg/day.