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.