Case Presentation:
A 47-year-old Hispanic female with a history of KD diagnosed 13 years
ago presented with recurrent fevers, desquamative rash on the legs, and
diffuse arthralgias. She reported daily fevers at noon and in the
evening starting one month ago. One week prior to her presentation, she
developed abdominal pain associated with nausea, anorexia, and dark
urine. Vital signs on arrival were unremarkable without evidence of
hypotension, tachycardia, or hypoxia. Physical examination was
significant for a desquamative rash on her thighs bilaterally with no
clinical lymphadenopathy. Infectious work up including chest x-ray,
urinalysis, complete blood count, and blood cultures were unremarkable.
Further laboratory results revealed an elevated AST 241 units/L, ALT 277
units/L, and ALP 118 units/L (previously normal 3 months ago).
Autoimmune work up including ANA, anti-dsDNA, anti-SSA/SSB were negative
but did have a weakly positive ASMA with titers of 1:40. Ultrasound of
the abdomen showed cholelithiasis without cholecystitis and hepatic
steatosis. CT neck showed a 1.8 cm right submandibular, 1.6 cm left
submandibular, subcentimeter submental and supraclavicular lymph nodes.
Her lymphadenopathy with daily fevers, skin rash and joint pain was
concerning for recurrence of her KD. Given her elevated liver function
tests, there was also concern for concomitant acute AIH. Patient then
underwent liver biopsy which confirmed AIH. Patient was subsequently
started on methylprednisolone with improvement in her liver function
tests along with her fevers and arthralgias. She was started on
Azathioprine at discharge and continued to follow up in rheumatology and
liver clinic as outpatient for management of her disease.
The patient had a known history of AIH associated with KD, which was
diagnosed 13 years ago via liver biopsy and axillary lymph node biopsy,
respectively (Figure 1) . Work up at the time of diagnosis was
negative for ANA, anti-dsDNA, anti-Sm, anti-RNP and positive for
elevated liver enzymes. She was started on corticosteroids and
azathioprine, which was self-discontinued after 8 years. Her symptoms
were quiescent for years until now.